Frank Hanley

All Publications

• The Role of Microenvironment in Preserving the Potency of Adult Porcine Pulmonary Valve Stem Cells In Vitro INTERNATIONAL JOURNAL OF STEM CELLS Chalajour, F., Siyahian, A., Hanley, F. L. 2018; 11 (1): 121-+

  Abstract

  The potency of tissue resident stem cells is regulated primarily by inputs from the local microenvironment. Isolation of stem cells through enzymatic digestion of tissue may affect epigenetic regulation of cell fate and performance. Here we employ a non-enzymatic method to harvest and investigate tissue resident stem cells from the adult porcine pulmonary valve.The presence of c-Kit⁺ stem cells within the valve tissue was confirmed by immunohistochemistry. An in vitro culture of minced valve leaflets was developed under the standard conditions (37°C with 5% CO₂). The viability of the cellular outgrowths was evaluated over the subsequent 12 weeks. Under this culture condition, we identified a population of non-adherent c-Kit⁺ cells and multiple cellular structures mimicking the phenotype of embryonic stem cells at different stages of development. Formation of multinucleated cells through cell fusion provided an active niche area for homing and interaction of the non-adherent c-Kit⁺ cells. Expression of pluripotency markers Oct-4 and Nanog was detected in the newly formed multinucleated cells but not in mature colonies. Partial cell fusion was shown by fluorescent live-cell tracking, which confirmed intercellular molecular exchange between donor and recipient cells, resulting in altered cytoplasmic protein expression by the recipient cell.These results suggest a role for the microenvironment in decrypting the potential of the valve somatic stem cells in vitro. In addition, our data provide evidence for cell fusion, which may play a critical role in reversing somatic cell fate and spontaneous cellular reprogramming.

  View details for PubMedID 29843194

• Repair of Ductus or Hemi-Truncus to One Lung and Major Aortopulmonary Collaterals to the Other Lung. The Annals of thoracic surgery Mainwaring, R. D., Rosenblatt, T. R., Patrick, W. L., Ma, M., Peng, L., Hanley, F. L. 2018

  Abstract

  BACKGROUND: There are patients born with pulmonary atresia and a ductus arteriosus or hemi-truncus to one lung and major aortopulmonary collateral arteries (MAPCAs) to the contralateral lung. The purpose of this study was to review our surgical results for this relatively rare subset of patients.METHODS: This was a retrospective review of 35 patients with ductus/hemi-truncus in association with pulmonary atresia with ventricular septal defect and MAPCAs. Our surgical algorithm is bifurcated into two strategies: 1) patients with "favorable" MAPCAs are candidates for single stage complete repair, and 2) patients with "unfavorable" MAPCAs undergo a staged approach. The median age at surgery was 3 months (range 1-7 months), and the mean number of MAPCAs was 3.1 ± 1.3.RESULTS: Twenty patients underwent a single stage complete repair. All 20 of these patients are alive with a right ventricle to aortic pressure ratio of 0.34 ± 0.07. The distribution of pulmonary blood flow by lung perfusion scan was 44% to the right and 56% to the left lung. Fifteen patients underwent a staged approach, including unifocalization of the MAPCAs to a central shunt. There were two interim deaths. Twelve patients have undergone complete repair with a right ventricle to aortic pressure ratio of 0.38 ± 0.07. There was one late death after complete repair. The distribution of pulmonary blood flow was 39% to the right and 61% to the left lung.CONCLUSIONS: The data demonstrate that 32 of 35 patients with ductus/hemi-truncus and MAPCAs ultimately achieved complete repair with relatively low pulmonary artery pressures.

  View details for PubMedID 29684371

• Surgical algorithm and results for repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals. The Journal of thoracic and cardiovascular surgery Mainwaring, R. D., Patrick, W. L., Roth, S. J., Kamra, K., Wise-Faberowski, L., Palmon, M., Hanley, F. L. 2018

  Abstract

  OBJECTIVE: Pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries is a complex and heterogeneous form of congenital heart disease. There is a controversy regarding the optimal treatment of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries. The purpose of this study was to summarize our algorithm and surgical results for pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries.METHODS: This was a retrospective review of 307 patients undergoing primary surgical treatment of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries. Excluded from this analysis were patients who had undergone prior surgical treatment at another institution and patients with single ventricle and major aortopulmonary collateral arteries. There were 3 surgical pathways, including midline unifocalization (n=241), creation of an aortopulmonary window (n=46), and other (n=20).RESULTS: For the 241 patients who underwent midline unifocalization, 204 (85.4%) had a single-stage complete repair. There were 37 patients who underwent a midline unifocalization and central shunt, and 24 have subsequently undergone complete repair. Forty-six patients underwent an aortopulmonary window, of whom 36 have subsequently had a complete repair. There were 20 patients who had complex anatomy and underwent procedures other than described, and14 have subsequently undergone complete repair. Thus, for the patients currently eligible, 280 (93.0%) have achieved complete repair. For the 204 patients who had a single-stage complete repair, the mean right ventricle to aortic pressure ratio was 0.36±0.09. Seventy-six patients underwent a staged repair, and the mean right ventricle to aortic pressure ratio was 0.40±0.09 (P<.05 compared with single-stage repair). There were 3 (1.5%) early and 8 (4.0%) late deaths for the single-stage complete repair cohort versus 4 (4.0%) early and 15 (14.9%) late deaths for all other procedures (P<.01).CONCLUSIONS: The data demonstrate that more than 90% of patients with pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries achieved complete repair. The overall mortality was significantly lower in the subgroup of patients who underwent single-stage complete repair.

  View details for PubMedID 29789151

• Pulmonary reinterventions after complete unifocalization and repair in infants and young children with tetralogy of Fallot with major aortopulmonary collaterals JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY Bauser-Heaton, H., Borquez, A., Asija, R., Wise-Faberowski, L., Zhang, Y., Downey, L., Perry, S. B., Koth, A., Peng, L. F., Algaze, C. A., Hanley, F. L., McElhinney, D. B. 2018; 155 (4): 1696–1707

  Abstract

  Our institutional approach to tetralogy of Fallot (TOF) with major aortopulmonary collaterals (MAPCAs) emphasizes unifocalization and augmentation of the reconstructed pulmonary arterial (PA) circulation and complete intracardiac repair in infancy, usually in a single procedure. This approach yields a high rate of complete repair with excellent survival and low right ventricular (RV) pressure. However, little is known about remodeling of the unifocalized and reconstructed pulmonary circulation or about reinterventions on the reconstructed PAs or the RV outflow tract conduit.We reviewed patients who underwent complete repair of TOF with MAPCAs at our center at <2 years of age, either as a single-stage procedure or after previous procedures. Outcomes included freedom from conduit or PA intervention after repair, which were assessed by Cox regression and Kaplan-Meier analysis.The study cohort included 272 patients. There were 6 early deaths and a median of follow-up of 3.6 years after complete repair. Reinterventions on the pulmonary circulation were performed in 134 patients, including conduit interventions in 101 patients, branch PA interventions in 101, and closure of residual MAPCAs in 9. The first conduit reintervention consisted of surgical conduit replacement in 77 patients, transcatheter pulmonary valve replacement with a Melody valve in 14, and angioplasty or bare metal stenting in 10. Surgical PA reinterventions were performed in 46 patients and transcatheter reinterventions in 75 (both in 20). Most PA reinterventions involved a single lung, and most transcatheter reinterventions a single vessel. Freedom from conduit replacement or transcatheter pulmonary valve replacement was 70 ± 3% at 5 years and was shorter in patients with smaller initial conduit size. Freedom from any PA reintervention was 64 ± 3% at 5 years, with the greatest rate during the first year. On multivariable analysis, factors associated with longer freedom from any PA reintervention included lower postrepair RV:aortic pressure ratio and larger original conduit size.We were able to obtain follow-up data for the majority of patients, which demonstrated freedom from PA reintervention for two thirds of patients. The time course of and risk factors for conduit reintervention in this cohort appeared similar to previously reported findings in patients who received RV-PA conduits in early childhood for other anomalies. Relative to the severity of baseline pulmonary vascular anatomy in TOF with MAPCAs, reinterventions on the reconstructed PAs were uncommon after repair according to our approach, and major reinterventions were rare. Nevertheless, PA reinterventions are an important aspect of the overall management strategy.

  View details for PubMedID 29352588

• Interstage evaluation of homograft-valved right ventricle to pulmonary artery conduits for palliation of hypoplastic left heart syndrome JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY Sandeep, N., Punn, R., Balasubramanian, S., Smith, S. N., Reinhartz, O., Zhang, Y., Wright, G. E., Peng, L. F., Wise-Faberowski, L., Hanley, F. L., McElhinney, D. B. 2018; 155 (4): 1747-+

  Abstract

  Palliation of hypoplastic left heart syndrome with a standard nonvalved right ventricle to pulmonary artery conduit results in an inefficient circulation in part due to diastolic regurgitation. A composite right ventricle pulmonary artery conduit with a homograft valve has a hypothetical advantage of reducing regurgitation, but may differ in the propensity for stenosis because of valve remodeling.This retrospective cohort study included 130 patients with hypoplastic left heart syndrome who underwent a modified stage 1 procedure with a right ventricle to pulmonary artery conduit from 2002 to 2015. A composite valved conduit (cryopreserved homograft valve anastomosed to a polytetrafluoroethylene tube) was placed in 100 patients (47 aortic, 32 pulmonary, 13 femoral/saphenous vein, 8 unknown), and a nonvalved conduit was used in 30 patients. Echocardiographic functional parameters were evaluated before and after stage 1 palliation and before the bidirectional Glenn procedure, and interstage interventions were assessed.On competing risk analysis, survival over time was better in the valved conduit group (P = .040), but this difference was no longer significant after adjustment for surgical era. There was no significant difference between groups in the cumulative incidence of bidirectional Glenn completion (P = .15). Patients with a valved conduit underwent more interventions for conduit obstruction in the interstage period, but this difference did not reach significance (P = .16). There were no differences between groups in echocardiographic parameters of right ventricle function at baseline or pre-Glenn.In this cohort of patients with hypoplastic left heart syndrome, inclusion of a valved right ventricle to pulmonary artery conduit was not associated with any difference in survival on adjusted analysis and did not confer an identifiable benefit on right ventricle function.

  View details for PubMedID 29223842

• Impact of Phrenic Nerve Palsy and Need for Diaphragm Plication Following Surgery for Pulmonary Atresia With Ventricular Septal Defect and Major Aortopulmonary Collaterals. Seminars in thoracic and cardiovascular surgery Greene, C. L., Mainwaring, R. D., Sidell, D., Yarlagadda, V. V., Patrick, W. L., Hanley, F. L. 2018

  Abstract

  Injury to the phrenic nerves may occur during surgery for pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries (PA-VSD and MAPCAs). These patients may develop respiratory failure and require diaphragm plication. The purpose of this study was to evaluate the impact of phrenic nerve palsy on recovery following surgery for PA-VSD and MAPCAs. Between 2007 and 2016, approximately 500 patients underwent surgery for PA-VSD and MAPCAs at our institution. Twenty-four patients (4.8%) subsequently had evidence of new phrenic nerve palsy. Sixteen patients were undergoing their first surgical procedure, whereas 8 were undergoing reoperations. All 24 patients underwent diaphragm plication. A cohort of matched controls was identified based on identical diagnosis and procedures but did not sustain a phrenic nerve palsy. Eighteen of the 24 patients (75%) had clinical improvement following diaphragm plication as evidenced by the ability to undergo successful extubation (5±2 days), transition out of the intensive care unit (32±16 days), and discharge from the hospital (42±19 days). In contrast, there were 6 patients (25%) who did not demonstrate a temporal improvement following diaphragm plication, as evidenced by intervals of 61±38, 106±45, and 108±46 days, respectively (P<0.05 for all 3 comparisons). The 6 patients who failed to improve following diaphragm plication had a significantly greater number of comorbidities compared to the 18 patients who demonstrated improvement (2.2 vs 0.6 per patient, P<0.05). When compared with the control group, patients who improved following diaphragm plication spent an additional 22 days and patients who failed to improve an additional 90 days in the hospital. The data demonstrate a bifurcation of clinical outcome in patients undergoing diaphragm plication following surgery for PA-VSD and MAPCAs. This bifurcation appears to be linked to the presence or absence of other comorbidities.

  View details for PubMedID 29545034

• THE DEVELOPMENT AND EFFICACY OF A PEDIATRIC CARDIOLOGY FELLOWSHIP ONLINE PREPARATORY COURSE Motonaga, K., Sacks, L., Olson, I., Balasubramanian, S., Chen, S., Peng, L., Feinstein, J., Silverman, N., Hanley, F., Axelrod, D., Krawczeski, C., Ceresnak, S. ELSEVIER SCIENCE INC. 2018: 2622

  View details for DOI 10.1016/S0735-1097(18)33163-2

  View details for Web of Science ID 000429659705072

• Comprehensive Management of Major Aortopulmonary Collaterals in the Repair of Tetralogy of Fallot. Seminars in thoracic and cardiovascular surgery. Pediatric cardiac surgery annual Ma, M., Mainwaring, R. D., Hanley, F. L. 2018; 21: 75–82

  Abstract

  The heterogenous anatomy of Tetralogy of Fallot with major aortopulmonary collateral arteries has engendered a similar degree of diversity in its management and, ultimately, outcome. We summarize our comprehensive treatment paradigm for this lesion evolved over 15 years of experience through 458 patients and the results obtained. An updated analysis of 307 patients treated primarily at our institution is included. A review of recent literature, comparison of management strategies, and discussion of ongoing controversies are provided.

  View details for PubMedID 29425528

• Anesthetic Management During Surgery for Tetralogy of Fallot With Pulmonary Atresia and Major Aortopulmonary Collateral Arteries WORLD JOURNAL FOR PEDIATRIC AND CONGENITAL HEART SURGERY Quinonez, Z. A., Downey, L., Abbasi, R. K., Kuan, C., Asija, R., McElhinney, D. B., Hanley, F. L., Mainwaring, R. D., Wise-Faberowski, L. 2018; 9 (2): 236–41

  Abstract

  Tetralogy of Fallot with pulmonary atresia and major aortopulmonary collaterals (TOF/PA/MAPCAs) is a heterogeneous disease with varying degrees of severity, requiring complex anesthetic management. Our institution has adopted the approach of early complete repair with incorporation of all lung segments, extensive lobar and branch segmental pulmonary artery reconstruction, and ventricular septal defect closure. While the surgical management of TOF/PA/MAPCAs has been extensively described and varies depending on the institution, there is a paucity of literature on the anesthetic management for such procedures. Herein, we describe our anesthetic management based on our own institution's surgical approach at Lucile Packard Children's Hospital/Stanford University.

  View details for PubMedID 29544416

• An analysis of patients requiring unifocalization revision following midline unifocalization for pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals. European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery Mainwaring, R. D., Patrick, W. L., Ma, M., Hanley, F. L. 2018

  Abstract

  OBJECTIVES: Midline unifocalization has been developed for the surgical treatment of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries. All patients will eventually require reoperation because of the presence of a conduit, and some patients may also require revision of the distal unifocalized bed. The purpose of this study was to analyse the need for unifocalization revision following midline unifocalization.METHODS: This was a retrospective review of 241 patients who underwent midline unifocalization for the treatment of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries. Two hundred and four (85.4%) patients had a single-stage complete repair, whereas 37 patients had a unifocalization and placement of a central shunt. Seventy-eight patients have subsequently undergone reoperations at our institution, including 44 operations that required unifocalization revision. These 44 patients were compared with the 191 operative survivors who did not require revision.RESULTS: An analysis of risk factors for requiring unifocalization revision included the following: (i) single-stage complete repair versus unifocalization and shunt (14.7% vs 37.8%, P<0.001), (ii) right ventricle to aortic pressure ratio at the initial repair (0.33±0.07 vs 0.44±0.08, P<0.001) and (iii) absence of central pulmonary arteries (32.8% vs 13.4%, P<0.001).CONCLUSIONS: Data demonstrate that 44 of 241 (18%) patients who underwent midline unifocalization have subsequently required revision of their unifocalization. The need for unifocalization revision was associated with 3 factors, all of which were known at the time of discharge from the initial unifocalization. These data suggest that potentially higher risk patients should be monitored more closely than their lower risk counterparts.

  View details for PubMedID 29447337

• Echocardiography-Derived Left Ventricular Outflow Tract Gradient and Left Ventricular Posterior Wall Thickening Are Associated with Outcomes for Anatomic Repair in Congenitally Corrected Transposition of the Great Arteries. Journal of the American Society of Echocardiography Moodley, S., Balasubramanian, S., Tacy, T. A., Chan, F., Hanley, F. L., Punn, R. 2017

  Abstract

  Congenitally corrected transposition of the great arteries is a rare form of congenital heart disease. Management is controversial; options include observation, physiologic repair, and anatomic repair. Assessment of morphologic left ventricle preparedness is key in timing anatomic repair. This study's purpose was to review the modalities used to assess the morphologic left ventricle preoperatively and to determine if any echocardiographic variables are associated with outcomes.A retrospective review of patients with congenitally corrected transposition of the great arteries eligible for anatomic repair at Lucile Packard Children's Hospital from 2000 to 2016 was conducted. Inclusion criteria were (1) presurgical echocardiography, magnetic resonance imaging, and cardiac catheterization and (2) clinical follow-up information. Echocardiographic measurements included left ventricular (LV) single-plane Simpson's ejection fraction, LV eccentricity index, LV posterior wall thickening, pulmonary artery band (PAB)/LV outflow tract (LVOT) pressure gradient, and LV and right ventricular strain. Magnetic resonance imaging measurements included LV mass, ejection fraction, eccentricity index, and LV thickening. LV pressure, PAB/LVOT gradient, right ventricular pressure, pulmonary vascular resistance, and Qp/Qs constituted catheterization data. Outcomes included achieving anatomic repair within 1 year of assessment in patients with LVOT obstruction or within 1 year of pulmonary artery banding and freedom from death, transplantation, or heart failure at last follow-up.Forty-one patients met the inclusion criteria. PAB/LVOT gradients of 85.2 ± 23.4 versus 64.0 ± 32.1 mm Hg (P = .0282) by echocardiography and 60.1 ± 19.4 versus 35.9 ± 18.9 mm Hg (P = .0030) by catheterization were associated with achieving anatomic repair and freedom from death, transplantation, and heart failure. Echocardiographic LV posterior wall thickening of 35.4 ± 19.8% versus 20.6 ± 15.0% (P = .0017) and MRI LV septal wall thickening of 37.1 ± 18.8% versus 19.3 ± 18.8% (P = .0306) were associated with achieving anatomic repair. Inter- and intraobserver variability for echocardiographic measurements was very good.PAB/LVOT gradient and LV posterior wall thickening are highly reproducible echocardiographic measurements that reflect morphologic LV performance and can be used in assessing patients with congenitally corrected transposition of the great arteries undergoing anatomic repair.

  View details for DOI 10.1016/j.echo.2017.03.019

  View details for PubMedID 28579248

• Major Aortopulmonary Collateral Arteries With Anatomy Other Than Pulmonary Atresia/Ventricular Septal Defect. Annals of thoracic surgery Patrick, W. L., Mainwaring, R. D., Reinhartz, O., Punn, R., Tacy, T., Hanley, F. L. 2017

  Abstract

  Major aortopulmonary collateral arteries (MAPCAs) are frequently found in association with pulmonary atresia with ventricular septal defect (PA/VSD). However, some patients with MAPCAs do not have PA/VSD but have a variety of other "atypical" anatomic diagnoses.This was a retrospective review of patients with MAPCAs and atypical anatomy. The 50 patients with MAPCAs could be divided into two subgroups: (1) single ventricle anatomy (n = 33) and (2) two ventricle anatomy (n = 17).The 33 patients with MAPCAs and single ventricle included 15 with unbalanced complete atrioventricular canal (CAVC), 6 with pulmonary atresia-intact ventricular septum, and 12 with other forms of single ventricle. The initial cardiac operation included unifocalization/shunt in 24 patients and creation of aortopulmonary window or central shunt in 9 patients. There were seven operative and eight late deaths. Sixteen patients have had a bidirectional Glenn procedure and 6 had a Fontan procedure. The 17 patients with MAPCAs and two ventricles included 5 with CAVC, 4 with corrected transposition, 3 with double outlet right ventricle, 3 with scimitar syndrome, and 2 with complex D-transposition. The initial cardiac operation included single-stage complete repair in 5 patients, unifocalization/shunt in 10 patients, and aortopulmonary window in 2 patients. There were two operative and two late deaths. Thirteen patients have achieved complete repair status.The data demonstrate the wide diversity of anatomy seen in patients with MAPCAs when evaluating diagnoses other than PA/VSD. Two-thirds of the patients had single ventricle and was associated with a relatively high mortality.

  View details for DOI 10.1016/j.athoracsur.2017.02.029

  View details for PubMedID 28527961

• Bronchoscopy in children with tetralogy of fallot, pulmonary atresia, and major aortopulmonary collaterals. Pediatric pulmonology Sidell, D. R., Koth, A. M., Bauser-Heaton, H., McElhinney, D. B., Wise-Faberowski, L., Tracy, M. C., Hanley, F. L., Asija, R. 2017

  Abstract

  Children with Tetralogy of Fallot, Pulmonary Atresia, and Major Aortopulmonary Collaterals (TOF/PA/MAPCAs) undergoing unifocalization surgery are at risk for developing more postoperative respiratory complications than children undergoing other types of congenital heart surgery. Bronchoscopy is used in the perioperative period for diagnostic and therapeutic purposes. In this study, we describe bronchoscopic findings and identify factors associated with selection for bronchoscopy.Retrospective case-control.All patients with TOF/PA/MAPCAs who underwent unifocalization surgery from September 2005 through March 2016 were included. Patients who underwent bronchoscopy in the perioperative period were compared to a randomly selected cohort of 172 control patients who underwent unifocalization without bronchoscopy during the study period.Forty-three children underwent perioperative bronchoscopy at a median of 9 days postoperatively. Baseline demographics were similar in bronchoscopy patients and controls. Patients who underwent bronchoscopy were more likely to have a chromosome 22q11 deletion and were more likely have undergone unifocalization surgery without intracardiac repair. These patients had a longer duration of mechanical ventilation, ICU duration, and length of hospitalization. Abnormalities were detected on bronchoscopy in 35 patients (81%), and 20 (35%) of bronchoscopy patients underwent a postoperative intervention related to abnormalities identified on bronchoscopy.Bronchoscopy is a useful therapeutic and diagnostic instrument for children undergoing unifocalization surgery, capable of identifying abnormalities leading to an additional intervention in over one third of patients. Special attention should be given to children with a 22q11 deletion to expedite diagnosis and intervention for possible airway complications.

  View details for DOI 10.1002/ppul.23732

  View details for PubMedID 28504356

• Programmatic Approach to Management of Tetralogy of Fallot With Major Aortopulmonary Collateral Arteries A 15-Year Experience With 458 Patients CIRCULATION-CARDIOVASCULAR INTERVENTIONS Bauser-Heaton, H., Borquez, A., Han, B., Ladd, M., Asija, R., Downey, L., Koth, A., Algaze, C. A., Wise-Faberowski, L., Perry, S. B., Shin, A., Peng, L. F., Hanley, F. L., McElhinney, D. B. 2017; 10 (4)

  Abstract

  Tetralogy of Fallot with major aortopulmonary collateral arteries is a complex and heterogeneous condition. Our institutional approach to this lesion emphasizes early complete repair with the incorporation of all lung segments and extensive lobar and segmental pulmonary artery reconstruction.We reviewed all patients who underwent surgical intervention for tetralogy of Fallot and major aortopulmonary collateral arteries at Lucile Packard Children's Hospital Stanford (LPCHS) since November 2001. A total of 458 patients underwent surgery, 291 (64%) of whom underwent their initial procedure at LPCHS. Patients were followed for a median of 2.7 years (mean 4.3 years) after the first LPCHS surgery, with an estimated survival of 85% at 5 years after first surgical intervention. Factors associated with worse survival included first LPCHS surgery type other than complete repair and Alagille syndrome. Of the overall cohort, 402 patients achieved complete unifocalization and repair, either as a single-stage procedure (n=186), after initial palliation at our center (n=74), or after surgery elsewhere followed by repair/revision at LPCHS (n=142). The median right ventricle:aortic pressure ratio after repair was 0.35. Estimated survival after repair was 92.5% at 10 years and was shorter in patients with chromosomal anomalies, older age, a greater number of collaterals unifocalized, and higher postrepair right ventricle pressure.Using an approach that emphasizes early complete unifocalization and repair with incorporation of all pulmonary vascular supply, we have achieved excellent results in patients with both native and previously operated tetralogy of Fallot and major aortopulmonary collateral arteries.

  View details for DOI 10.1161/CIRCINTERVENTIONS.116.004952

  View details for PubMedID 28356265

• Left Ventricular Retraining and Double Switch in Patients With Congenitally Corrected Transposition of the Great Arteries. World journal for pediatric & congenital heart surgery Ibrahimiye, A. N., Mainwaring, R. D., Patrick, W. L., Downey, L., Yarlagadda, V., Hanley, F. L. 2017; 8 (2): 203-209

  Abstract

  Congenitally corrected transposition of the great arteries (CC-TGA) is a complex form of congenital heart defect with numerous anatomic subgroups. The majority of patients with CC-TGA are excellent candidates for a double-switch procedure. However, in the absence of an unrestrictive ventricular septal defect or subpulmonary stenosis, the left ventricle (LV) may undergo involution and require retraining prior to double switch. The purpose of this study was to review our experience with patients having CC-TGA who required LV retraining prior to a double-switch procedure.This was a retrospective review of 24 patients with CC-TGA who were enrolled in an LV retraining program in preparation for a double-switch procedure. The median age at the time of enrollment for retraining was 11 months (range 1 month-24 years). The average left ventricle to right ventricle pressure ratio was 0.39 ± 0.07 prior to intervention. All 24 patients underwent placement of an initial pulmonary artery band (PAB) for LV retraining.Eighteen (75%) of the 24 patients underwent a double-switch procedure with no operative mortality. Of these 18 patients, 9 had a single PAB and 9 required a second band for retraining. Six patients have not undergone a double-switch procedure to date. Five patients are good candidates for a double switch and are 2 weeks, 3 weeks, 4 weeks, 8 months, and 35 months since their last PAB. One patient died from a noncardiac cause 26 months after PAB retightening. The 18 patients who underwent a double switch were followed for an average of 5 ± 1 years (range 0.1-10.3 years). There has been no late mortality, and only 2 patients required further reinterventions.The data demonstrate that LV retraining has been highly effective in this select group of patients with CC-TGA. The data also demonstrate that the results of the double-switch procedure have been excellent at midterm follow-up. These results suggest that LV retraining and double switch offer a reliable strategy option for patients with CC-TGA.

  View details for DOI 10.1177/2150135116683939

  View details for PubMedID 28329464

• Practice Patterns in Postoperative Echocardiographic Surveillance after Congenital Heart Surgery in Children: A Single Center Experience JOURNAL OF PEDIATRICS Arunamata, A., Axelrod, D. M., Kipps, A. K., McElhinney, D. B., Shin, A. Y., Hanley, F. L., Olson, I. L., Roth, S. J., Tierney, E. S. 2017; 180: 87-?

  Abstract

  To review current institutional practice and describe factors contributing to variation in inpatient postoperative imaging surveillance after congenital heart surgery.We reviewed records of all children who underwent congenital heart surgery from June to December 2014. Number and primary indications for postoperative transthoracic echocardiograms (TTEs), providers involved, cardiovascular intensive care unit (CVICU) and total hospital length of stay, and Risk-Adjustment for Congenital Heart Surgery-1 scores were recorded.A total of 253 children (age at surgery: 8 months [2 days-19 years]) received 556 postoperative TTEs (median 1 TTE/patient [1-14]), and 23% had ≥3 TTEs. Fifteen of 556 TTEs (2.7%) revealed a new abnormal finding. The majority of TTEs (59%) were performed in the CVICU (1.5 ± 1.1 TTEs/week/patient), with evaluation of function as the most common indication (44%). Attending physician practice >10 years was not associated with fewer TTEs (P = .12). Patients with ≥3 TTEs had higher Risk-Adjustment for Congenital Heart Surgery-1 scores (P = .001), longer CVICU lengths of stay (22 vs 3 days; P < .0001), longer overall hospitalizations (28 vs 7 days; P < .0001), and a higher incidence of mechanical circulatory support (10% vs 0%; P < .0001) than those with <3 TTEs. Eight patients with ≥3 TTEs did not survive, compared with 3 with <3 TTEs (P = .0004).There was wide intra-institutional variation in echocardiographic use among similar complexity surgeries. Frequency of postoperative echocardiographic surveillance was associated with degree of surgical complexity and severity of postoperative clinical condition. Few studies revealed new abnormal findings. These results may help establish evidence-based guidelines for inpatient echocardiographic surveillance after congenital heart surgery.

  View details for DOI 10.1016/j.jpeds.2016.09.061

  View details for Web of Science ID 000390028100018

• Computational simulation of postoperative pulmonary flow distribution in Alagille patients with peripheral pulmonary artery stenosis Computational simulation of postoperative pulmonary flow distribution in Alagille patients with peripheral pulmonary artery stenosis Yang, W., Feinstein, J., Marsden, A., Vignon-Clementel, I. 2017; 00: 1–10

  View details for DOI 10.1111/chd.12556

• An Analysis of Left Ventricular Retraining in Patients With Dextro- and Levo-Transposition of the Great Arteries. The Annals of thoracic surgery Mainwaring, R. D., Patrick, W. L., Ibrahimiye, A. N., Watanabe, N. n., Lui, G. K., Hanley, F. L. 2017

  Abstract

  Patients with dextro (D)-transposition of the great arteries (TGA) who have undergone a previous atrial switch and for some patients with levo (L)-TGA (ie, no ventricular septal defect or outflow tract obstruction), the left ventricle (LV) may require retraining before late arterial switch. The purpose of this study was to analyze the results of LV retraining for these two entities.This was a retrospective review of 51 patients enrolled in an LV retraining program. There were 25 patients with D-TGA and 26 with L-TGA. The median age of the D-TGA patients was 15 years, and 22 of 25 were in New York Heart Association class III or IV. The median age of the L-TGA patients was 12 months, and 3 of 26 patients were in New York Heart Association class III or IV.LV retraining was successful in 13 of the 25 patients (52%) with D-TGA, and 10 these 13 patients (77%) underwent successful late arterial switch. Of the 26 patients with L-TGA, LV retraining was successful in 24 (92%), and a double-switch operation was successful in 19 of 19 (100%) who subsequently underwent that procedure. A mathematical formula based on the incremental gain in left-to-right ventricular pressure ratio correctly predicted the success or failure in 94% of the patients.The data demonstrate differences in the success of LV retraining and late arterial switch for D-TGA and L-TGA. We would propose that the LV retraining ratio may be useful in objectively selecting patients eligible for late arterial switch.

  View details for PubMedID 29274314

• Acute Kidney Injury in Patients Undergoing the Extracardiac Fontan Operation With and Without the Use of Cardiopulmonary Bypass PEDIATRIC CRITICAL CARE MEDICINE Algaze, C. A., Koth, A. M., Faberowski, L. W., Hanley, F. L., Krawczeski, C. D., Axelrod, D. M. 2017; 18 (1): 34-43

  Abstract

  To describe the prevalence and risk factors for acute kidney injury in patients undergoing the extracardiac Fontan operation with and without cardiopulmonary bypass, and to determine whether acute kidney injury is associated with duration of mechanical ventilation, cardiovascular ICU and hospital postoperative length of stay, and early mortality.Single-center retrospective cohort study.Pediatric cardiovascular ICU, university-affiliated children's hospital.Patients with a preoperative creatinine before undergoing first-time extracardiac Fontan between January 1, 2004, and April 30, 2012.None.Acute kidney injury occurred in 55 of 138 patients (39.9%), including 41 (29.7%) with stage 1, six (4.4%) with stage 2, and eight (5.8%) with stage 3 acute kidney injury. Cardiopulmonary bypass was strongly associated with a higher risk of any acute kidney injury (adjusted odds ratio, 4.8 [95% CI, 1.4-16.0]; p = 0.01) but not stage 2/3 acute kidney injury. Lower renal perfusion pressure on the day of surgery (postoperative day, 0) was associated with a higher risk of stage 2/3 acute kidney injury (adjusted odds ratio, 1.2 [95% CI, 1.0-1.5]; p = 0.03). Higher vasoactive-inotropic score on postoperative day 0 was associated with a higher risk for stage 2/3 acute kidney injury (adjusted odds ratio, 1.9 [95% CI, 1.0-3.4]; p = 0.04). Stage 2/3 acute kidney injury was associated with longer cardiovascular ICU length of stay (mean, 7.3 greater d [95% CI, 3.4-11.3]; p < 0.001) and hospital postoperative length of stay (mean, 6.4 greater d [95% CI, 0.06-12.5]; p = 0.04).Postoperative acute kidney injury in patients undergoing the extracardiac Fontan operation is common and is associated with lower postoperative renal perfusion pressure and higher vasoactive-inotropic score. Cardiopulmonary bypass was strongly associated with any acute kidney injury, although not stage 2/3 acute kidney injury. Stage 2/3 acute kidney injury is a compelling risk factor for longer cardiovascular ICU and hospital postoperative length of stay. Increased attention to and management of renal perfusion pressure may reduce postoperative acute kidney injury and improve outcomes.

  View details for DOI 10.1097/PCC.0000000000000984

  View details for Web of Science ID 000392249300005

• Practice Patterns in Postoperative Echocardiographic Surveillance after Congenital Heart Surgery in Children: A Single Center Experience. journal of pediatrics Arunamata, A., Axelrod, D. M., Kipps, A. K., McElhinney, D. B., Shin, A. Y., Hanley, F. L., Olson, I. L., Roth, S. J., Selamet Tierney, E. S. 2016

  Abstract

  To review current institutional practice and describe factors contributing to variation in inpatient postoperative imaging surveillance after congenital heart surgery.We reviewed records of all children who underwent congenital heart surgery from June to December 2014. Number and primary indications for postoperative transthoracic echocardiograms (TTEs), providers involved, cardiovascular intensive care unit (CVICU) and total hospital length of stay, and Risk-Adjustment for Congenital Heart Surgery-1 scores were recorded.A total of 253 children (age at surgery: 8 months [2 days-19 years]) received 556 postoperative TTEs (median 1 TTE/patient [1-14]), and 23% had ≥3 TTEs. Fifteen of 556 TTEs (2.7%) revealed a new abnormal finding. The majority of TTEs (59%) were performed in the CVICU (1.5 ± 1.1 TTEs/week/patient), with evaluation of function as the most common indication (44%). Attending physician practice >10 years was not associated with fewer TTEs (P = .12). Patients with ≥3 TTEs had higher Risk-Adjustment for Congenital Heart Surgery-1 scores (P = .001), longer CVICU lengths of stay (22 vs 3 days; P < .0001), longer overall hospitalizations (28 vs 7 days; P < .0001), and a higher incidence of mechanical circulatory support (10% vs 0%; P < .0001) than those with <3 TTEs. Eight patients with ≥3 TTEs did not survive, compared with 3 with <3 TTEs (P = .0004).There was wide intra-institutional variation in echocardiographic use among similar complexity surgeries. Frequency of postoperative echocardiographic surveillance was associated with degree of surgical complexity and severity of postoperative clinical condition. Few studies revealed new abnormal findings. These results may help establish evidence-based guidelines for inpatient echocardiographic surveillance after congenital heart surgery.

  View details for DOI 10.1016/j.jpeds.2016.09.061

  View details for PubMedID 28029346

• Acute Kidney Injury in Patients Undergoing the Extracardiac Fontan Operation With and Without the Use of Cardiopulmonary Bypass. Pediatric critical care medicine Algaze, C. A., Koth, A. M., Faberowski, L. W., Hanley, F. L., Krawczeski, C. D., Axelrod, D. M. 2016: -?

  Abstract

  To describe the prevalence and risk factors for acute kidney injury in patients undergoing the extracardiac Fontan operation with and without cardiopulmonary bypass, and to determine whether acute kidney injury is associated with duration of mechanical ventilation, cardiovascular ICU and hospital postoperative length of stay, and early mortality.Single-center retrospective cohort study.Pediatric cardiovascular ICU, university-affiliated children's hospital.Patients with a preoperative creatinine before undergoing first-time extracardiac Fontan between January 1, 2004, and April 30, 2012.None.Acute kidney injury occurred in 55 of 138 patients (39.9%), including 41 (29.7%) with stage 1, six (4.4%) with stage 2, and eight (5.8%) with stage 3 acute kidney injury. Cardiopulmonary bypass was strongly associated with a higher risk of any acute kidney injury (adjusted odds ratio, 4.8 [95% CI, 1.4-16.0]; p = 0.01) but not stage 2/3 acute kidney injury. Lower renal perfusion pressure on the day of surgery (postoperative day, 0) was associated with a higher risk of stage 2/3 acute kidney injury (adjusted odds ratio, 1.2 [95% CI, 1.0-1.5]; p = 0.03). Higher vasoactive-inotropic score on postoperative day 0 was associated with a higher risk for stage 2/3 acute kidney injury (adjusted odds ratio, 1.9 [95% CI, 1.0-3.4]; p = 0.04). Stage 2/3 acute kidney injury was associated with longer cardiovascular ICU length of stay (mean, 7.3 greater d [95% CI, 3.4-11.3]; p < 0.001) and hospital postoperative length of stay (mean, 6.4 greater d [95% CI, 0.06-12.5]; p = 0.04).Postoperative acute kidney injury in patients undergoing the extracardiac Fontan operation is common and is associated with lower postoperative renal perfusion pressure and higher vasoactive-inotropic score. Cardiopulmonary bypass was strongly associated with any acute kidney injury, although not stage 2/3 acute kidney injury. Stage 2/3 acute kidney injury is a compelling risk factor for longer cardiovascular ICU and hospital postoperative length of stay. Increased attention to and management of renal perfusion pressure may reduce postoperative acute kidney injury and improve outcomes.

  View details for PubMedID 27792123

• Prevalence and Anatomy of Retroesophageal Major Aortopulmonary Collateral Arteries. Annals of thoracic surgery Mainwaring, R. D., Patrick, W. L., Carrillo, S. A., Ibrahimye, A. N., Muralidaran, A., Hanley, F. L. 2016; 102 (3): 877-882

  Abstract

  Major aortopulmonary collateral arteries (MAPCAs) are the sole source of pulmonary blood flow in patients with pulmonary atresia and absent ductus arteriosus. The anatomy of MAPCAs can be highly variable, both in the number of MAPCAs supplying each lung and the anatomic origin and course of the MAPCAs. This study evaluated the prevalence and anatomy of retroesophageal MAPCAs in patients undergoing repair of pulmonary atresia/ventricular septal defect/MAPCAs.This was a concurrent analysis of 68 consecutive patients (March 2013 through October 2015) undergoing a primary surgical procedure for pulmonary atresia/ventricular septal defect/MAPCAs. A detailed analysis of the MAPCA anatomy was made intraoperatively for each patient, including the total number of MAPCAs to each lung and the presence or absence of a retroesophageal course. These data were correlated with the preoperative cardiac catheterization images.A retroesophageal MAPCA was identified during the operation in 45 of the 68 patients (67%), all of which were located on the side opposite the arch. For the 36 patients with a left aortic arch, 77% had a retroesophageal MAPCA compared with 53% of patients with a right arch. Forty-six percent of retroesophageal MAPCAs coursed within the muscular fibers of the esophagus (intraesophageal) and were more common to the left lung than the right (72% vs 32%). A midsegment stenosis was present in 84% of the retroesophageal MAPCAs, and this was more severe when the MAPCAs were intraesophageal than when they were not (80% vs 42%).These data demonstrate that two-thirds of patients had a retroesophageal MAPCA and that there were significant differences in prevalence and anatomy depending on the side of the aortic arch. These data provide important insights into the origin and course of retroesophageal MAPCAs.

  View details for DOI 10.1016/j.athoracsur.2016.03.011

  View details for PubMedID 27209604

• Right Ventricular Outflow Tract Obstruction: Pulmonary Atresia With Intact Ventricular Septum, Pulmonary Stenosis, and Ebstein's Malformation. Pediatric critical care medicine Kwiatkowski, D. M., Hanley, F. L., Krawczeski, C. D. 2016; 17 (8): S323-9

  Abstract

  The objectives of this review are to discuss the anatomy, pathophysiology, clinical course, and current treatment strategies for pulmonary atresia with intact ventricular septum, pulmonary stenosis, and Ebstein's anomaly.MEDLINE and PubMed.Considerable advances have been made in management strategies for these complex congenital heart lesions, which have led to improved outcomes.

  View details for DOI 10.1097/PCC.0000000000000818

  View details for PubMedID 27490618

• Surgical Techniques for Repair of Peripheral Pulmonary Artery Stenosis. Seminars in thoracic and cardiovascular surgery Mainwaring, R. D., Hanley, F. L. 2016; 28 (2): 418-424

  Abstract

  Peripheral pulmonary artery stenosis (PPAS) is a rare form of congenital heart disease that is most frequently associated with Williams and Alagille syndromes. These patients typically have systemic level right ventricular pressures secondary to obstruction at the lobar, segmental, and subsegmental branches. The current management of patients with PPAS remains somewhat controversial. We have pioneered an entirely surgical approach for the reconstruction of PPAS. This approach initially entailed a surgical patch augmentation of all major lobar branches and effectively reduced the right ventricular pressures by more than half. This was the first report demonstrating an effective approach to this disease. Over the past 5 years, we have gradually evolved our technique of reconstruction to include segmental and subsegmental branch stenoses. An important technical aspect of this approach entails the division of the main pulmonary and separation of the branch pulmonary arteries to access the lower lobe branches. Pulmonary artery homograft patches are used to augment hypoplastic pulmonary artery branches. In addition, we perform a Heinecke-Miculicz-type ostioplasty for isolated ostial stenoses. The technical details of the surgical approach to PPAS are outlined in this article and can also be used for other complex peripheral pulmonary artery reconstructions.

  View details for DOI 10.1053/j.semtcvs.2016.07.003

  View details for PubMedID 28043454

• Pulmonary Valve Repair for Patients With Acquired Pulmonary Valve Insufficiency ANNALS OF THORACIC SURGERY Said, S. M., Mainwaring, R. D., Ma, M., Tacy, T. A., Hanley, F. L. 2016; 101 (6): 2294-2301

  Abstract

  Pulmonary valve (PV) insufficiency is often an acquired condition after treatment for pulmonary stenosis. It is recognized that PV insufficiency has serious deleterious effects. Although surgical replacement of the PV is efficacious, artificial valves inevitably fail and require re-intervention. The purpose of this study was to summarize our experience with PV repair in patients with acquired PV insufficiency.This was a retrospective review of 16 patients with marked PV insufficiency who underwent PV repair. Thirteen of these patients were born with tetralogy of Fallot (TOF) and had undergone a previous transannular patch repair. Three patients were born with critical pulmonary stenosis and had a surgical valvotomy or balloon valvuloplasty.The 13 patients with TOF had resection of their previously placed transannular patch with re-approximation of the anterior commissure. All 13 patients experienced a marked reduction in the degree of pulmonary insufficiency. None of these patients have experienced any increase in insufficiency during follow-up. The 3 patients with critical pulmonary stenosis had a variety of pathologic findings identified at the surgical procedure. One patient had a large gap between a commissure and underwent closure of that commissure. The second and third patients had torn leaflets repaired with pericardial and Gore-Tex patches (Gore, Inc, Flagstaff, AZ). The degree of PV insufficiency was decreased to mild in all 3 patients. However, 2 of these 3 patients have subsequently had an increase in the degree of pulmonary insufficiency.Patients with TOF who underwent a previous transannular patch may be candidates for bicuspidization of their native PV, and the results of this procedure have been quite stable at follow-up. PV repair for torn leaflets was effective in the short term but was less stable over time.

  View details for DOI 10.1016/j.athoracsur.2016.01.035

  View details for Web of Science ID 000376502600043

  View details for PubMedID 27083251

• Postoperative Outcomes of Children With Tetralogy of Fallot, Pulmonary Atresia, and Major Aortopulmonary Collaterals Undergoing Reconstruction of Occluded Pulmonary Artery Branches ANNALS OF THORACIC SURGERY Asija, R., Koth, A. M., Velasquez, N., Chan, F. P., Perry, S. B., Hanley, F. L., McElhinney, D. 2016; 101 (6): 2329-2334

  Abstract

  Patients with tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collaterals (TOF/PA/MAPCAs) undergoing unifocalization surgery are at risk for prolonged postoperative respiratory failure. We sought to understand whether patients undergoing reconstruction and incorporation of occluded pulmonary arterial branches were at risk for worse postoperative outcomes.We performed a retrospective chart review to identify patients who underwent unifocalization or unifocalization revision with incorporation of occluded pulmonary artery branches. Patients with and without occluded branches were compared, with a focus on clinical outcomes.We studied 92 patients who underwent unifocalization procedures between 2010 and 2014, 17 (18%) of whom underwent reconstruction of occluded pulmonary artery branches. Patients with occluded vessels were more likely to require staged unifocalization procedures, although more than two thirds of this cohort eventually underwent complete intracardiac repair. Durations of mechanical ventilation, intensive care, hospital stay, and the need for early reoperation were similar between the two groups.Occluded pulmonary arterial branches can be safely recruited into the pulmonary vasculature in patients with TOF/PA/MAPCAs without a significant difference in postoperative outcomes compared with patients who did not have an occluded branch. Incorporation of occluded branches may also facilitate ultimate complete intracardiac repair in this complex population of patients.

  View details for DOI 10.1016/j.athoracsur.2015.12.049

  View details for PubMedID 26947013

• Surgical Repair of 115 Patients With Anomalous Aortic Origin of a Coronary Artery From a Single Institution. World journal for pediatric & congenital heart surgery Mainwaring, R. D., Murphy, D. J., Rogers, I. S., Chan, F. P., Petrossian, E., Palmon, M., Hanley, F. L. 2016; 7 (3): 353-359

  Abstract

  Anomalous aortic origin of a coronary artery (AAOCA) has been associated with myocardial ischemia and sudden death. The past decade has provided important insights into the natural history and typical patterns of presentation. However, there are also a number of unresolved controversies regarding the indications for surgery and the efficacy of that surgery. The purpose of this study was to review our surgical experience with AAOCA in 115 patients at a single institution.One hundred and fifteen patients have undergone surgical repair of AAOCA at our institution. There were 82 males and 33 females, and the median age at surgery was 16 years. Fifty-nine patients had preoperative symptoms of myocardial ischemia, including 56 with exertional chest pain or syncope and 3 sudden death events. Twenty-four patients had associated congenital heart defects. Seven patients had an associated myocardial bridge.Surgical repair was accomplished by unroofing of an intramural coronary in 86, reimplantation in 9, and pulmonary artery translocation in 20. There has been no early or late mortality. Fifty-seven (97%) of the 59 symptomatic patients have been free of any cardiac symptoms postoperatively. Two patients had recurrent symptoms and underwent reoperation (one had revision of the initial repair and one had repair of a myocardial bridge).Surgical repair of AAOCA can be safely performed and is highly efficacious in relieving symptoms of myocardial ischemia. The two "surgical failures" in this series had an anatomic basis and underscore the need to reassess both the proximal and distal anatomy in these patients.

  View details for DOI 10.1177/2150135116641892

  View details for PubMedID 27142404

• Exploring the Role of Polycythemia in Patients With Cyanosis After Palliative Congenital Heart Surgery. Pediatric critical care medicine Siehr, S. L., Shi, S., Hao, S., Hu, Z., Jin, B., Hanley, F., Reddy, V. M., McElhinney, D. B., Ling, X. B., Shin, A. Y. 2016; 17 (3): 216-222

  Abstract

  To understand the relationship between polycythemia and clinical outcome in patients with hypoplastic left heart syndrome following the Norwood operation.A retrospective, single-center cohort study.Pediatric cardiovascular ICU, university-affiliated children's hospital.Infants with hypoplastic left heart syndrome admitted to our medical center from September 2009 to December 2012 undergoing stage 1/Norwood operation.None.Baseline demographic and clinical information including first recorded postoperative hematocrit and subsequent mean, median, and nadir hematocrits during the first 72 hours postoperatively were recorded. The primary outcomes were in-hospital mortality and length of hospitalization. Thirty-two patients were included in the analysis. Patients did not differ by operative factors (cardiopulmonary bypass time and cross-clamp time) or traditional markers of severity of illness (vasoactive inotrope score, lactate, saturation, and PaO2/FIO2 ratio). Early polycythemia (hematocrit value > 49%) was associated with longer cardiovascular ICU stay (51.0 [± 38.6] vs 21.4 [± 16.2] d; p < 0.01) and total hospital length of stay (65.0 [± 46.5] vs 36.1 [± 20.0] d; p = 0.03). In a multivariable analysis, polycythemia remained independently associated with the length of hospitalization after controlling for the amount of RBC transfusion (weight, 4.36 [95% CI, 1.35-7.37]; p < 0.01). No difference in in-hospital mortality rates was detected between the two groups (17.6% vs 20%).Early polycythemia following the Norwood operation is associated with longer length of hospitalization even after controlling for blood cell transfusion practices. We hypothesize that polycythemia may be caused by hemoconcentration and used as an early marker of capillary leak syndrome.

  View details for DOI 10.1097/PCC.0000000000000654

  View details for PubMedID 26825044

• Mitral Stenosis and Aortic Atresia-A Risk Factor for Mortality After the Modified Norwood Operation in Hypoplastic Left Heart Syndrome ANNALS OF THORACIC SURGERY Siehr, S. L., Maeda, K., Connolly, A. A., Tacy, T. A., Reddy, V. M., Hanley, F. L., Perry, S. B., Wright, G. E. 2016; 101 (1): 162-168

  Abstract

  There are conflicting reports regarding the importance of mitral stenosis and aortic atresia as a risk factor for Norwood mortality. This study reviews outcomes of this anatomic subgroup at our institution and examines the utility of preoperative cardiac catheterization and its correlation with clinical outcomes and pathology findings.This is a single-center, retrospective review of hypoplastic left heart syndrome patients who underwent modified Norwood operation between October 2005 and May 2013.Fourteen of 74 hypoplastic left heart syndrome patients (19%) had mitral stenosis and aortic atresia. Operative mortality for MS/AA was 29% versus 7% for all other hypoplastic left heart syndrome anatomic subgroups (p = 0.04). Although only 19% of the entire cohort, the mitral stenosis and aortic atresia subgroup constituted 50% of the total operative mortality and the only interstage deaths. Autopsies support myocardial ischemia as the mechanism of death. Although preoperative angiography defined the presence of ventriculo-coronary connections, it did not clearly risk stratify patients in regard to operative mortality.Mitral stenosis and aortic atresia is a risk factor for perioperative myocardial ischemia and mortality. Further exploration of myocardial reserve is warranted.

  View details for DOI 10.1016/j.athoracsur.2015.09.056

  View details for Web of Science ID 000368184300032

• Critical Role of Coaptive Strain in Aortic Valve Leaflet Homeostasis: Use of a Novel Flow Culture Bioreactor to Explore Heart Valve Mechanobiology. Journal of the American Heart Association Maeda, K., Ma, X., Hanley, F. L., Riemer, R. K. 2016; 5 (8)

  Abstract

  Aortic valve (AV) disease presents critical situations requiring surgery in over 2% of the US population and is increasingly the reason for cardiac surgery. Throughout the AV cycle, mechanical forces of multiple types and varying intensities are exerted on valve leaflets. The mechanisms whereby forces regulate leaflet homeostasis are incompletely understood. We used a novel flow bioreactor culture to investigate alteration of AV opening or closure on leaflet genes.Culture of rat AV was conducted in a flow bioreactor for 7 days at 37°C under conditions approximating the normal stroke volume. Three force condition groups were compared: Cycling (n=8); always open (Open; n=3); or always closed (Closed; n=5). From each culture, AV leaflets were pooled by force condition and RNA expression evaluated using microarrays. Hierarchical clustering of 16 transcriptome data sets from the 3 groups revealed only 2 patterns of gene expression: Cycling and Closed groups clustered together, whereas Open AV were different (P<0.05). Sustained AV opening induced marked changes in expression (202 transcripts >2-fold; P<0.05), whereas Closed AV exhibited similar expression pattern as Cycling (no transcripts >2-fold; P<0.05). Comparison with human sclerotic and calcific AV transcriptomes demonstrated high concordance of >40 Open group genes with progression toward disease.Failure of AV to close initiates an extensive response characterized by expression changes common to progression to calcific aortic valve disease. AV coaptation, whether phasic or chronic, preserved phenotypic gene expression. These results demonstrate, for the first time, that coaptation of valve leaflets is a fundamentally important biomechanical cue driving homeostasis.

  View details for DOI 10.1161/JAHA.116.003506

  View details for PubMedID 27464792

• Anatomic Factors Associated With Truncal Valve Insufficiency and the Need for Truncal Valve Repair. World journal for pediatric & congenital heart surgery Patrick, W. L., Mainwaring, R. D., Carrillo, S. A., Ma, M., Reinhartz, O., Petrossian, E., Selamet Tierney, E. S., Reddy, V. M., Hanley, F. L. 2016; 7 (1): 9-15

  Abstract

  Truncus arteriosus is a complex and heterogeneous form of congenital heart defect. Many of the risk factors from several decades ago, including late repair and interrupted aortic arch, have been mitigated through better understanding of the entity and improved surgical techniques. However, truncal valve dysfunction remains an important cause of morbidity and mortality. The purpose of this study was to evaluate the anatomic factors associated with truncal valve dysfunction and the need for truncal valve surgery.This was a retrospective review of 72 infants who underwent repair of truncus arteriosus at our institution. The median age at surgery was nine days, and the median weight was 3.1 kg. Preoperative assessment of truncal valve insufficiency by echocardiography revealed no or trace insufficiency in 30, mild in 25, moderate in 10, and severe in 7. The need for truncal valve surgery was dictated by the severity of truncal valve insufficiency.Sixteen (22%) of the 72 patients undergoing truncus arteriosus repair had concomitant truncal valve surgery. Anatomic factors associated with the need for truncal valve surgery included an abnormal number of truncal valve cusps (P < .005), presence of valve dysplasia (P < .005), and the presence of an anomalous coronary artery pattern (P < .005). Fifteen (94%) of the sixteen patients who underwent concomitant surgery had two or all three of these anatomic factors (sensitivity = 94%, specificity = 85%).This study demonstrates that the presence of specific anatomic factors was closely associated with the presence of truncal valve insufficiency and the need for concomitant truncal valve surgery. Preoperative evaluation of these anatomic factors may provide a useful tool in determining who should undergo concomitant truncal valve surgery.

  View details for DOI 10.1177/2150135115608093

  View details for PubMedID 26714988

• Mitral Stenosis and Aortic Atresia-A Risk Factor for Mortality After the Modified Norwood Operation in Hypoplastic Left Heart Syndrome. Annals of thoracic surgery Siehr, S. L., Maeda, K., Connolly, A. A., Tacy, T. A., Reddy, V. M., Hanley, F. L., Perry, S. B., Wright, G. E. 2016; 101 (1): 162-167

  Abstract

  There are conflicting reports regarding the importance of mitral stenosis and aortic atresia as a risk factor for Norwood mortality. This study reviews outcomes of this anatomic subgroup at our institution and examines the utility of preoperative cardiac catheterization and its correlation with clinical outcomes and pathology findings.This is a single-center, retrospective review of hypoplastic left heart syndrome patients who underwent modified Norwood operation between October 2005 and May 2013.Fourteen of 74 hypoplastic left heart syndrome patients (19%) had mitral stenosis and aortic atresia. Operative mortality for MS/AA was 29% versus 7% for all other hypoplastic left heart syndrome anatomic subgroups (p = 0.04). Although only 19% of the entire cohort, the mitral stenosis and aortic atresia subgroup constituted 50% of the total operative mortality and the only interstage deaths. Autopsies support myocardial ischemia as the mechanism of death. Although preoperative angiography defined the presence of ventriculo-coronary connections, it did not clearly risk stratify patients in regard to operative mortality.Mitral stenosis and aortic atresia is a risk factor for perioperative myocardial ischemia and mortality. Further exploration of myocardial reserve is warranted.

  View details for DOI 10.1016/j.athoracsur.2015.09.056

  View details for PubMedID 26602002

• Surgical Repair of Pulmonary Atresia With Ventricular Septal Defect and Major Aortopulmonary Collaterals With Absent Intrapericardial Pulmonary Arteries ANNALS OF THORACIC SURGERY Carrillo, S. A., Mainwaring, R. D., Patrick, W. L., Bauser-Heaton, H. D., Peng, L., Reddy, V. M., Hanley, F. L. 2015; 100 (2): 606-614

  Abstract

  One anatomic variant of pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals (PA/VSD/MAPCAs) is characterized by the absence of intrapericardial pulmonary arteries. This anatomy obviates the possibility of incorporating the pulmonary arteries for reconstruction or palliative procedures. The purpose of this study was to evaluate the surgical results in patients undergoing repair of PA/VSD/MAPCAs with absent pulmonary arteries.This was a retrospective review of 35 patients who underwent surgical repair of PA/VSD/MAPCAs with absent pulmonary arteries between 2007 and 2014. The median age at the time of surgery was 3.4 months, and the median weight was 4.9 kg. All patients underwent unifocalization of MAPCAs, with an average of 3.5 ± 1.4 MAPCAs per patient.Twenty-eight of the 35 patients (80%) underwent complete single-stage surgical repair, including unifocalization of MAPCAs, VSD closure, and right ventricle to pulmonary artery conduit. After complete repair, the average right ventricular to aortic pressure ratio was 0.33 ± 0.07. There were no deaths in this subgroup. Seven patients (20%) were not deemed suitable candidates for VSD closure after their unifocalization procedure, and therefore underwent palliation with a central shunt. There was 1 operative death and 1 interim death. Three patients have subsequently undergone complete repair, and 2 are awaiting further evaluation and treatment.The majority of patients with PA/VSD/MAPCAs and absent pulmonary arteries can undergo complete single-stage repair with satisfactory postoperative hemodynamics. These results suggest that unifocalization of MAPCAs can provide a reasonable pulmonary vascular bed in the absence of intrapericardial pulmonary arteries.

  View details for DOI 10.1016/j.athoracsur.2015.03.110

  View details for PubMedID 26138766

• Left Ventricular Retraining and Late Arterial Switch for D-Transposition of the Great Arteries. Annals of thoracic surgery Watanabe, N., Mainwaring, R. D., Carrillo, S. A., Lui, G. K., Reddy, V. M., Hanley, F. L. 2015; 99 (5): 1655-1661

  Abstract

  For many decades, patients with d-transposition of the great arteries underwent an atrial switch procedure. Although many of these patients have continued to do well, a subset experience profound right ventricular failure. Some may be candidates for left ventricular (LV) retraining and late arterial switch. The purpose of this study was to review our experience with LV retraining and late arterial switch.This was a retrospective review of 32 patients with d-transposition. Thirty patients underwent a previous atrial switch and subsequently experienced right ventricular failure, whereas 2 presented late (8 months and 6 years) without previous intervention. The median age at the time of enrollment in this program was 15 years. Seven patients proceeded directly to late arterial switch owing to systemic LV pressures. The remaining 25 underwent a pulmonary artery band for LV retraining.Twenty of the 32 (63%) patients enrolled in this program were able to undergo a late arterial switch. There were 2 operative mortalities (10%). Two additional patients survived surgery but died in the early outpatient time period. There has been no late mortality after the arterial switch with a median follow-up of 5 years. Twelve patients underwent one or more pulmonary artery band procedures without evidence of effective LV retraining. There have been 2 early and 3 late (42%) deaths in this subgroup.The outcomes after arterial switch are encouraging and suggest that LV retraining and late arterial switch provide a viable option for this complex group of patients.

  View details for DOI 10.1016/j.athoracsur.2014.12.084

  View details for PubMedID 25817887

• Fate of Right Ventricle to Pulmonary Artery Conduits After Complete Repair of Pulmonary Atresia and Major Aortopulmonary Collaterals ANNALS OF THORACIC SURGERY Mainwaring, R. D., Patrick, W. L., Punn, R., Palmon, M., Reddy, V. M., Hanley, F. L. 2015; 99 (5): 1685-1691

  Abstract

  Surgical repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals (PA/VSD/MAPCAs) requires insertion of a conduit from the right ventricle to the reconstructed pulmonary arteries. Although there is extensive literature on conduit longevity for many forms of congenital heart defects, there is currently limited information for PA/VSD/MAPCAs. It is likely that conduits will have a different longevity in PA/VSD/MAPCAs because pulmonary artery pressures are higher after unifocalization than in other congenital heart defects. The purpose of this study was to evaluate the fate of right ventricle to pulmonary artery conduits after complete repair of PA/VSD/MAPCAs.This was a retrospective review of 103 infants who underwent complete repair of PA/VSD/MAPCAs with insertion of an aortic homograft conduit. The average age at complete repair was 5.1 ± 2.8 months, average conduit size was 13 ± 2 mm, and average conduit z score was a positive 1.6 ± 0.9.Conduit z scores were linearly correlated with increasing homograft conduit size. The average longevity of small homografts (8 to 11 mm) was 15 ± 4 months, medium homografts (12 to 14 mm) was 39 ± 7 months, and large homografts (15 to 17 mm) was 50 ± 14 months. There was a negative correlation between increasing pulmonary artery pressure and conduit longevity.Conduit z scores and conduit size worked synergistically to predict the longevity of right ventricle to pulmonary artery conduits after complete repair of PA/VSD/MAPCAs. Pulmonary artery pressure was an additional modifier of longevity. These data provide a framework for expectations of aortic homograft conduits used in the repair of PA/VSD/MAPCAs.

  View details for DOI 10.1016/j.athoracsur.2014.12.071

  View details for PubMedID 25805573

• Left Ventricular Retraining and Late Arterial Switch for D-Transposition of the Great Arteries ANNALS OF THORACIC SURGERY Watanabe, N., Mainwaring, R. D., Carrillo, S. A., Lui, G. K., Reddy, V. M., Hanley, F. L. 2015; 99 (5): 1655-1663

  Abstract

  For many decades, patients with d-transposition of the great arteries underwent an atrial switch procedure. Although many of these patients have continued to do well, a subset experience profound right ventricular failure. Some may be candidates for left ventricular (LV) retraining and late arterial switch. The purpose of this study was to review our experience with LV retraining and late arterial switch.This was a retrospective review of 32 patients with d-transposition. Thirty patients underwent a previous atrial switch and subsequently experienced right ventricular failure, whereas 2 presented late (8 months and 6 years) without previous intervention. The median age at the time of enrollment in this program was 15 years. Seven patients proceeded directly to late arterial switch owing to systemic LV pressures. The remaining 25 underwent a pulmonary artery band for LV retraining.Twenty of the 32 (63%) patients enrolled in this program were able to undergo a late arterial switch. There were 2 operative mortalities (10%). Two additional patients survived surgery but died in the early outpatient time period. There has been no late mortality after the arterial switch with a median follow-up of 5 years. Twelve patients underwent one or more pulmonary artery band procedures without evidence of effective LV retraining. There have been 2 early and 3 late (42%) deaths in this subgroup.The outcomes after arterial switch are encouraging and suggest that LV retraining and late arterial switch provide a viable option for this complex group of patients.

  View details for DOI 10.1016/j.athoracsur.2014.12.084

  View details for Web of Science ID 000353877900040

  View details for PubMedID 25817887

• Contemporaneous comparison of the Yasui and Norwood procedures at a single institution. journal of thoracic and cardiovascular surgery Carrillo, S. A., Mainwaring, R. D., Schaffer, J. M., Wright, G., Maeda, K., Hanley, F. L., Reddy, V. M. 2015; 149 (2): 508-513

  Abstract

  It is recognized that there are numerous anatomic variants that result in hypoplastic left heart physiology. One such variant includes critical aortic stenosis or atresia, a hypoplastic aortic arch, and a reasonably well-developed left ventricle due to the presence of a ventricular septal defect. These patients are candidates for 1 of 3 surgical options: (1) a Norwood procedure followed by a single-ventricle pathway; (2) a Norwood procedure followed by a Rastelli procedure (2-stage Yasui); or (3) a single-stage Yasui procedure. Because 2 of the 3 options include a Norwood procedure as the initial step, the purpose of this study was to evaluate the contemporaneous results of the Yasui and Norwood procedures at a single institution.This was a retrospective review of patients who underwent a Yasui or Norwood procedure at Lucile Packard Children's Hospital between 2004 and 2013. Eighteen patients underwent a Yasui, of whom 15 had a single-stage procedure and 3 had a 2-stage procedure. During this time frame, 113 patients underwent a Norwood procedure. Kaplan-Meier survival curves and freedom from reoperation were compared for the 2 procedures.The operative mortality (using the Society of Thoracic Surgeons definition) for the single-stage Yasui was 6.7% compared with 16% for the Norwood procedure (P < .05); survival was 85% versus 62% at 1 year, 85% versus 60% at 3 years, and 85% versus 58% at 5 years, respectively (log-rank P = .06). The average interval to first reoperation was 13.5 ± 3 months versus 4.5 ± 1 months for the Yasui and Norwood procedures, respectively (P < .001).The Yasui procedure had a significantly lower operative mortality compared with the Norwood procedure. Early and midterm survival was also higher in the Yasui group versus the Norwood followed by a single ventricle pathway. These results indicate that the Yasui procedure has significant midterm benefits compared with the Norwood procedure and should be pursued when the anatomy is amenable for this approach.

  View details for DOI 10.1016/j.jtcvs.2014.09.120

  View details for PubMedID 25451485

• Are preoperative B-type natriuretic peptide levels associated with outcome after pulmonary artery banding and the double switch operation in patients with congenitally corrected transposition of the great arteries: A pilot study JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY Char, D. S., Shiboski, S. C., Hanley, F. L., Fineman, J. R. 2014; 148 (5): 2434-2436

  View details for DOI 10.1016/j.jtcvs.2014.07.003

  View details for Web of Science ID 000345132600141

• Reperfusion pulmonary edema in children with tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collateral arteries undergoing unifocalization procedures: A pilot study examining potential pathophysiologic mechanisms and clinical significance JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY Asija, R., Roth, S. J., Hanley, F. L., Peng, L., Liu, K., Abbott, J., Zhuo, H., Matthay, M. 2014; 148 (4): 1560-1565

  Abstract

  Children with tetralogy of Fallot (TOF), pulmonary atresia (PA), and major aortopulmonary collateral arteries (MAPCAs) are at risk for reperfusion pulmonary edema (RPE) after unifocalization procedures to reconstruct the central pulmonary arteries. The purpose of this study was to determine the incidence of RPE, describe the clinical course of patients with RPE, and explore the mechanism of RPE in this population by measuring plasma biomarkers of alveolar epithelial and endothelial injury and lung inflammation.Levels of plasma receptor for advanced glycation end products (RAGE), intercellular adhesion molecule 1 (ICAM-1), and interleukin 6 (IL-6) were measured at baseline and postoperative day (POD) 0, 1, and 2 after unifocalization. A pediatric radiologist reviewed chest radiographs from the same time points and scored each lung segment for the degree of pulmonary edema. A pediatric interventional cardiologist reviewed the preoperative angiograms for each patient and determined the degree of stenosis for each aortopulmonary collateral vessel. RPE was defined as localized pulmonary edema with a pulmonary edema score of at least 2 occurring in the lung segment demonstrating the greatest degree of angiographic stenosis within the first 48 hours after surgery and with resolution by discharge.Thirty-five patients who underwent 37 unifocalization procedures were enrolled, and 32 patients were included in the analysis. Of these, 16 of 32 (50%) demonstrated evidence of RPE based on our defined criteria. There was no significant difference in RAGE (P=.60), ICAM-1 (P=.34), or IL-6 (P=.31) levels between those with and without RPE at any time point. The mean duration of mechanical ventilation in patients with RPE versus those without was not significantly different (5.1±4.2 vs 5.6±4.5 days, respectively; P=.57).Fifty percent of children with TOF/PA/MAPCAs undergoing unifocalization surgery developed RPE. Levels of plasma biomarkers of alveolar epithelial and endothelial injury and lung inflammation were not increased in patients with RPE compared with those without RPE. The presence of RPE did not affect the duration of respiratory failure and mechanical ventilation. The process of RPE is clinically self-limited and seems unlikely to be associated with vascular changes.

  View details for DOI 10.1016/j.jtcvs.2014.01.017

  View details for Web of Science ID 000342896200077

• Tetralogy of Fallot: aorto-pulmonary collaterals and pulmonary arteries have distinctly different transcriptomes PEDIATRIC RESEARCH Ma, X., Barboza, L. A., Siyahian, A., Reinhartz, O., Maeda, K., Reddy, V. M., Hanley, F. L., Riemer, R. K. 2014; 76 (4): 341-346

  Abstract

  Tetralogy of Fallot patients with pulmonary atresia (TOF/PA) present a pulmonary blood supply directly from aortic collateral arteries. Major aorto-pulmonary collateral arteries (MAPCAs) present substantial clinical and surgical management challenges. Surgical operations to reestablish and promote further development of a pulmonary arterial connection preferentially utilize MAPCAs for reconstruction of central pulmonary arteries. However, the propensity of some MAPCAs to develop stenosis rather than growth may impair the response to reconstructions.Probe sets prepared from MAPCAs, PA, and aorta mRNA were used to interrogate human genome microarrays. We compared expression differences between pairs of the three vessels to determine whether MAPCAs display distinct expression patterns.Functional clustering analysis identified differences in gene expression, which were further analyzed by gene ontology classification. A subset of highly regulated genes was validated using quantitative PCR. Expression differences among vessel types were observed for multiple gene classes. Of note, we observed that MAPCAs differentially express several genes at much higher levels than either PA or aorta.MAPCAs differ from PA or aorta by significantly altered levels in gene expression, suggesting a transcriptional basis for their physiology that will guide a further understanding of the pathobiology of MAPCAs and TOF.

  View details for DOI 10.1038/pr.2014.101

  View details for Web of Science ID 000342332000003

• Coronary sinus obstruction after atrioventricular canal defect repair. Congenital heart disease Peng, D. M., Sun, H. Y., Hanley, F. L., Olson, I., Punn, R. 2014; 9 (4): E121-4

  Abstract

  The coronary sinus can become obstructed with any instrumentation at or near the ostium such as in atrioventricular canal defect repairs. This complication may lead to a wide range of consequences including dyspnea, angina, myocardial infarction, and sudden death. The following report illustrates the importance of careful perioperative echocardiographic evaluation of the coronary sinus in procedures that may affect the sinus and its ostium.

  View details for DOI 10.1111/chd.12096

  View details for PubMedID 23682752

• Completion of the Three-Stage Fontan Pathway Without Cardiopulmonary Bypass. World journal for pediatric & congenital heart surgery Mainwaring, R. D., Reddy, V. M., Hanley, F. L. 2014; 5 (3): 427-433

  Abstract

  The three-stage surgical approach is now accepted as the standard for management of children born with functional single ventricle. However, there is little consensus on the cardiopulmonary bypass strategies employed for these procedures. We have attempted to avoid cardiopulmonary bypass in patients with single ventricle whenever possible to eliminate the adverse effects that are induced by this process. The purpose of this study was to review our experience in patients who underwent all three stages of the Fontan pathway without ever being exposed to bypass.A total of 52 patients with single ventricle underwent "off-pump" treatment at all three stages of their surgical management. The time period of the study was from 2002 to 2013. There were 31 males and 21 females. Anatomic diagnoses included double inlet left ventricle (n = 11), pulmonary atresia with intact ventricular septum (n = 11), tricuspid atresia (n = 10), double outlet right ventricle (n = 9), and other (n = 11).There was no operative mortality in the 52 patients undergoing Fontan completion. The patients have been followed for an average of 5.1 ± 2.5 years, with one late mortality. The median length of hospital stay for the three stages was 17, 5, and 9 days, respectively. Of the 52 patients, 42 were able to undergo all three stages without the need for a blood transfusion.This series demonstrates the feasibility of achieving a Fontan circulation without patients exposed to cardiopulmonary bypass. There was no operative mortality and low mid-term mortality. It is notable that 80% of patients never required a blood transfusion with this approach. The elimination of cardiopulmonary bypass provides several potential clinical benefits in this highly select subset of patients with single ventricle.

  View details for DOI 10.1177/2150135114536908

  View details for PubMedID 24958046

• Preoperative B-type natriuretic peptide levels are associated with outcome after total cavopulmonary connection (Fontan). journal of thoracic and cardiovascular surgery Radman, M., Keller, R. L., Oishi, P., Datar, S. A., Wellnitz, K., Azakie, A., Hanley, F., Char, D., Hsu, J., Amrinovin, R., Adatia, I., Fineman, J. R. 2014; 148 (1): 212-219

  Abstract

  The study objective was to determine the association between preoperative B-type natriuretic peptide levels and outcome after total cavopulmonary connection. Surgical palliation of univentricular cardiac defects requires a series of staged operations, ending in a total cavopulmonary connection. Although outcomes have improved, there remains an unpredictable risk of early total cavopulmonary connection takedown. The prediction of adverse postoperative outcomes is imprecise, despite an extensive preoperative evaluation.We prospectively enrolled 50 patients undergoing total cavopulmonary connection. We collected preoperative clinical data, preoperative plasma B-type natriuretic peptide levels, and postoperative outcomes, including the incidence of an adverse outcome within 1 year of surgery (defined as death, total cavopulmonary connection takedown, or the need for cardiac transplantation).The mean age of patients was 4.7 years (standard deviation, 2.1 years). The median (interquartile range) preoperative B-type natriuretic peptide levels were higher in patients who required total cavopulmonary connection takedown and early postoperative mechanical cardiac support (n = 3; median, 55; interquartile range, 42-121) compared with those with a good outcome (n = 47; median, 11; interquartile range, 5-17) (P < .05). A preoperative B-type natriuretic peptide level of 40 pg/mL or greater was highly associated with the need for total cavopulmonary connection takedown (sensitivity, 100%; specificity, 93%; P < .05), yielding a positive predictive value of 50% and a negative predictive value of 100%. Higher preoperative B-type natriuretic peptide levels also were associated with longer intensive care unit length of stay, longer hospital length of stay, and increased incidence of low cardiac output syndrome (P < .05).Preoperative B-type natriuretic peptide blood levels are uniquely associated with the need for mechanical support early after total cavopulmonary connection and total cavopulmonary connection takedown, and thus may provide important information in addition to the standard preoperative assessment.

  View details for DOI 10.1016/j.jtcvs.2013.08.009

  View details for PubMedID 24079880

  View details for PubMedCentralID PMC3968237

• Surgical repair of anomalous aortic origin of a coronary artery†. European journal of cardio-thoracic surgery Mainwaring, R. D., Reddy, V. M., Reinhartz, O., Petrossian, E., Punn, R., Hanley, F. L. 2014; 46 (1): 20-26

  Abstract

  Anomalous aortic origin of a coronary artery (AAOCA) is a rare congenital heart defect that has been associated with myocardial ischaemia and sudden death. There is an ongoing controversy over the indications for surgical intervention and the efficacy of that treatment compared with the natural history. The purpose of this study was to evaluate the medium-term results of surgical repair of AAOCA.Seventy-six patients underwent surgical repair of AAOCA at our institution from 1999 to 2013. There were 55 males and 21 females, and the median age at surgery was 15 years. Forty-seven (62%) of the 76 patients had an anomalous right coronary artery, 27 had an anomalous left coronary and 2 had an eccentric single coronary ostia. Forty-one patients had preoperative symptoms of myocardial ischaemia.Surgical repair was accomplished by unroofing of an intramural coronary in 55, reimplantation in 7 and pulmonary artery translocation in 14. There has been no early or late mortality, with a median duration of follow-up of 6 years. One patient presented with severe myocardial ischaemia and subsequently underwent heart transplantation a year following AAOCA surgery. The remaining patients have all remained free of cardiac symptoms.The results of this study demonstrate two major principles. First, surgical repair of AAOCA is quite safe in centres that take care of a significant number of patients with this entity. Secondly, the surgery is highly effective in eliminating symptoms of myocardial ischaemia. The growing amount of data on postoperative patients suggests that surgical repair can prevent the adverse events seen in the untreated 'natural' history. Based on these observations, it is our current recommendation that all teenagers identified with AAOCA should undergo surgical repair.

  View details for DOI 10.1093/ejcts/ezt614

  View details for PubMedID 24431169

• Incidence and Risk Factors of Complete Atrioventricular Block after Operative Ventricular Septal Defect Repair CONGENITAL HEART DISEASE Siehr, S. L., Hanley, F. L., Reddy, V. M., Miyake, C. Y., Dubin, A. M. 2014; 9 (3): 211-215

  Abstract

  BACKGROUND: Complete atrioventricular block (AVB) is a recognized complication of ventricular septal defect (VSD) repair. The objective of this study was to examine incidence and risk factors. METHODS: This is a single-center, retrospective study. All pediatric patients (age <18 years) who underwent VSD repair between November 2001 and August 2009 with concordant atrioventricular and ventriculoarterial connections were included. Patients were classified as having no AVB or transient AVB, and outcomes of early pacemaker placement (before hospital discharge) or late pacemaker placement (after hospital discharge) were recorded. RESULTS: Eight hundred twenty-eight patients (48.6% female) underwent VSD repair during the study period. Mean age at repair was 14 ± 29.4 months. A total of 64 patients (7.7%) developed surgical AVB. Among those patients who developed AVB, 48 (75%) had transient AVB. Sixteen patients (1.9%) required a pacemaker, 14 early (88%) and 2 late (12%). Patients <4 kg (4.2% vs. 1%, P ≤ .01) and those with inlet VSDs (11.6% vs. 1.4%, P ≤ .01) were more likely to develop surgical AVB. Surgical AVB was not influenced by the presence of chromosomal abnormalities or other congenital heart disease. CONCLUSION: The overall incidence of surgical AVB is consistent with previous reports. Weight <4 kg and presence of an inlet VSD were significant risk factors.

  View details for DOI 10.1111/chd.12110

  View details for Web of Science ID 000337721700012

• Early complete repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals. Annals of thoracic surgery Watanabe, N., Mainwaring, R. D., Reddy, V. M., Palmon, M., Hanley, F. L. 2014; 97 (3): 909-915

  Abstract

  Pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals (PA/VSD/MAPCAs) is a complex and diverse form of congenital heart defect. Although most patients with PA/VSD/MAPCAs can wait until they are 3 to 6 months of age to undergo surgical reconstruction, there are three specific criteria that merit an earlier repair. These 3 criteria are (1) unremitting heart failure; (2) a ductus to one lung and MAPCAs to the other; and (3) hemitruncus to one lung and MAPCAs to the other. The purpose of this study was to evaluate our surgical experience with early complete repair of PA/VSD/MAPCAs.This was a retrospective review of patients undergoing complete repair of PA/VSD/MAPCAs within the first 60 days of life. Twenty-seven patients were identified in our database (2002 to 2013) who met these criteria. Fifteen had congestive heart failure, 9 had a ductus plus MAPCAs, and 3 had hemitruncus plus MAPCAs. The median age at surgery was 5 weeks.There was no operative mortality in this cohort of 27 patients. Hemodynamics at the conclusion of the complete repair demonstrated an average right ventricular peak systolic pressure of 32 ± 5 mm Hg and an average right ventricle to aortic pressure ratio of 0.36 ± 0.06. The median length of hospital stay was 26 days. There have been 2 subsequent mortalities (7%), with a median follow-up duration of 4 years. Eight of the 27 patients have subsequently undergone conduit replacements at our institution. The hemodynamics at the conclusion of the conduit change were statistically unchanged compared with the hemodynamics after complete repair.The data demonstrate that early complete repair of PA/VSD/MAPCAs can be accomplished with low mortality and excellent postoperative hemodynamics. These early hemodynamic results are maintained at medium-term follow-up. We conclude that early complete repair is an appropriate choice for this highly select subgroup of patients.

  View details for DOI 10.1016/j.athoracsur.2013.10.115

  View details for PubMedID 24480261

• Home monitoring program reduces interstage mortality after the modified Norwood procedure. journal of thoracic and cardiovascular surgery Siehr, S. L., Norris, J. K., Bushnell, J. A., Ramamoorthy, C., Reddy, V. M., Hanley, F. L., Wright, G. E. 2014; 147 (2): 718-23 e1

  Abstract

  BACKGROUND: From 2002 to 2005, the interstage mortality after a modified Norwood procedure was 7% in our program. An interstage home monitoring program (HMP) was established to identify Norwood procedure patients at increased risk of decompensation and to reduce interstage mortality. METHODS: Results of the first 5 years of the Norwood HMP were reviewed retrospectively. Interstage was defined as the time between Norwood hospital discharge and admission for second stage surgical palliation. In the HMP, families documented oxygen saturation, heart rate, weight, and feedings daily. Nurse practitioners called each family at least weekly, and when issues arose, action plans were determined based on symptom severity. RESULTS: Between October 2005 and October 2010 there were 46 Norwood procedure patients who survived to hospital discharge. All were enrolled in the HMP. Forty-five patients had a Norwood procedure with right ventricle to pulmonary artery conduit, and 1 patient had a modified Blalock-Taussig shunt. Interstage survival was 100%. Nineteen patients (41%) were admitted interstage; 5 patients were admitted twice, 1 patient was admitted 4 times. Seventeen patients (37%) required interstage interventions. Eight patients (17%) required major interventions: conduit stenting, aortic arch balloon angioplasty, emergent shunt, or early Glenn surgery. Minor interventions included supplemental oxygen, blood transfusion, intravenous hydration, diuresis, anti-arrhythmic therapy, or feeding adjustments. CONCLUSIONS: In the first 5 years of the HMP, all infants discharged after a modified Norwood procedure survived the interstage period. The HMP altered clinical management in 37% of patients. Home monitoring of oxygen saturation, heart rate, weight, and feedings, along with comprehensive care coordination, allowed timely interventions and reduced interstage mortality from 7% to 0%.

  View details for DOI 10.1016/j.jtcvs.2013.04.006

  View details for PubMedID 23663957

• Home monitoring program reduces interstage mortality after the modified Norwood procedure JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY Siehr, S. L., Norris, J. K., Bushnell, J. A., Ramamoorthy, C., Reddy, V. M., Hanley, F. L., Wright, G. E. 2014; 147 (2): 718-?

  Abstract

  BACKGROUND: From 2002 to 2005, the interstage mortality after a modified Norwood procedure was 7% in our program. An interstage home monitoring program (HMP) was established to identify Norwood procedure patients at increased risk of decompensation and to reduce interstage mortality. METHODS: Results of the first 5 years of the Norwood HMP were reviewed retrospectively. Interstage was defined as the time between Norwood hospital discharge and admission for second stage surgical palliation. In the HMP, families documented oxygen saturation, heart rate, weight, and feedings daily. Nurse practitioners called each family at least weekly, and when issues arose, action plans were determined based on symptom severity. RESULTS: Between October 2005 and October 2010 there were 46 Norwood procedure patients who survived to hospital discharge. All were enrolled in the HMP. Forty-five patients had a Norwood procedure with right ventricle to pulmonary artery conduit, and 1 patient had a modified Blalock-Taussig shunt. Interstage survival was 100%. Nineteen patients (41%) were admitted interstage; 5 patients were admitted twice, 1 patient was admitted 4 times. Seventeen patients (37%) required interstage interventions. Eight patients (17%) required major interventions: conduit stenting, aortic arch balloon angioplasty, emergent shunt, or early Glenn surgery. Minor interventions included supplemental oxygen, blood transfusion, intravenous hydration, diuresis, anti-arrhythmic therapy, or feeding adjustments. CONCLUSIONS: In the first 5 years of the HMP, all infants discharged after a modified Norwood procedure survived the interstage period. The HMP altered clinical management in 37% of patients. Home monitoring of oxygen saturation, heart rate, weight, and feedings, along with comprehensive care coordination, allowed timely interventions and reduced interstage mortality from 7% to 0%.

  View details for DOI 10.1016/j.jtcvs.2013.04.006

  View details for Web of Science ID 000329724700031

  View details for PubMedID 23663957

• Atherosclerosis causing recurrent catastrophic aortopulmonary shunt dehiscence in a patient with Alagille syndrome. Pediatric cardiology May, L., Hanley, F. L., Connolly, A. J., Reddy, S. 2013; 34 (8): 1945-1948

  Abstract

  Alagille syndrome (ALGS) is an autosomal dominant disorder associated with cholestatic liver disease, pulmonary valvar stenosis or atresia, vasculopathy, and renal disease. Although the liver and cardiac manifestations contribute to overall morbidity and mortality during their life span, these patients also carry a burden of important but often underappreciated vascular abnormalities. This report describes a 3 year-old girl with Alagille syndrome, hepatic cholestasis, systemic hypertension, hypercholesterolemia, hypertriglyceridemia, and tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collaterals (TOF/PA/MAPCAs). She presented for bilateral pulmonary artery plasty and central shunt upsizing. She then experienced three shunt dehiscence episodes, necessitating emergent intervention. Autopsy showed diffuse atherosclerosis and significant atherosclerotic plaque at the site of shunt dehiscence. This is the first reported case of ALGS with TOF/PA/MAPCAs and catastrophic shunt dehiscence due to significant generalized vasculopathy caused by dyslipidemia and atherosclerosis. Dyslipidemia, a known comorbidity in ALGS, is one of few modifiable risk factors that should be screened for and treated, particularly before cardiac surgery.

  View details for DOI 10.1007/s00246-012-0484-4

  View details for PubMedID 22923029

• Innominate artery cannulation and antegrade cerebral perfusion for aortic arch reconstruction in infants and children. World journal for pediatric & congenital heart surgery Nasirov, T., Mainwaring, R. D., Reddy, V. M., Sleasman, J., Margetson, T., Hanley, F. L. 2013; 4 (4): 356-361

  Abstract

  Innominate artery cannulation has been widely adopted as a means to perform aortic arch reconstruction with continuous cerebral perfusion in the newborn. Although this technique has been subsequently utilized in infants and children, there is currently no data regarding the safety or efficacy in these older children. The purpose of this study was to review our experience with innominate artery cannulation for aortic arch reconstruction in patients beyond the neonatal period.This was a retrospective review of 42 infants and children who underwent aortic arch reconstruction using the technique of innominate artery cannulation with continuous cerebral perfusion. Of these procedures, 29 (69%) were primary arch reconstructions, while 13 (31%) were aortic arch reoperations. The median age at surgery was 8 months, and 22 patients underwent concomitant intracardiac repairs.There was no operative mortality in this cohort of 42 patients undergoing aortic arch reconstruction. There was also no overt evidence of neurologic injury. Specifically, none of the patients demonstrated a neurologic deficit, clinical seizure, or unexpected delay in regaining a normal state of consciousness. The median duration of antegrade cerebral perfusion was 34 minutes. The median duration of hospital stay was 11 days. No patient required reoperation on the aortic arch with a median of 45-month follow-up.Innominate artery cannulation is a safe and effective technique for aortic arch reconstruction in nonneonates. We conclude that antegrade cerebral perfusion is a useful technique for aortic arch reconstruction in this patient population.

  View details for DOI 10.1177/2150135113497767

  View details for PubMedID 24327627

• Fetal cardiac intervention: Improved results of fetal cardiac bypass in immature fetuses using the TinyPump device. journal of thoracic and cardiovascular surgery Sebastian, V. A., Ferro, G., Kagawa, H., Nasirov, T., Maeda, K., Ferrier, W. T., Takatani, S., Riemer, R. K., Hanley, F. L., Reddy, V. M. 2013; 145 (6): 1460-1464

  Abstract

  Fetal cardiac surgery is a potential innovative treatment for certain congenital heart defects that have significant mortality and morbidity in utero or after birth, but it has been limited by placental dysfunction after fetal cardiac bypass. We have used the TinyPump device for fetal cardiac bypass in sheep fetuses at 90 to 110 days gestation.Ten mixed-breed pregnant ewes were used over a period of 6 months, and 10 fetuses were placed on bypass for 30 minutes. Five fetuses with a mean gestational age of 104 ± 4.5 days and mean weight of 1.4 ± 0.4 kg were placed on bypass using the TinyPump device, and 5 fetuses with a mean gestational age of 119 ± 4.5 days and mean weight of 3.4 ± 0.4 kg were placed on bypass using the roller head pump. The fetuses were monitored for up to 3 hours after bypass or until earlier demise.Progressive respiratory and metabolic acidosis developed in all fetuses. The TinyPump group had a lower gestational age and weight compared with the roller head pump group. However, the rate of postbypass deterioration in the TinyPump group, as measured with blood gases, was noted to be significantly slower compared with the roller head pump group.We demonstrate the feasibility of the TinyPump device for fetal cardiac bypass in a fetal sheep model. The TinyPump group showed improved results compared with the roller head group despite more immature fetuses. The TinyPump device seems to be a promising device for future studies of fetal cardiac bypass in immature fetal sheep and in primates.

  View details for DOI 10.1016/j.jtcvs.2012.08.014

  View details for PubMedID 22944083

• Surgical management of neonatal atrioventricular septal defect with aortic arch obstruction. Annals of thoracic surgery Shuhaiber, J., Shin, A. Y., Gossett, J. G., Wypij, D., Backer, C. L., Hanley, F. L., Khan, M. S., Fraser, C. D., Jacques, F., Manning, P. B., Van Arsdell, G., Mayer, J. E., Costello, J. M. 2013; 95 (6): 2071-2077

  Abstract

  For neonates with atrioventricular septal defect and aortic arch obstruction including coarctation of the aorta, we sought to determine whether a difference in outcomes exists after a primary neonatal versus staged surgical repair (neonatal arch repair with delayed intracardiac repair).This retrospective cohort study included consecutive neonates with atrioventricular septal defect and aortic arch obstruction who underwent cardiac surgery before 28 days of age at six centers from 1990 to 2009. Characteristics and outcomes between patients undergoing neonatal versus staged repair were compared.Of 66 study patients, 31 (47%) underwent primary neonatal repair and 35 (53%) underwent staged repair. At baseline echocardiogram, a greater percentage of neonatal repair patients had relative unbalanced ventricular size (56% versus 35%, p = 0.02). There were no other differences in demographic characteristics, cardiac anatomical or functional details, or surgical technique. Those undergoing neonatal repair tended to be more likely to have at least moderate left atrioventricular valve regurgitation early after repair (42% versus 19%, p = 0.05) and to have at least one major in-hospital complication (42% versus 20%, p = 0.06). After the initial cardiac operation, compared with the neonatal repair group, patients undergoing staged repair had greater survival (87% versus 57% at 6 years, log-rank p = 0.02) and freedom from the first unplanned cardiac reoperation (69% versus 45% at 6 years, log-rank p = 0.005).For neonates with atrioventricular septal defect and aortic arch obstruction, when compared with neonatal repair, a staged approach was associated with improved survival and lower morbidity.

  View details for DOI 10.1016/j.athoracsur.2012.11.069

  View details for PubMedID 23415240

• Aortic valve disease in pulmonary atresia and major aortopulmonary collaterals. Annals of thoracic surgery Mainwaring, R. D., Dimeling, G., Punn, R., Hanley, F. L. 2013; 95 (6): 2122-2125

  Abstract

  Aortic valve disease in association with pulmonary atresia, ventricular septal defect, and major aortopulmonary collaterals (PA/VSD/MAPCAs) is an extremely rare combination of congenital heart defects. The presence of aortic stenosis or insufficiency or both imposes an additional physiologic burden that complicates the management of PA/VSD/MAPCAs. This report summarizes our experience with 7 patients who underwent surgical repair of this rare combination of defects.This was a retrospective review of patients who had both aortic valve disease and PA/VSD/MAPCAs. Four patients had predominantly aortic stenosis, 2 patients had aortic insufficiency, and 1 had mixed stenosis and insufficiency. Anatomically, this corresponded to a dysplastic trileaflet valve in 4, bicuspid valve in 2, and quadricuspid valve in 1.Four of the 7 patients underwent aortic valve surgery at the time of their first operation for PA/VSD/MAPCAs. The remaining 3 patients had aortic valve surgery at subsequent operations. All 7 patients are alive, with a median duration of follow-up of 3 years. One patient has required aortic valve replacement 2 years after repair of an insufficient bicuspid valve.This manuscript summarizes our experience with 7 patients who had the rare combination of aortic valve disease and PA/VSD/MAPCAs. Although aortic valve disease has been reported for other conotruncal defects, we believe this is the first report of its presence in association with PA/VSD/MAPCAs.

  View details for DOI 10.1016/j.athoracsur.2013.03.070

  View details for PubMedID 23643545

• Postoperative respiratory failure in children with tetralogy of fallot, pulmonary atresia, and major aortopulmonary collaterals: a pilot study. Pediatric critical care medicine Asija, R., Hanley, F. L., Roth, S. J. 2013; 14 (4): 384-389

  Abstract

  : Children with tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collaterals (TOF/PA/MAPCAs), who undergo unifocalization surgery are at risk for prolonged postoperative respiratory failure. We sought to identify risk factors that are associated with prolonged mechanical ventilation in this population.: We collected preoperative and operative data from medical records and postoperative data prospectively. Mechanical ventilation beyond postoperative day 5, which was greater than the 50th percentile for the subjects enrolled, was considered prolonged. Risk factors were analyzed using multiple logistic regression, independent samples t test, Fisher's exact test, and Kruskal-Wallis test. Enrollment occurred over a 20-month period between May 2009 and January 2011.: Lucile Packard Children's Hospital (Palo Alto, CA).: All patients with TOF/PA/MAPCAs presenting for unifocalization or pulmonary artery revision procedures were eligible, including those with additional structural cardiac abnormalities requiring surgical intervention. We excluded patients with single-ventricle cardiac anatomy and preoperative respiratory failure or infection.: We enrolled 35 consecutive patients undergoing 37 procedures over the study period. One patient was excluded for single-ventricle anatomy. There were 12 cases (32%) of prolonged mechanical ventilation. Delayed sternal closure was the only risk factor associated with prolonged mechanical ventilation (p = 0.01). Age, weight, cardiopulmonary bypass time, 22q11 microdeletion, postoperative fluid balance, bronchospasm, and nonrespiratory infection were not significantly associated with prolonged mechanical ventilation. Respiratory complications occurred in both groups, and patients with pneumonia were more likely to have a prolonged course (p = 0.03). There was no significant association between the type of surgery performed and duration of mechanical ventilation.: Prolonged postoperative respiratory failure in children undergoing surgery for TOF/PA/MAPCAs was independently associated with delayed sternal closure. Respiratory complications occur after unifocalization surgery, and pneumonia is associated with prolonged mechanical ventilation. Our pilot study suggests that clinical features common in this patient population, such as bronchospasm and 22q11 microdeletion, were not associated with more postoperative respiratory failure.

  View details for DOI 10.1097/PCC.0b013e318272062b

  View details for PubMedID 23439458

• Hemodynamic Assessment After Complete Repair of Pulmonary Atresia With Major Aortopulmonary Collaterals ANNALS OF THORACIC SURGERY Mainwaring, R. D., Reddy, V. M., Peng, L., Kuan, C., Palmon, M., Hanley, F. L. 2013; 95 (4): 1397-1402

  Abstract

  Pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals (PA/VSD/MAPCAs) is a complex form of congenital heart defect. There are limited data regarding late hemodynamics of patients after repair of PA/VSD/MAPCAs. This study evaluated the hemodynamics of patients who underwent complete repair of PA/VSD/MAPCSs and subsequently returned for a conduit change.This was a retrospective review of 80 children undergoing a right ventricle (RV)-to-pulmonary artery conduit replacement after complete repair of PA/VSD/MAPCAs. All patients underwent preoperative cardiac catheterization to define the cardiac physiology. Patients were an average age of 6.5±1.2 years, and the average interval between complete repair and conduit change was 4.5±1.1 years.The preoperative cardiac catheterization demonstrated an average RV right peak systolic pressure of 70±22 mm Hg and pulmonary artery pressure of 38±14 mm Hg. This pressure gradient of 32 mm Hg reflects the presence of conduit obstruction. After conduit change, the intraoperative RV systolic pressure was 34±8 mm Hg, similar to 36±9 mm Hg at the conclusion of the previous complete repair. The corresponding RV/aortic pressure ratios were 0.36±0.07 and 0.39±0.09, respectively.The data demonstrate that patients who underwent complete repair of PA/VSD/MAPCAs had nearly identical pulmonary artery pressures when they returned for conduit change some 4.5 years later. This finding indicates that the growth and development of the unifocalized pulmonary vascular bed is commensurate with visceral growth. We would hypothesize that complete repair, along with low RV pressures, will confer a long-term survival advantage.

  View details for DOI 10.1016/j.athoracsur.2012.12.066

  View details for PubMedID 23453744

• Surgical Reconstruction of Pulmonary Stenosis With Ventricular Septal Defect and Major Aortopulmonary Collaterals ANNALS OF THORACIC SURGERY Mainwaring, R. D., Punn, R., Reddy, V. M., Hanley, F. L. 2013; 95 (4): 1417-1421

  Abstract

  Pulmonary stenosis with ventricular septal defect and major aortopulmonary collaterals (PS/VSD/MAPCAs) is an extremely rare form of congenital heart defect. Although it has been assumed that PS/VSD/MAPCAs would be similar to pulmonary atresia (PA) with VSD/MAPCA, there are currently no data to support this conjecture. This study reviewed our surgical experience with reconstruction of PS/VSD/MAPCA.This was a retrospective review of 25 patients (14 boys, 11 girls) who were born with PS/VSD/MAPCA and underwent surgical reconstruction. Preoperative pulmonary angiography was used to define the central branch pulmonary arteries and MAPCA. Patients were a median age of 4 months at the first operation.There was one operative death (4%) in this cohort of 25 patients, and complete repair was achieved in the 24 survivors (96%). There were two distinct subgroups of patients: 11 demonstrated cyanosis in the neonatal timeframe and underwent an initial procedure to augment pulmonary blood flow (+PBF). The remaining 14 patients formed the second group (-PBF). The median age at the first operation was 0.8 months in the +PBF group and 5.2 months in the -PBF group (p<0.005). Complete repair was achieved in 91% of patients in the +PBF group and in 100% in the -PBF group; however, the average number of procedures to achieve complete repair was 2.8 in the +PBF group vs 1.0 in the -PBF group (p<0.005).Outcomes for PS/VSD/MAPCAs as a whole were excellent, with a low surgical mortality and high rate of complete repair. There were two identifiable subgroups with distinctive differences required in their surgical management. These results provide a prognostic outlook for patients with PS/VSD/MAPCAs that can be compared and contrasted with PA/VSD/MAPCAs.

  View details for DOI 10.1016/j.athoracsur.2013.01.007

  View details for Web of Science ID 000317150600039

  View details for PubMedID 23434256

• MAJOR AORTOPULMONARY COLLATERAL ARTERY NOMENCLATURE 62nd Annual Scientific Session of the American-College-of-Cardiology Algaze, C. A., Peng, L. F., Feinstein, J. A., Reddy, V. M., Hanley, F. L., Perry, S. B. ELSEVIER SCIENCE INC. 2013: E543–E543

  View details for Web of Science ID 000316555200543

• EXTRACARDIAC CONDUIT FONTAN PROCEDURE WITHOUT THE USE OF CARDIOPULMONARY BYPASS: EARLY OUTCOMES 62nd Annual Scientific Session of the American-College-of-Cardiology Algaze, C. A., Reddy, V. M., Hanley, F. L., Perry, S. B. ELSEVIER SCIENCE INC. 2013: E503–E503

  View details for Web of Science ID 000316555200503

• Surgical reconstruction of peripheral pulmonary artery stenosis in Williams and Alagille syndromes 38th Annual Meeting of the Western-Thoracic-Surgical-Association Monge, M. C., Mainwaring, R. D., Sheikh, A. Y., Punn, R., Reddy, V. M., Hanley, F. L. MOSBY-ELSEVIER. 2013: 476–81

  Abstract

  Peripheral pulmonary artery stenosis is a rare congenital heart defect frequently found in association with Williams and Alagille syndromes. Controversy exists regarding the optimal treatment of peripheral pulmonary artery stenosis, with most centers favoring catheter-based interventions. In contrast, we have preferentially used surgical reconstruction of peripheral pulmonary artery stenosis. The purpose of the present study was to review our experience with surgical reconstruction of peripheral pulmonary artery stenosis.We performed a retrospective review of patients who underwent surgical reconstruction of peripheral pulmonary artery stenosis. A total of 16 patients were identified: 7 had Williams syndrome, 6 had Alagille syndrome, and 3 had no identifiable syndrome. Detailed pulmonary angiography was performed in all patients to define stenoses at the main, branch, lobar, and segmental arterial levels. The mean preoperative right ventricular/left ventricular pressure ratio was 0.88 ± 0.07. The surgical approach was a median sternotomy with cardiopulmonary bypass. All peripheral stenoses were augmented with pulmonary artery homograft tissue. The median age at surgery was 14 months, and concomitant procedures were performed in 9 of the 16 patients.There was 1 operative mortality (6%). The mean right ventricular/left ventricular pressure ratio decreased to 0.40 ± 0.04 postoperatively (P < .005), representing a 55% reduction compared with the preoperative values. The patients were followed up for a median of 5 years. No late mortality occurred and reoperation was not required.The data have demonstrate that this comprehensive surgical approach to the treatment of peripheral pulmonary artery stenosis was associated with low early and no late mortality. Surgical reconstruction of the peripheral pulmonary artery stenosis resulted in a significant decrease in right ventricular pressure. We hypothesize that this reduction in right ventricular pressures will confer a long-term survival advantage for this cohort of patients.

  View details for DOI 10.1016/j.jtcvs.2012.09.102

  View details for Web of Science ID 000313634700030

  View details for PubMedID 23228407

• Computational fluid dynamic simulations for determination of ventricular workload in aortic arch obstructions JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY Coogan, J. S., Chan, F. P., LaDisa, J. F., Taylor, C. A., Hanley, F. L., Feinstein, J. A. 2013; 145 (2): 489-U535

  View details for DOI 10.1016/j.jtcvs.2012.03.051

  View details for Web of Science ID 000313634700032

  View details for PubMedID 22516390

• Computational fluid dynamic simulations for determination of ventricular workload in aortic arch obstructions. journal of thoracic and cardiovascular surgery Coogan, J. S., Chan, F. P., LaDisa, J. F., Taylor, C. A., Hanley, F. L., Feinstein, J. A. 2013; 145 (2): 489-495 e1

  Abstract

  The cardiac workload associated with various types of aortic obstruction was determined using computational fluid dynamic simulations.Computed tomography image data were collected from 4 patients with 4 distinct types of aortic arch obstructions and 4 controls. The categorization of arch hypoplasia corresponded to the "A, B, C" nomenclature of arch interruption; a type "D" was added to represent diffuse arch hypoplasia. Measurements of the vessel diameter were compared against the normal measurements to determine the degree of narrowing. Three-dimensional models were created for each patient, and additional models were created for type A and B hypoplasia to represent 25%, 50%, and 75% diameter narrowing. The boundary conditions for the computational simulations were chosen to achieve realistic flow and pressures in the control cases. The simulations were then repeated after changing the boundary conditions to represent a range of cardiac and vascular adaptations. The resulting cardiac workload was compared with the control cases.Of the 4 patients investigated, 1 had aortic coarctation and 3 had aortic hypoplasia. The cardiac workload of the patients with 25% narrowing type A and B hypoplasia was not appreciably different from that of the control. When comparing the different arch obstructions, 75% type A, 50% type B, and 50% type D hypoplasia required a greater workload increase than 75% coarctation.The present study has determined the hemodynamic significance of aortic arch obstruction using computational simulations to calculate the cardiac workload. These results suggest that all types of hypoplasia pose more of a workload challenge than coarctation with an equivalent degree of narrowing.

  View details for DOI 10.1016/j.jtcvs.2012.03.051

  View details for PubMedID 22516390

• Prevalence of Anomalous Coronary Arteries in Pulmonary Atresia with Ventricular Septal Defect and Major Aortopulmonary Collaterals. Journal of the American College of Cardiology Muralidaran, A. n., Mainwaring, R. D., Reddy, V. M., Hanley, F. L. 2013

  View details for PubMedID 23871887

• Early Outcomes After Extracardiac Conduit Fontan Operation Without Cardiopulmonary Bypass PEDIATRIC CARDIOLOGY McCammond, A. N., Kuo, K., Parikh, V. N., Abdullah, K., Balise, R., Hanley, F. L., Roth, S. J. 2012; 33 (7): 1078-1085

  Abstract

  Cardiopulmonary bypass is associated with a systemic inflammatory response. The authors hypothesized that avoiding cardiopulmonary bypass would lead to improved postoperative outcomes for patients undergoing the extracardiac Fontan operation, the final stage in surgical palliation of univentricular congenital heart defects. A review of the Children's Heart Center Database showed a total of 73 patients who underwent an initial Fontan operation at Lucile Packard Children's Hospital at Stanford between 1 November 2001 and 1 November 2006. These patients were divided into two groups: those who underwent cardiopulmonary bypass (n = 26) and those who avoided cardiopulmonary bypass (n = 47). Preoperative demographics, hemodynamics, and early postoperative outcomes were analyzed. The two groups had comparable preoperative demographic characteristics and hemodynamics except that the average weight of the off-bypass group was greater (17.9 ± 9.1 vs 14.2 ± 2.7 kg; P = 0.01). Intraoperatively, the off-bypass group trended toward a lower rate of Fontan fenestration (4.3 vs 19.2%; P = 0.09), had lower common atrial pressures (4.6 ± 1.4 vs 5.5 ± 1.5 mmHg; P = 0.05), and Fontan pressures (11.9 ± 2.1 vs 14.2 ± 2.4 mmHg; P ≤ 0.01), and required less blood product (59.1 ± 37.6 vs 91.9 ± 49.4 ml/kg; P ≤ 0.01). Postoperatively, there were no significant differences in hemodynamic parameters, postoperative colloid requirements, duration of mechanical ventilation, volume or duration of pleural drainage, or duration of cardiovascular intensive care unit or hospital stay. Avoiding cardiopulmonary bypass influenced intraoperative hemodynamics and the incidence of fenestration but did not have a significant impact on the early postoperative outcomes of children undergoing the Fontan procedure.

  View details for DOI 10.1007/s00246-012-0228-5

  View details for PubMedID 22349678

• Late Outcomes in Patients Undergoing Aortopulmonary Window for Pulmonary Atresia/Stenosis and Major Aortopulmonary Collaterals ANNALS OF THORACIC SURGERY Mainwaring, R. D., Reddy, V. M., Perry, S. B., Peng, L., Hanley, F. L. 2012; 94 (3): 842-849

  Abstract

  Pulmonary atresia with ventricular septal defect (PA/VSD) and major aortopulmonary collateral arteries (MAPCAs) is a complex form of congenital heart defect. One identifiable subset has small (<2.5 mm) intrapericardial branch pulmonary arteries that are (1) confluent, (2) have normal arborization, and (3) have dual-supplied collateral vessels. When this anatomy is associated with limited pulmonary blood flow, the patients are candidates for creation of an aortopulmonary window to stimulate growth of the pulmonary arteries. The purpose of this study was to review our experience with creation of an aortopulmonary window as the initial palliative procedure.This was a retrospective review of our surgical experience with 35 children undergoing aortopulmonary window creation from 2002 to 2011. Patients were identified by preoperative cardiac catheterization to define the cardiac and pulmonary artery anatomy.There was no mortality in 35 patients undergoing aortopulmonary window creation. These patients have subsequently undergone 78 cardiac procedures (with 2 operative mortalities). Eighteen of these patients have achieved complete repair, 4 patients in a second procedure, 6 patients in a third procedure, 5 patients in a fourth procedure, and 3 patients in a fifth procedure.The data demonstrate that patients can undergo creation of an aortopulmonary window with excellent early results. Few patients were amenable to complete repair at the second operation, and most required multiple reoperations to recruit sufficient arborization. We interpret these counterintuitive results to suggest that hypoplastic central pulmonary arteries and diminished pulmonary blood flow are markers for a less well developed pulmonary vascular bed.

  View details for DOI 10.1016/j.athoracsur.2012.03.061

  View details for PubMedID 22857982

• Predictors for use of temporary epicardial pacing wires after pediatric cardiac surgery JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY Gupta, P., Jines, P., Gossett, J. M., Maurille, M., Hanley, F. L., Reddy, V. M., Miyake, C. Y., Roth, S. J. 2012; 144 (3): 557-562

  Abstract

  The objectives of this study were (1) to determine the use of temporary epicardial pacing wires to diagnose and treat early postoperative arrhythmias in pediatric cardiac surgical patients and (2) to determine the predictive factors for the need of pacing wires for diagnostic or therapeutic purposes.We collected preoperative, intraoperative, and postoperative data in a prospective, observational format from patients undergoing pediatric cardiac surgery between August 2010 and January 2011 at a single academic children's hospital.A total of 157 patients met the inclusion criteria during the study period. Of these 157 patients, pacing wires were placed in 127 (81%). Pacing wires were used in 25 patients (19.6%) for diagnostic purposes, 26 patients (20.4%) for therapeutic purposes, 15 patients (11.8%) for both diagnostic and therapeutic purposes, and 36 patients (28.3%) for diagnostic or therapeutic purposes. Need for cardioversion in the operating room, presence of 2 or more intracardiac catheters, severely reduced ventricular ejection fraction, and elevated serum lactate level at the time of operating room discharge were found to be independent predictors for the use of pacing wires. The only complication noted in the cohort was a skin infection at a pacing wire insertion site in 1 patient. A permanent pacemaker was required in 8 (6.2%) of all patients with temporary pacing wires.Our data support the use of temporary epicardial pacing wires in approximately 30% of children after congenital heart surgery. We found the need for cardioversion in the operating room, presence of 2 or more intracardiac catheters, severely reduced ventricular ejection fraction, and high serum lactate level at the time of discharge from the operating room to be independent predictors of the use of pacing wires in the early postoperative period.

  View details for DOI 10.1016/j.jtcvs.2011.12.060

  View details for Web of Science ID 000308064200037

  View details for PubMedID 22329984

• Surgical outcomes for patients with pulmonary atresia/major aortopulmonary collaterals and Alagille syndrome 25th Annual Meeting of the European-Association-for-Cardio-Thoracic-Surgery (EACTS) Mainwaring, R. D., Sheikh, A. Y., Punn, R., Reddy, V. M., Hanley, F. L. OXFORD UNIV PRESS INC. 2012: 235–41

  Abstract

  Pulmonary atresia with major aortopulmonary collateral arteries (PA/MAPCAs) is a complex congenital heart defect that has undergone significant advances in treatment over the past 15 years. A small subset of patients with PA/MAPCAs have associated Alagille syndrome, which can have an adverse impact on many other organ systems. The purpose of this study was to review our institutional outcomes for the surgical patients with PA/MAPCAs and Alagille syndrome.This was a retrospective review of patients with PA/MAPCA's and Alagille who underwent surgical reconstruction from November 2001 to August 2011. Fifteen patients were identified in our data base. Thirteen had pulmonary atresia with ventricular septal defect (PA/VSD) and two had pulmonary atresia with intact ventricular septum (PA-IVS).There has been no early or late mortality in this cohort of 15 patients with PA/MAPCA' and Alagille syndrome. The patients have undergone a total of 38 cardiac surgical procedures. Ten of the 13 patients with PA/VSD have achieved complete repair, including unifocalization, a right ventricle to pulmonary artery conduit and closure of all intra-cardiac shunts. The three unrepaired patients with PA/VSD remain potential candidates for eventual complete repair, while the two patients with PA-IVS remain viable candidates for a single ventricle pathway. The patients in this series have also undergone 12 major non-cardiac procedures.The data demonstrate that surgical reconstruction of PA/MAPCAs can be successfully achieved in patients with Alagille syndrome. The longer-term prognosis remains guarded on the basis of the multi-organ system involvement of Alagille syndrome.

  View details for DOI 10.1093/ejcts/ezr310

  View details for Web of Science ID 000306365600013

  View details for PubMedID 22402453

• Midterm Results of the Modified Ross/Konno Procedure in Neonates and Infants ANNALS OF THORACIC SURGERY Maeda, K., Rizal, R. E., Lavrsen, M., Malhotra, S. P., Akram, S. A., Davies, R., Suleman, S., Reinhartz, O., Murphy, D. J., Hanley, F. L., Reddy, V. M. 2012; 94 (1): 156-163

  Abstract

  The management of congenital aortic stenosis in neonates and infants continues to be a surgical challenge. We have performed the modified Ross-Konno procedure for patients who have severe aortic insufficiency or significant residual stenosis after balloon aortic dilation. The midterm results of this procedure were evaluated in this subset of patients.Between 1994 and 2010, a total of 24 patients younger than 1 year of age underwent the modified Ross-Konno procedure. The diagnoses were aortic stenosis with or without subaortic stenosis (n = 16), Shone's complex (n = 7), and interrupted aortic arch with subaortic stenosis (n = 1). The aortic root was replaced with a pulmonary autograft, and the left ventricular outflow tract (LVOT) was enlarged with a right ventricular infundibular free wall muscular extension harvested with the autograft.Age at operation ranged from 1 to 236 days (median 28 days). The median follow-up period was 81 months (range 1-173 months). There was 1 early death and no late mortality. Overall the 1-, 2-, and 5-year survival rate was 95% ± 4.5%. Freedom from aortic stenosis was 94.7% ± 5.1% at 1, 2, and 5 years. Less than mild aortic insufficiency was 93.3% ± 6.4% at 2 years, and 74.7% ± 12.9% at 5 years. In total, 23 reoperations and reinterventions were performed; 14 were allograft conduit replacements. Two patients required aortic valve plasty. None required valve replacement. The reintervention-free rate was 64.6% ± 10.8% at 2 years and 36.9% ± 11.3% at 5 years.Pulmonary autografts demonstrated good durability with low mortality and morbidity. This study shows that the modified Ross-Konno procedure can be a practical choice in selective cases for complex LVOT stenosis in neonates and infants.

  View details for DOI 10.1016/j.athoracsur.2012.03.007

  View details for Web of Science ID 000305801600033

  View details for PubMedID 22626750

• Pulmonary reperfusion injury after the unifocalization procedure for tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collateral arteries JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY Maskatia, S. A., Feinstein, J. A., Newman, B., Hanley, F. L., Roth, S. J. 2012; 144 (1): 184-189

  Abstract

  The aims of our study are to describe the incidence, clinical profile, and risk factors for pulmonary reperfusion injury after the unifocalization procedure for tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collateral arteries. We hypothesized the following: (1) Pulmonary reperfusion injury is more likely to occur after unifocalization procedures in which a septated circulation is not achieved, (2) pulmonary reperfusion injury is directly related to the severity of stenosis in major aortopulmonary collateral arteries, and (3) pulmonary reperfusion injury leads to longer intubation time and longer hospitalization.Consecutive patients with tetralogy of Fallot/pulmonary atresia/major aortopulmonary collateral arteries who underwent unifocalization procedures over a 5-year period were identified in our institutional database. Chest radiographs before the unifocalization procedure, from postoperative days 0 to 4, and from 2 weeks after the unifocalization procedure or at discharge were evaluated by a pediatric radiologist for localized pulmonary edema. Determination of stenosis severity was based on review of preoperative angiograms. Statistical analyses using multivariate repeated-measures analyses were performed with generalized estimating equations.Pulmonary reperfusion injury was present after 42 of 65 (65%) unifocalization procedures. In 36 of 42 cases of reperfusion injury, unilateral injury was present. Risk factors for the development of reperfusion injury included bilateral unifocalization (P = .01) and degree of stenosis (P = .03). We did not identify an association between pulmonary reperfusion injury and time to tracheal extubation or hospital discharge.Pulmonary reperfusion injury is common after the unifocalization procedure for tetralogy of Fallot/pulmonary atresia/major aortopulmonary collateral arteries. Severity of stenosis and bilateral unifocalization are associated with the development of reperfusion injury.

  View details for DOI 10.1016/j.jtcvs.2011.12.030

  View details for Web of Science ID 000305412200031

  View details for PubMedID 22244564

• Reconstruction of Pulmonary Artery in a Newborn Using a Porcine Small Intestinal Submucosal Patch ANNALS OF THORACIC SURGERY Chalajour, F., Barboza, L. A., Boni, L., Snyder, R., Hanley, F. L., Reddy, V. M., Riemer, R. K. 2012; 93 (4): 1311-1315

  Abstract

  In this case report, we evaluated cellular structure and the growth potential of a porcine small intestinal submucosal patch used for pulmonary artery augmentation in a 20-day-old newborn with pulmonary atresia. The patch was resected 2 months postoperatively due to apparent abnormal wall thickening and evaluated by histologic and immunohistologic staining.

  View details for DOI 10.1016/j.athoracsur.2011.08.055

  View details for Web of Science ID 000302120200064

  View details for PubMedID 22450088

• Surgical Reconstruction of Tracheal Stenosis in Conjunction With Congenital Heart Defects Basic Science Forum of the 58th Annual Meeting of the Southern-Thoracic-Surgical-Association Mainwaring, R. D., Shillingford, M., Davies, R., Koltai, P., Navaratnam, M., Reddy, V. M., Hanley, F. L. ELSEVIER SCIENCE INC. 2012: 1266–73

  Abstract

  Surgical reconstruction is the primary method of treating airway obstruction in children. Tracheal stenosis is frequently associated with congenital heart defects, which may further complicate the overall management strategy. The purpose of this study was to review our experience with surgical reconstruction of airway obstruction in conjunction with congenital heart defects.This was a retrospective review of our surgical experience with tracheal stenosis from February 2003 to August 2011. Twenty-seven patients were identified in our database. Six patients had isolated, congenital tracheal stenosis, and 21 had tracheal stenosis in association with congenital heart defects. There were two identifiable subgroups. Thirteen patients had airway stenoses identified concurrently with congenital heart defects and underwent combined repair. The second group comprised 8 patients who had previous correction of their congenital heart defects and experienced delayed presentation of tracheal (n = 6) or bronchial (n = 2) obstruction.The median age at surgery was 9 months. There were 2 postoperative deaths, both in children with single ventricle. The median duration of follow-up for the entire cohort of 25 surviving patients was 4 years. None of the patients have required reoperations on the trachea; 5 have had minor reinterventions.The data demonstrate that tracheal obstruction is frequently found in conjunction with congenital heart defects. Nearly one third of our patients had delayed presentation of airway obstruction that was identified subsequent to previous congenital heart defect repair. Tracheal reconstructive techniques were effective regardless of the cause of the airway obstruction.

  View details for DOI 10.1016/j.athoracsur.2011.12.063

  View details for PubMedID 22381444

• Late Repair of the Native Pulmonary Valve in Patients With Pulmonary Insufficiency After Surgery for Tetralogy of Fallot ANNALS OF THORACIC SURGERY Mainwaring, R. D., Pirolli, T., Punn, R., Hanley, F. L. 2012; 93 (2): 677-679

  Abstract

  Pulmonary regurgitation developing late after tetralogy of Fallot repair is now recognized as a serious threat to the long-term welfare of these patients. This article summarizes our experience with 5 patients who underwent reoperations for treatment of severe pulmonary regurgitation after transannular patch repair of tetralogy of Fallot. In each case, the intraoperative findings revealed anatomy favorable for valve repair and enabled preservation of the native pulmonary valves.

  View details for DOI 10.1016/j.athoracsur.2011.09.016

  View details for Web of Science ID 000299540200069

  View details for PubMedID 22269747

• Surgical Results in Patients With Pulmonary Atresia-Major Aortopulmonary Collaterals in Association With Total Anomalous Pulmonary Venous Connection ANNALS OF THORACIC SURGERY Mainwaring, R. D., Reddy, M., Reinhartz, O., Punn, R., Tacy, T., Hanley, F. L. 2011; 92 (5): 1756-1760

  Abstract

  Pulmonary atresia and major aortopulmonary collaterals (PA/MAPCAs) is a complex form of congenital heart disease. One to two percent of patients with PA/MAPCAs will also have total anomalous pulmonary venous connection (TAPVC). This study summarizes our surgical experience with this rare combination of life-threatening congenital heart defects.A retrospective review was performed to identify patients who had surgery for PA/MAPCAs in association with TAPVC. From November 2001 to March 2011, 9 patients presented with this combination of defects. Eight of the 9 patients had heterotaxy with an unbalanced atrioventricular canal and functional single ventricle. The ninth patient had double outlet right ventricle (two ventricles). Timing of surgical intervention was typically predicated on the degree of pulmonary venous obstruction. The median age at surgery was 14 days. All nine patients had surgical correction of TAPVC, unifocalization of MAPCA's into a central confluence, and placement of a shunt.There was one early mortality (< 30 days) and two late mortalities. For the 6 survivors, 5 have subsequently undergone a bidirectional Glenn procedure, and 3 had completion of their Fontan. Two patients are currently at the bidirectional Glenn stage; one is a good candidate for Fontan completion while the other is not suitable. The sixth patient is awaiting further assessment.The PA/MAPCAs, in association with TAPVC, is a challenging combination of defects. The data suggest that the combination of PA/MAPCAs and TAPVC can be undertaken with a reasonable midterm prognosis.

  View details for DOI 10.1016/j.athoracsur.2011.06.020

  View details for Web of Science ID 000296925400045

  View details for PubMedID 21944736

• Anomalous Aortic Origin of a Coronary Artery: Medium-Term Results After Surgical Repair in 50 Patients ANNALS OF THORACIC SURGERY Mainwaring, R. D., Reddy, V. M., Reinhartz, O., Petrossian, E., Macdonald, M., Nasirov, T., Miyake, C. Y., Hanley, F. L. 2011; 92 (2): 691-697

  Abstract

  Anomalous aortic origin of a coronary artery (AAOCA) is a rare congenital heart defect that has been associated with myocardial ischemia and sudden death. Controversies exist regarding the diagnosis, treatment, and long-term recommendations for patients with AAOCA. The purpose of this study is to evaluate the medium-term results of surgical repair for AAOCA.From January 1999 through August 2010, 50 patients underwent surgical repair of AAOCA. The median age at surgery was 14 years (range, 5 days to 47 years). Thirty-one patients had the right coronary originate from the left sinus of Valsalva, 17 had the left coronary originate from the right sinus, and 2 had an eccentric single coronary ostium. Twenty six of the 50 patients had symptoms of myocardial ischemia preoperatively, and 14 patients had associated congenital heart defects. Repair was accomplished by unroofing in 35, reimplantation in 6, and pulmonary artery translocation in 9.There was no operative mortality. The median time of follow-up has been 5.7 years. Two patients were lost to follow-up, and 1 patient required heart transplantation 1 year after AAOCA repair. In the remaining 47 postoperative patients, all have remained free of cardiac symptoms and no one has experienced a sudden death event.The surgical treatment of AAOCA is safe and appears to be highly effective in eliminating ischemic symptoms. These medium-term results are encouraging and suggest that many patients may be able to resume normal activities.

  View details for DOI 10.1016/j.athoracsur.2011.03.127

  View details for Web of Science ID 000293221000050

  View details for PubMedID 21718962

• Can randomized clinical trials impact the surgical approach for hypoplastic left heart syndrome? World journal for pediatric & congenital heart surgery Tabbutt, S., Goldberg, C., Ohye, R. G., Morell, V. O., Hanley, F. L., Lamberti, J. J., Jacobs, M. L., Jacobs, J. P. 2011; 2 (3): 445-456

  Abstract

  The Eighth International Conference of the Pediatric Cardiac Intensive Care Society was held in Miami, Florida, December 8 to 11, 2010. The program included a session dedicated to the management of hypoplastic left heart syndrome (HLHS), with particular emphasis on the innovations that have led to contemporary schemes of management and the role of clinical trials in the evolution and acceptance of these strategies. An invited panel of experts reviewed the historical evolution of staged surgical reconstruction, the randomized clinical trials that have been undertaken thus far, and the extent to which these have, or have not, influenced individual and institutional approaches to management of HLHS.

  View details for DOI 10.1177/2150135111406942

  View details for PubMedID 23803996

• Survival after extreme left atrial hypertension and pulmonary hemorrhage in an infant supported with extracorporeal membrane oxygenation for refractory atrial flutter PEDIATRIC CRITICAL CARE MEDICINE Cisco, M. J., Asija, R., Dubin, A. M., Perry, S. B., Hanley, F. L., Roth, S. J. 2011; 12 (3): E149-E152

  Abstract

  We report here the survival of an infant who developed extreme left atrial hypertension and severe pulmonary hemorrhage while supported with extracorporeal membrane oxygenation for refractory atrial flutter. The patient recovered after decompression of the left heart and catheter ablation of the atrioventricular node.Lucile Packard Children's Hospital (Stanford, CA).Chart review.Recovery of lung function is possible despite systemic-level left atrial pressure resulting in pulmonary hemorrhage and complete solidification of lung parenchyma on gross inspection. Resolution of pulmonary hemorrhage despite anticoagulation while on extracorporeal membrane oxygenation can occur after relief of left atrial hypertension.

  View details for DOI 10.1097/PCC.0b013e3181e8b3e5

  View details for PubMedID 20693934

• PULMONARY REPERFUSION INJURY AFTER THE UNIFOCALIZATION PROCEDURE FOR TETRALOGY OF FALLOT, PULMONARY ATRESIA AND MAJOR AORTOPULMONARY COLLATERAL ARTERIES 60th Annual Scientific Session and Expo of the American-College-of-Cardiology (ACC) / I2 Summit / ACCF/Herman K. Gold Young Investigator's Award in Molecular and Cellular Cardiology Maskatia, S., Feinstein, J. A., Newman, B., Hanley, F. L., Roth, S. J. ELSEVIER SCIENCE INC. 2011: E419–E419

  View details for Web of Science ID 000291695100420

• The hybrid procedure for the borderline left ventricle CARDIOLOGY IN THE YOUNG Davis, C. K., Pastuszko, P., Lamberti, J., Moore, J., Hanley, F., El Said, H. 2011; 21 (1): 26-30

  Abstract

  IntroductionIn patients with varying degrees of left heart hypoplasia, it is often difficult to determine whether the left heart structures are adequate in size to support biventricular circulation. Historically, the decision to pursue a single ventricle or biventricular repair needed to be made early and was often irreversible. The hybrid procedure may be a better initial approach for patients with borderline left ventricles.We describe a series of four patients with various congenital cardiac malformations, all of whom had borderline left ventricles. Based on pre-operative echocardiograms, several scoring systems and left ventricle volumes were used to predict the optimal type of repair. A left ventricular volume of 20 millilitres per square metre was used as the minimum cut-off value for adequacy of biventricular repair.The left ventricular volumes for the patients were 17.1, 23.7, 25.4, and 25.8 millilitres per square metre. In none of the four patients were the calculations unanimous in the recommendation to pursue either type of repair. All patients underwent the hybrid procedure and then eventual single ventricle palliation (two patients) or biventricular repair (two patients). All survived with a mean follow-up of 18 plus or minus 3.9 months.The hybrid procedure may be the best option in patients with a borderline left ventricle. It can serve as a bridge to a more definitive repair when patients are older, larger, and for whom the decision between single ventricle and biventricular repair can be more easily made.

  View details for DOI 10.1017/S1047951110001423

  View details for Web of Science ID 000285981400005

  View details for PubMedID 21070689

• The hemi-Mustard/bidirectional Glenn atrial switch procedure in the double-switch operation for congenitally corrected transposition of the great arteries: Rationale and midterm results 90th Annual Meeting of the American-Association-for-Thoracic-Surgery Malhotra, S. P., Reddy, V. M., Qiu, M., Pirolli, T. J., Barboza, L., Reinhartz, O., Hanley, F. L. MOSBY-ELSEVIER. 2011: 162–70

  Abstract

  This study was undertaken to assess the risks and benefits of the double-switch operation using a hemi-Mustard atrial switch procedure and the bidirectional Glenn operation for congenitally corrected transposition of the great arteries. To avoid complications associated with the complete Senning and Mustard procedures and to assist right-heart hemodynamics, we favor a modified atrial switch procedure, consisting of a hemi-Mustard procedure to baffle inferior vena caval return to the tricuspid valve in conjunction with a bidirectional Glenn operation.Between January 1994 and September 2009, anatomic repair was achieved in 48 patients. The Rastelli-atrial switch procedure was performed in 25 patients with pulmonary atresia and the arterial-atrial switch procedure was performed in 23 patients. A hemi-Mustard procedure was the atrial switch procedure for 70% (33/48) of anatomic repairs.There was 1 in-hospital death after anatomic repair. There were no late deaths or transplantation. At a median follow-up of 59.2 months, 43 of 47 survivors are in New York Heart Association class I. Bidirectional Glenn operation complications were uncommon (2/33), limited to the perioperative period, and seen in patients less than 4 months of age. Atrial baffle-related reoperations or sinus node dysfunction have not been observed. Tricuspid regurgitation decreased from a mean grade of 2.3 to 1.2 after repair (P = .00002). Right ventricle-pulmonary artery conduit longevity is significantly improved.We describe a 15-year experience with the double-switch operation using a modified atrial switch procedure with favorable midterm results. The risks of the hemi-mustard and bidirectional Glenn operation are minimal and are limited to a well-defined patient subset. The benefits include prolonged conduit life, reduced baffle- and sinus node-related complications, and technical simplicity.

  View details for DOI 10.1016/j.jtcvs.2010.08.063

  View details for Web of Science ID 000285407500029

  View details for PubMedID 21055773

• Anomalous coronary arteries from the opposite sinus of Valsalva in asymptomatic siblings treated with pulmonary arterial translocation JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY Obayashi, D. Y., Yeh, J., Silverman, N. H., Hanley, F. L. 2010; 140 (6): 1430-1432

  View details for DOI 10.1016/j.jtcvs.2010.05.012

  View details for Web of Science ID 000284149200036

  View details for PubMedID 21078428

• Cerebral Oxygen Metabolism During Total Body Flow and Antegrade Cerebral Perfusion at Deep and Moderate Hypothermia ARTIFICIAL ORGANS Sasaki, T., Boni, L., Riemer, R. K., Yeung, J. T., Ramamoorthy, C., Beckman, R., Gisner, C., Shuttleworth, P., Hanley, F. L., Reddy, V. M. 2010; 34 (11): 980-986

  Abstract

  The aim of this study is to evaluate the effect of temperature on cerebral oxygen metabolism at total body flow bypass and antegrade cerebral perfusion (ACP). Neonatal piglets were put on cardiopulmonary bypass (CPB) with the initial flow rate of 200mL/kg/min. After cooling to 18°C (n=6) or 25°C (n=7), flow was reduced to 100mL/kg/min (half-flow, HF) for 15min and ACP was initiated at 40mL/kg/min for 45min. Following rewarming, animals were weaned from bypass and survived for 4h. At baseline, HF, ACP, and 4 h post-CPB, cerebral blood flow (CBF) was measured using fluorescent microspheres. Cerebral oxygen extraction (CEO(2) ) and cerebral metabolic rate of oxygen (CMRO(2) ) were monitored. Regional cranial oxygen saturation (rSO(2) ) was continuously recorded throughout the procedure using near-infrared spectroscopy. At 18°C, CBF trended lower at HF and ACP and matched baseline after CPB. CEO(2) trended lower at HF and ACP, and trended higher after CPB compared with baseline. CMRO(2) at ACP matched that at HF. Cranial rSO(2) was significantly greater at HF and ACP (P<0.001, P<0.001) and matched baseline after CPB. At 25°C, CBF trended lower at HF, rebounded and trended higher at ACP, and matched baseline after CPB. CEO(2) was equal at HF and ACP and trended higher after CPB compared with baseline. CMRO(2) at ACP was greater than that at HF (P=0.001). Cranial rSO(2) was significantly greater at HF (P=0.01), equal at ACP, and lower after CPB (P=0.03). Lactate was significantly higher at all time points (P=0.036, P<0.001, and P<0.001). ACP provided sufficient oxygen to the brain at a total body flow rate of 100mL/kg/min at deep hypothermia. Although ACP provided minimum oxygenation to the brain which met the oxygen requirement, oxygen metabolism was altered during ACP at moderate hypothermia. ACP strategy at moderate hypothermia needs further investigation.

  View details for DOI 10.1111/j.1525-1594.2010.01131.x

  View details for Web of Science ID 000284588300019

  View details for PubMedID 21092040

• Surgical management of life threatening events caused by intermittent aortic insufficiency in a native valve: case report JOURNAL OF CARDIOTHORACIC SURGERY Martin, M. H., Perry, S. B., Prochazka, J. V., Hanley, F. L., Silverman, N. H. 2010; 5

  Abstract

  We describe a case of a patient admitted with apparent life threatening events characterized by hypotension and bradycardia. The patient was ultimately found to have intermittent severe aortic insufficiency. Upon surgical exploration, abnormalities were discovered in the aortic valve, which had a small left coronary cusp with absence of the nodulus of Arantius. Following surgical repair of the valve, aimed at preventing the small cusp from becoming stuck in the open position, the patient has remained episode free for over one year.

  View details for DOI 10.1186/1749-8090-5-94

  View details for PubMedID 21034457

• Optimal flow rate for antegrade cerebral perfusion JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY Sasaki, T., Tsuda, S., Riemer, R. K., Ramamoorthy, C., Reddy, V. M., Hanley, F. L. 2010; 139 (3): 530-535

  Abstract

  Antegrade cerebral perfusion is widely used in neonatal heart surgery, yet commonly used flow rates have never been standardized. The objective of this study was to determine the antegrade cerebral perfusion flow rate that most closely matches standard cardiopulmonary bypass conditions.Nine neonatal piglets underwent deep hypothermic cardiopulmonary bypass at a total body flow of 100 mL/kg/min (baseline). Antegrade cerebral perfusion was conducted via innominate artery cannulation at perfusion rates of 10, 30, and 50 mL/kg/min in random order. Cerebral blood flow was measured using fluorescent microspheres. Regional oxygen saturation and cerebral oxygen extraction were monitored.Cerebral blood flow was as follows: baseline, 60 +/- 17 mL/100 g/min; antegrade cerebral perfusion at 50 mL/kg/min, 56 +/- 17 mL/100 g/min; antegrade cerebral perfusion at 30 mL/kg/min, 36 +/- 9 mL/100 g/min; and antegrade cerebral perfusion at 10 mL/kg/min, 13 +/- 6 mL/100 g/min. At an antegrade cerebral perfusion rate of 50 mL/kg/min, cerebral blood flow matched baseline (P = .87), as did regional oxygen saturation (P = .13). Antegrade cerebral perfusion at 30 mL/kg/min provided approximately 60% of baseline cerebral blood flow (P < .002); however, regional oxygen saturation was equal to baseline (P = .93). Antegrade cerebral perfusion at 10 mL/kg/min provided 20% of baseline cerebral blood flow (P < .001) and a lower regional oxygen saturation than baseline (P = .011). Cerebral oxygen extraction at antegrade cerebral perfusion rates of 30 and 50 mL/kg/min was equal to baseline (P = .53, .48) but greater than baseline (P < .0001) at an antegrade cerebral perfusion rate of 10 mL/kg/min. The distributions of cerebral blood flow and regional oxygen saturation were equal in each brain hemisphere at all antegrade cerebral perfusion rates.Cerebral blood flow increased with antegrade cerebral perfusion rate. At an antegrade cerebral perfusion rate of 50 mL/kg/min, cerebral blood flow was equal to baseline, but regional oxygen saturation and cerebral oxygen extraction trends suggested more oxygenation than baseline. An antegrade cerebral perfusion rate of 30 mL/kg/min provided only 60% of baseline cerebral blood flow, but cerebral oxygen extraction and regional oxygen saturation were equal to baseline. An antegrade cerebral perfusion rate that closely matches standard cardiopulmonary bypass conditions is between 30 and 50 mL/kg/min.

  View details for DOI 10.1016/j.jtcvs.2009.12.005

  View details for Web of Science ID 000274735400002

  View details for PubMedID 20176202

• Selective Right Ventricular Unloading and Novel Technical Concepts in Ebstein's Anomaly 45th Annual Meeting of the Society-of-Thoracic-Surgeons Malhotra, S. P., Petrossian, E., Reddy, V. M., Qiu, M., Maeda, K., Suleman, S., Macdonald, M., Reinhartz, O., Hanley, F. L. ELSEVIER SCIENCE INC. 2009: 1975–81

  Abstract

  Favorable outcomes in Ebstein's anomaly are predicated on tricuspid valve competence and right ventricular function. Successful valve repair should be aggressively pursued to avoid the morbidity of prosthetic tricuspid valve replacement. We report our experience with valve-sparing intracardiac repair, emphasizing novel concepts and techniques of valve repair supplemented by selective bidirectional Glenn (BDG).Between June 1993 and December 2008, 57 nonneonatal patients underwent Ebstein's anomaly repairs. The median age at operation was 8.1 years. All were symptomatic in New York Heart Association (NYHA) functional class II (n = 38), III (n = 17), or IV (n = 1). Preoperatively, 26 had mild or moderate cyanosis at rest. We used a number of valve reconstructive techniques that differed substantially from those currently described. BDG was performed in 31 patients (55%) who met specific criteria.No early or late deaths occurred. At the initial repair, 3 patients received a prosthetic valve. Four patients required reoperation for severe tricuspid regurgitation. Repeat repairs were successful in 2 patients. At follow-up (range, 3 months to 6 years), all patients were acyanotic and in NYHA class I. Tricuspid regurgitation was mild or less in 49 (86%) and moderate in 6 (11%). Freedom from a prosthesis was 91% (52 of 57).Following a protocol using BDG for ventricular unloading in selected patients with Ebstein's anomaly can achieve a durable valve-sparing repair using the techniques described. Excellent functional midterm outcomes can be obtained with a selective one and a half ventricle approach to Ebstein's anomaly.

  View details for DOI 10.1016/j.athoracsur.2009.07.019

  View details for Web of Science ID 000272029100037

  View details for PubMedID 19932271

• Deep Brain Hyperthermia While Rewarming from Hypothermic Circulatory Arrest 41st Annual Meeting of the Society-of-Thoracic-Surgeons Amir, G., Ramamoorthy, C., Riemer, R. K., Hanley, F. L., Reddy, V. M. WILEY-BLACKWELL PUBLISHING, INC. 2009: 606–10

  Abstract

  Neurologic injury is a feared and serious long-term complication of cardiopulmonary bypass (CPB) and deep hypothermic circulatory arrest (DHCA). Postoperative hyperthermia was found to enhance postischemic neurologic injury. The use of core temperature as the reference point through CPB assumes parallel changes in brain temperature. We tested the hypothesis that regional and deep brain temperature (DBT) differ during cooling, DHCA, and rewarming.Neonatal piglets (n = 9) were subject to CPB and cooled to rectal temperature (RT) of 18 degrees C, 30 minutes of DHCA were initiated, and subsequently the piglets were rewarmed to RT of 36.5 degrees C and weaned from CPB. Temperature probes were inserted into the DBT targeting the caudate and thalamic nuclei, their position confirmed by pathology. Superficial brain temperature was measured by a temperature probe inserted extradurally. RT, nasopharyngeal (NPT), and tympanic (TT) temperatures were recorded.During cooling the deep brain cooled faster and to lower temperatures compared to RT and TT; NPT reflected DBT accurately. During rewarming DBT was significantly higher than RT and TT. By the end of rewarming the difference between the deep brain and the RT reached statistical significance (30 minutes: 35.1 +/- 0.7 vs. 32.3 +/- 0.7 p < 0.05, respectively, 40 minutes: 37.5 +/- 0.3 vs. 34.7 +/- 0.8 p < 0.05, respectively).Deep brain hyperthermia routinely occurs during the last stages of rewarming following DHCA. DBT is accurately reflected by NPT and is directly correlated with inflow temperature. Therefore, during rewarming inflow temperatures should not exceed 36 degrees C and NPT should be closely monitored.

  View details for DOI 10.1111/j.1540-8191.2009.00883.x

  View details for Web of Science ID 000269540900034

  View details for PubMedID 19740304

• Outcome Analysis for a Small, Start-up Congenital Heart Surgery Program 1st Meeting of the World-Society-for-Pediatric-and-Congenital-Heart-Surgery Mainwaring, R. D., Reddy, V. M., Reinhartz, O., Lamberti, J. J., Jacobson, J. G., Jimenez, D. L., Hanley, F. L. WILEY-BLACKWELL PUBLISHING, INC. 2008: 622–26

  Abstract

  There will be a continuing need to start new congenital heart surgery programs to serve communities experiencing significant population growth. However, small congenital heart programs frequently underperform their larger counterparts. This study summarizes the clinical outcome data for the first 42 months of a small, start-up congenital heart surgery program. Clinical outcomes were summarized from the start of the program in September 2003 through March 2007. Risk adjustment analysis was performed using the risk adjustment in congenital heart surgery (Rachs-1) risk adjustment model and Society of Thoracic Surgeons (STS) congenital database. Three hundred eighty-six operations have been performed during this time period, including 47 newborns, 96 infants, 217 children/adolescents, and 26 adults (greater than 18 years). There have been two operative mortalities (0.5%). Assigning these cases to the Rachs-1 categories, there were 64 level I, 188 level II, 90 level III, 12 level IV cases, with 32 "others." The predicted mortality for the 354 categorized cases calculates to be 17.4 (Rachs-1) and 10.2 (STS). The data demonstrate that a start-up program with a relatively modest surgical volume can achieve satisfactory clinical results. This model has relied upon careful case selection and direct, senior-level surgeon involvement through an affiliation with a university-based program. These results suggest that a small congenital heart surgery program can be successfully started if the circumstances are carefully controlled.

  View details for DOI 10.1111/j.1540-8191.2008.00675.x

  View details for Web of Science ID 000260499400007

  View details for PubMedID 19016985

• Dynamics of human myocardial progenitor cell Populations in the neonatal period ANNALS OF THORACIC SURGERY Amir, G., Ma, X., Reddy, V. M., Hanley, F. L., Reinhartz, O., Ramamoorthy, C., Riemer, R. K. 2008; 86 (4): 1311-1320

  Abstract

  Pluripotent cardiac progenitor cells resident in myocardium offer a potentially promising role in promoting recovery from injury. In pediatric congenital heart disease (CHD) patients, manipulation of resident progenitor cells may provide important new approaches to improving outcomes. Our study goals were to identify and quantitate populations of progenitor cells in human neonatal myocardium during the early postnatal period and determine the proliferative capacity of differentiated cardiac myocytes.Immunologic markers of cell lineage (stage-specific embryonic antigen 4 [SSEA-4], islet cell antigen 1 [Isl1], c-kit, Nkx2.5, sarcoplasmic reticulum calcium-regulated ATPase type 2 [SERCA2]) and proliferation (Ki67) were localized in right ventricular biopsies from 32 CHD patients aged 2 to 93 days.Neonatal myocardium contains progenitor cells and transitional cells expressing progenitor and differentiated myocyte marker proteins. Some cells expressed the pluripotent cell marker c-kit and also coexpressed the myocyte marker SERCA2. Multipotent progenitor cells, identified by the expression of Isl1, were found. Ki67 was expressed in some myocytes and in nonmyocyte cells. A few cells expressing SSEA-4 and Isl1 were observed during the early postnatal period. Cells expressing c-kit, the premyocyte marker Nkx2.5, and Ki67 were found throughout the first postnatal month. A progressive decline in cell density during the first postnatal month was observed for c-kit+ cells (p = 0.0013) and Nkx2.5+ cells (p = 0.0001). The percentage of cells expressing Ki67 declined during the first 3 postnatal months (p = 0.0030).Cells in an incomplete state of cardiomyocyte differentiation continue to reside in the infant heart. However, the relative density of progenitor cells declines during the first postnatal month.

  View details for DOI 10.1016/j.athoracsur.2008.06.058

  View details for Web of Science ID 000259848000036

  View details for PubMedID 18805183

• Morphological studies of pulmonary arteriovenous shunting in a lamb model of superior cavopulmonary anastomosis PEDIATRIC CARDIOLOGY McMullan, D. M., Reddy, V. M., Gottliebson, W. M., Silverman, N. H., Perry, S. B., Chan, F., Hanley, F. L., Riemer, R. K. 2008; 29 (4): 706-712

  Abstract

  We sought to identify and characterize the abnormal vascular structures responsible for pulmonary arteriovenous shunting following the Glenn cavopulmonary shunt. Superior cavopulmonary shunt is commonly performed as part of the staged pathway to total cavopulmonary shunt to treat univentricular forms of congenital heart disease, however, clinically significant pulmonary arteriovenous malformations develop in some patients after the procedure. The causes of pulmonary arteriovenous malformations and other pulmonary vascular changes that occur after cavopulmonary shunt are not known. Using a juvenile lamb model of superior cavopulmonary anastomosis that reliably produces pulmonary arteriovenous malformations, we performed echocardiography and morphological analyses to determine the anatomic site of shunting and to identify the vascular structures involved. Pulmonary arteriovenous shunting was identified by contrast echocardiography in all surviving animals (n = 40) following superior cavopulmonary anastomosis. Pulmonary vascular corrosion casts revealed abnormal tortuous vessels joining pulmonary arteries and veins in cavopulmonary shunt animals but not control animals. In conclusion, unusual channels that bridged pulmonary arteries and veins were identified. These may represent the vascular structures responsible for arteriovenous shunting following the classic Glenn cavopulmonary shunt. Detailed analysis of these structures may elucidate factors responsible for their development.

  View details for DOI 10.1007/s00246-007-9152-5

  View details for Web of Science ID 000257393800003

  View details for PubMedID 18043856

  View details for PubMedCentralID PMC2970565

• Routine continuous perfusion for aortic arch reconstruction in the neonate. Seminars in thoracic and cardiovascular surgery. Pediatric cardiac surgery annual Malhotra, S. P., Hanley, F. L. 2008: 57-60

  Abstract

  Continuous perfusion has evolved over the past 15 years as a viable cerebral protection strategy for neonatal aortic arch reconstruction. It presents an attractive alternative to deep hypothermic circulatory arrest. However, because of its relatively recent development, a standardized technique for its application is lacking. Here we describe our approach for continuous perfusion for repairs of the aortic arch based on experience with over 700 cases.

  View details for DOI 10.1053/j.pcsu.2007.12.004

  View details for PubMedID 18396226

• Surgical management of coronary artery arising from the wrong coronary sinus, using standard and novel approaches 85th Annual Meeting of the American-Association-for-Thoracic-Surgery Gulati, R., Reddy, V. M., Culbertson, C., Helton, G., Suleman, S., Reinhartz, O., Silverman, N., Hanley, F. L. MOSBY-ELSEVIER. 2007: 1171–U12

  Abstract

  Patients with a coronary artery arising from the wrong sinus are susceptible to ischemia and sudden death. Risk is higher when the artery courses interarterially--between the pulmonary artery and aorta--has an intramural course, or has an abnormal orifice. In single coronary ostium without intramural course, unroofing and coronary reimplantation are inappropriate, and coronary artery bypass grafting is suboptimal. For this variant, we have devised pulmonary artery translocation.A retrospective review of 18 patients undergoing repair between January 1999 and March 2005 was performed. Mean age was 8.1 years (range 6 weeks-16 years). All anomalous arteries coursed interarterially. Ten patients had a right coronary artery from the left coronary sinus; 8 had a left coronary artery from the right sinus. Eleven had an intramural course, and 4 had a single coronary ostium without an intramural course. Ten (56%) patients had symptoms: chest pain (9/10), syncope (3/10), or dyspnea (2/10). Repair was implemented by unroofing (n = 11), reimplantation (n = 3), or pulmonary artery translocation (1 lateral, 3 anterior). All patients were followed up clinically and echocardiographically.At a mean of 2.2 years (2 weeks-5 years), there was no mortality. Symptoms improved and function remained normal in all but 1 patient. He had sustained multiple infarcts in the anomalous artery's distribution and required transplantation despite repair.Repair is indicated in all patients with coronary insufficiency and in asymptomatic patients with high-risk morphologic abnormalities. We recommend unroofing when an intramural component (or slit-like orifice) is present, reimplantation for separate ostia without an intramural course, and pulmonary artery translocation for single ostium without an intramural course. Coronary artery bypass grafting is thus avoided.

  View details for DOI 10.1016/j.jtcvs.2007.02.051

  View details for Web of Science ID 000250576200011

  View details for PubMedID 17976445

• Ductus-associated proximal pulmonary artery stenosis in patients with right heart obstruction INTERNATIONAL JOURNAL OF CARDIOLOGY Moon-Grady, A. J., Teltel, D. F., Hanley, F. L., Moore, P. 2007; 114 (1): 41-45

  Abstract

  Proximal pulmonary artery stenosis is a common acquired lesion in infants treated for congenital heart disease. We hypothesized that a large number of stenoses develop at the site of ductal insertion in patients with right ventricular outflow tract obstruction (RVOTO) and that these patients are at risk for developing hypoplasia of the ipsilateral pulmonary artery. The surgical and cardiac catheterization databases at our institution during the years 1988-2000 were searched for all patients under 1 year of age carrying a diagnosis of pulmonary atresia with intact ventricular septum (PA), tetralogy of Fallot (TOF) or pulmonary stenosis (PS), yielding 700 patients (62 PA, 373 TOF, 265 PS). The cardiac catheterization database was also searched for all patients with any diagnosis under 1 year of age found at catheterization to have proximal pulmonary artery stenosis. Proximal pulmonary artery stenosis associated with the ductal insertion site was diagnosed at catheterization in 33 infants (18 with PA, 5 with TOF, 6 with PS, 4 other diagnoses). This represents 29% of patients with PA, 1% with TOF and 2% with PS. Among patients with RVOTO and ductal insertion site-associated stenosis, there was a high prevalence (59%) of associated distal pulmonary arterial hypoplasia, defined as diameter of the stenosed vessel at first distal branch < or = 80% the diameter of the contralateral vessel. Symptomatology failed to identify this lesion; therefore, a high index of suspicion is necessary if proximal pulmonary artery stenosis is to be detected early in these patients.

  View details for DOI 10.1016/j.ijcard.2006.01.005

  View details for Web of Science ID 000243051900008

  View details for PubMedID 16644039

• Visual light spectroscopy reflects flow-related changes in brain oxygenation during regional low-flow perfusion and deep hypothermic circulatory arrest 31st Annual Meeting of the Western-Thoracic-Surgical-Association Amir, G., Ramamoorthy, C., Riemer, R. K., Davis, C. R., Hanley, F. L., Reddy, V. M. MOSBY-ELSEVIER. 2006: 1307–13

  Abstract

  Regional low-flow perfusion has been used to minimize ischemic brain injury during complex heart surgery in children. However, optimal regional low-flow perfusion remains undetermined. Visible light spectroscopy is a reliable method for continuous determination of capillary oxygen saturation (SgvO2). We used visible light spectroscopy to follow deep and superficial brain SgvO2 during cardiopulmonary bypass, regional low-flow perfusion, and deep hypothermic circulatory arrest.Visible light spectroscopy probes were inserted into the superficial and deep brain of neonatal (3.9-4.5 kg) piglets, targeting the caudate and thalamic nuclei. The piglets were subjected to cardiopulmonary bypass and cooled to a rectal temperature of 18 degrees C using pH stat. Regional low-flow perfusion was initiated through the innominate artery at 18 degrees C, and pump flows were adjusted to 40, 30, 20, and 10 mL/kg/min for 10-minute intervals followed by 30 minutes of deep hypothermic circulatory arrest. Regional low-flow perfusion was reestablished, and flows were increased in a stepwise manner from 10 to 40 mL/kg/min. SgvO2 was continuously monitored. Carotid flow was measured using a flow probe, and cerebral blood flow (milliliters per kilogram body weight per minute) was calculated.There were no significant differences between the deep and superficial brain tissue oxygenation during regional low flow brain perfusion before deep hypothermic circulatory arrest. However, after deep hypothermic circulatory arrest, the superficial brain SgvO2 was lower than the deep brain SgvO2 (24 +/- 12 vs 55.3 +/- 8, P = .05, at flows of 30 mL/kg/min, and 34.2 +/- 17 vs 62.5 + 8, P = .06, at a flow rate of 40 mL/kg/min). During regional low-flow perfusion, SgvO2 was maintained at flows of 30 to 40 mL/kg/min (cerebral blood flows of 15 to 21 mL/kg/min and 19 to 24 mL/kg/min, respectively), but was significantly lower at pump flows of 20 mL/kg/min (cerebral blood flow of 10 to 14 mL/kg/min) and 10 mL/kg/min (cerebral blood flow of 5 to 9 mL/kg/min) compared with the values obtained just before regional low-flow perfusion (pre-deep hypothermic circulatory arrest, 37 +/- 6 vs 65.5 +/- 4.4, P < .05, and 21.6 +/- 3.7 vs 65.5 +/- 4.4, P < .01, respectively; and post-deep hypothermic circulatory arrest, 32 +/- 4.5 vs 65.5 +/- 4.4, P < .05, and 16.6 +/- 4.7 vs 65.5 +/- 4.4, P < .01, respectively).Regional low-flow perfusion at pump flows of 30 to 40 mL/kg/min with resulting cerebral blood flows of 14 to 24 mL/kg/min was adequate in maintaining both deep and superficial brain oxygenation. However, lower pump flows of 20 and 10 mL/kg/min, associated with cerebral blood flow of 9 to 14 mL/kg/min, resulted in significantly reduced SgvO2 values.

  View details for DOI 10.1016/j.jtcvs.2006.04.056

  View details for Web of Science ID 000242626200012

  View details for PubMedID 17140947

• The extracardiac conduit Fontan operation using minimal approach extracorporeal circulation: Early and midterm outcomes 85th Annual Meeting of the American-Association-for-Thoracic-Surgery Petrossian, E., Reddy, V. M., Collins, K. K., Culbertson, C. B., MacDonald, M. J., Lamberti, J. J., Reinhartz, O., Mainwaring, R. D., Francis, P. D., Malhotra, S. P., Gremmels, D. B., Suleman, S., Hanley, F. L. MOSBY-ELSEVIER. 2006: 1054–63

  Abstract

  Our approach to the extracardiac conduit Fontan operation has evolved over time from full-pump, to partial-pump, to completely off-pump. This study is designed to report our overall experience with the extracardiac conduit Fontan operation and to evaluate the evolution in bypass technique on postoperative outcomes.From September 1992 to April 2005, 285 patients, median age 4.5 years (1.4-44 years), median weight 16 kg (9.4-94 kg), underwent a primary extracardiac conduit Fontan procedure. Early and late outcomes were analyzed for the entire cohort and for 2 patient groups depending on whether an oxygenator was used in the bypass circuit (166 patients; 58%) or not (119 patients; 42%).Early failure (including death and takedown) occurred in 7 patients (2.5%). Prevalence of new early postoperative sinus node dysfunction necessitating a permanent pacemaker was 0.4%, and that of new tachyarrhythmias necessitating discharge home on a regimen of antiarrhythmia medications was 2.5%. Ten-year actuarial freedom from Fontan failure, new sinus node dysfunction necessitating a permanent pacemaker, and reoperation for conduit thrombosis or stenosis was 90%, 96%, and 98%, respectively. Fenestration rate was lower (P = .001) in the no-oxygenator group (8%) than in the oxygenator group (25%). Patients in the no-oxygenator group had lower intraoperative Fontan pressure (12.0 +/- 2.3 vs 13.5 +/- 2.4 mm Hg, P < .001), common atrial pressure (4.6 +/- 1.8 vs 5.3 +/- 1.8 mm Hg, P = .003), and transpulmonary gradient (7.5 +/- 2.1 vs 8.3 +/- 2.2 mm Hg, P = .013) than did the oxygenator group.The extracardiac conduit Fontan operation coupled with minimal use of extracorporeal circulation is associated with favorable intraoperative hemodynamics, low fenestration rate, minimal risk of thrombosis or stenosis, and minimal early and late rhythm disturbance.

  View details for DOI 10.1016/j.jtcvs.2006.05.066

  View details for PubMedID 17059923

• Esophageal saturation during antegrade cerebral perfusion: a preliminary report using visible light spectroscopy PEDIATRIC ANESTHESIA Heninger, C., Ramamoorthy, C., Amir, G., Kamra, K., Reddy, V. M., Hanley, F. L., Brock-Utne, J. G. 2006; 16 (11): 1133-1137

  Abstract

  Visible light spectroscopy (VLS) is newer technology that measures real-time tissue oxygenation. It has been validated in detecting mucosal ischemia in adults. During complex neonatal heart surgery, antegrade cerebral perfusion (ACP) maintains cerebral saturation. Whether ACP maintains peripheral tissue perfusion in humans is not known.Five patients undergoing neonatal open heart surgery with hypothermic cardiopulmonary bypass (CPB) were studied using a VLS esophageal probe in addition to bilateral near infrared cerebral oximetry. Three of five patients required ACP for arch repair, while two patients did not. VLS and cerebral saturation data were collected and analyzed in 5 min intervals prior to CPB, during CPB, and during ACP.In the two patients undergoing heart surgery with routine hypothermic CPB, both cerebral and esophageal saturations were maintained. However in all three neonates requiring ACP, although cerebral saturations did not decrease, esophageal saturation fell below the ischemic threshold (35%). Following establishment of normal CPB, esophageal saturation returned to baseline.Antegrade cerebral perfusion maintains cerebral oxygen delivery, however, it does not adequately perfuse the esophagus in neonates. This could have clinical implications.

  View details for DOI 10.1111/j.1460-9592.2006.01965.x

  View details for Web of Science ID 000241245400004

  View details for PubMedID 17040301

• Aortopulmonary window with anomalous origin of the right coronary artery from the pulmonary artery: Two cases highlighting the importance of complete pre-operative echocardiographic evaluation of the coronary arteries in all conotruncal anomalies EUROPEAN JOURNAL OF ECHOCARDIOGRAPHY Greenway, S. C., Bradley, T. J., Caldarone, C. A., Silverman, N. H., Hanley, F. L., Smallhorn, J. F. 2006; 7 (5): 379-382

  Abstract

  This report describes two infants with an aortopulmonary window in association with anomalous origin of the right coronary artery from the pulmonary artery. In both cases the diagnosis was made pre-operatively by transthoracic echocardiography, with the initial clue being extensive collateral flow within the myocardium. In each case there was surgical confirmation of the echocardiographic findings. These two cases demonstrate that coronary artery evaluation should be an integral part of every new echocardiographic evaluation, particularly in the setting of conotruncal anomalies.

  View details for DOI 10.1016/j.euje.2005.10.010

  View details for Web of Science ID 000242966600007

  View details for PubMedID 16356774

• Coarctation of the aorta in the right aortic arch with left aberrant innominate artery PEDIATRIC CARDIOLOGY Bein, S., Saba, Z., Patel, H., Reinhartz, O., Hanley, F. L. 2006; 27 (5): 621-623

  Abstract

  Right aortic arch with an aberrant left innominate artery is a rare combination. We report a unique case of coarctation of the aorta in a patient with right aortic arch and aberrant left innominate artery. The diverticulum of Kommerell gave rise to the left innominate artery and a left ligamentum arteriosum, completing a vascular ring. This unusual case raises interesting points regarding the development of coarctation in the context of a right-sided arch and the approach to management of these patients.

  View details for DOI 10.1007/s00246-006-1327-y

  View details for Web of Science ID 000240913400017

  View details for PubMedID 16933062

• Unifocalization of major aortopulmonary collaterals in single-ventricle patients 42nd Annual Meeting of the Society-of-Thoracic-Surgeons Reinhartz, O., Reddy, V. M., Petrossian, E., Suleman, S., Mainwaring, R. D., Rosenthal, D. N., Feinstein, J. A., Gulati, R., Hanley, F. L. ELSEVIER SCIENCE INC. 2006: 934–39

  Abstract

  Unifocalization of major aortopulmonary collateral arteries (MAPCAs) in pulmonary atresia with ventricular septal defect and intracardiac repair has become the standard of care. However, there are no reports addressing unifocalization of MAPCAs in single-ventricle patients. It is unknown whether their pulmonary vascular bed can be reconstructed and low enough pulmonary vascular resistance achieved to allow for superior or total cavopulmonary connections.We reviewed data on all patients with functional single ventricles and unifocalization procedures of MAPCAs. From 1997 to 2005, 14 consecutive children with various single-ventricle anatomies were operated on.Patients had a median of three surgical procedures (range, 1 to 5). Two patients had absent, all others diminutive central pulmonary arteries, with an average of 3.5 +/- 1.2 MAPCAs. Seven patients (50%) had bidirectional Glenn procedures, and 3 of these had Fontan procedures. Median postoperative pulmonary artery pressures measured 12.5 mm Hg (Glenn) and 14 mm Hg (Fontan), respectively. Six patients are alive today (46%), with 1 patient lost to follow-up. Three patients died early and 3 late after initial unifocalization to shunts. One other patient survived unifocalization, but was not considered a candidate for a Glenn procedure and died after high-risk two-ventricle repair. Another patient with right-ventricle-dependent coronary circulation died of sepsis late after Glenn.In selected patients with functional single ventricles and MAPCAs, the pulmonary vascular bed can be reconstructed sufficiently to allow for cavopulmonary connections. Venous flow to the pulmonary vasculature decreases cardiac volume load and is likely to increase life expectancy and quality of life for these patients.

  View details for DOI 10.1016/j.athoracsur.2006.03.063

  View details for PubMedID 16928512

• Homograft valved right ventricle to pulmonary artery conduit as a modification of the Norwood procedure 78th Annual Scientific Session of the American-Heart-Association Reinhartz, O., Reddy, V. M., Petrossian, E., Macdonald, M., Lamberti, J. J., Roth, S. J., Wright, G. E., Perry, S. B., Suleman, S., Hanley, F. L. LIPPINCOTT WILLIAMS & WILKINS. 2006: I594–I599

  Abstract

  The use of a right ventricle to pulmonary artery (RV-PA) conduit in the Norwood procedure has been proposed to increase postoperative hemodynamic stability. A valve within the conduit should further decrease RV volume load. We report our clinical experience with this modification.From February 2002 through August 2005, we performed 88 consecutive Norwood procedures using RV-PA conduits. We used composite valved conduits made from cryopreserved homograft and polytetrafluoroethylene (PTFE) in 66 cases (54 pulmonary, 12 aortic homografts), other valved conduits in 14, and unvalved PTFE in 8 cases. Hospital survival was 88.6% overall and increased to 93.1% after the initial year. Early interventions were required in 18 patients (16 for cyanosis). Prestage II cardiac catheterization was performed at a mean age of 126 days. Mean Qp/Qs was 1, with mean aortic saturation 71%, mean O2 extraction 24%, and mean right ventricular end-diastolic pressure 9 mm Hg. Patient weight, use of an aortic homograft valve in the conduit, stage I palliation within the first year of our experience, and low O2 extraction and high transpulmonary gradient prestage II were risk factors for overall death. Early interventions were more frequent in aortic valve conduits compared with all other conduits.The valved RV-PA conduit was associated with low early mortality after the Norwood procedure. The majority of these patients had normal cardiac output and well-maintained RV function. There may be a higher risk for early conduit interventions and death when aortic valve homografts are used in the RV-PA conduit.

  View details for PubMedID 16820644

• MAPCAs, bronchials, monkeys, and men EUROPEAN JOURNAL OF CARDIO-THORACIC SURGERY Hanley, F. L. 2006; 29 (5): 643-644

  View details for DOI 10.1016/j.ejcts.2006.02.031

  View details for Web of Science ID 000237923200001

  View details for PubMedID 16626963

• Profile of serum S-100 beta levels during maturation in fetal and neonatal sheep AMERICAN JOURNAL OF PERINATOLOGY Parry, A. J., McMullan, D. M., Yelich, S., Hanley, F. L. 2006; 23 (3): 153-157

  Abstract

  Serum levels of the protein S-100beta are dependent on three factors: rate of production, permeability of the blood-brain barrier, and rate of clearance. In the developing fetus and neonate all of these factors change at different rates. This study was performed to determine how serum S-100 levels varied during fetal and early postnatal life. Blood samples were obtained from 41 fetal and neonatal lambs. The blood was separated in a centrifuge and the serum drawn off and assayed for S-100beta using a commercially available radioimunoassay kit. S-100beta did not appear in the blood until halfway through pregnancy. Thereafter, levels steadily increased until 1 month after birth. Following this, S-100beta levels decreased progressively until by 1 year of age, they had reached a plateau. S-100beta levels change significantly with normal fetal and neonatal maturation. Valid interpretation of other data from subjects of similar developmental stage must take into consideration this physiological variation.

  View details for DOI 10.1055/s-2006-931914

  View details for Web of Science ID 000236848700002

  View details for PubMedID 16586228

• Management of systemic venous anomalies in the pediatric cardiovascular surgical patient. Seminars in thoracic and cardiovascular surgery. Pediatric cardiac surgery annual Gandy, K., Hanley, F. 2006: 63-74

  Abstract

  Systemic venous anomalies are rare and heterogeneous entities. Although these anomalies are rare in the general population, they occur more frequently in the subpopulation with congenital heart disease. In and of themselves, most of these lesions have no physiologic significance. However, in the setting of congenital heart disease these lesions may significantly alter surgical treatment. This review is dedicated to these lesions.

  View details for PubMedID 16638550

• Effect of surgical case volume on outcome after the Norwood procedure JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY MacDonald, M. J., Reinhartz, O., Hanley, F. L. 2005; 130 (6): 1731-1731

  View details for DOI 10.1016/j.jtcvs.2005.06.028

  View details for Web of Science ID 000233896300046

  View details for PubMedID 16308032

• Neonatal brain protection and deep hypothermic circulatory arrest: pathophysiology of ischemic neuronal injury and protective strategies ANNALS OF THORACIC SURGERY Amir, G., Ramamoorthy, C., Riemer, K., Reddy, V. M., Hanley, F. L. 2005; 80 (5): 1955-1964

  Abstract

  Deep hypothermic circulatory arrest (DHCA) has been used for the past 50 years in the surgical repair of complex congenital cardiac malformations and operations involving the aortic arch; it enables the surgeon to achieve precise anatomical reconstructions by creating a bloodless operative field. Nevertheless, DHCA has been associated with immediate and late neurodevelopmental morbidities. This review provides an overview of the pathophysiology of neonatal hypoxic brain injury after DHCA, focusing on cellular mechanisms of necrosis, apoptosis, and glutamate excitotoxicity. Techniques and strategies in neonatal brain protection include hypothermia, acid base blood gas management during cooling, and pharmacologic interventions such as the use of volatile anesthetics. Surgical techniques consist of intermittent cerebral perfusion during periods of circulatory arrest and continuous regional brain perfusion.

  View details for DOI 10.1016/j.athoracsur.2004.12.040

  View details for Web of Science ID 000232970500075

  View details for PubMedID 16242503

• Religion, politics... deep hypothermic circulatory arrest JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY Hanley, F. L. 2005; 130 (5): 1236-1241

  View details for DOI 10.1016/j.itcvs.2005.07.047

  View details for Web of Science ID 000233120100001

  View details for PubMedID 16256773

• Homograft valved right ventricle to pulmonary artery conduit as a modification of the norwood procedure 78th Annual Scientific Session of the American-Heart-Association Reinhartz, O., Reddy, V. M., MacDonald, M. J., Lamberti, J. J., Petrossian, E., Perry, S. B., Suleman, S., Hanley, F. L. LIPPINCOTT WILLIAMS & WILKINS. 2005: U577–U577

  View details for Web of Science ID 000232956403391

• Management of major aortopulmonary collateral arteries 78th Annual Scientific Session of the American-Heart-Association Hanley, F. L., Reinhartz, O., Suleman, S., Gremmels, D. B., Perry, S. B., Feinstein, J. A., Reddy, V. M. LIPPINCOTT WILLIAMS & WILKINS. 2005: U576–U576

  View details for Web of Science ID 000232956403388

• Isolated unilateral absence of right proximal pulmonary artery: Surgical repair and follow-up ANNALS OF THORACIC SURGERY Welch, K., Hanley, F., Johnston, T., Cailes, C., Shah, M. J. 2005; 79 (4): 1399-1402

  Abstract

  The isolated unilateral absence of a proximal pulmonary artery is a rare congenital lesion with a diverse clinical presentation. If the connecting ductus arteriosus closes after birth, the ipsilateral pulmonary artery will lose its source of blood supply, resulting in hypoplasia or obliteration of intrapulmonary vessels. Despite a seemingly benign early clinical course, a significant number of untreated patients will develop pulmonary hypertension, hemoptysis, and recurrent respiratory infections. Early detection and surgical repair provides restoration of physiologic pulmonary circulation, regression of pulmonary hypertension, and the potential for normal distal pulmonary vascular development.

  View details for DOI 10.1016/j.athoracsur.2003.10.037

  View details for Web of Science ID 000228091100052

  View details for PubMedID 15797092

• Preliminary results of fetal cardiac bypass in nonhuman primates 84th Annual Meeting of the American-Association-for-Thoracic-Surgery Ikai, A., Riemer, R. K., Ramamoorthy, C., Malhatra, S., Cassorla, L., Amir, G., Hanley, F. L., Reddy, V. M. MOSBY-ELSEVIER. 2005: 175–81

  Abstract

  Fetal cardiac surgery has potential benefits for treatment of some congenital heart defects. However, placental dysfunction as a result of fetal bypass, fetal stress, and fetal exposure to external milieu needs to be overcome to optimize the outcomes of fetal cardiac bypass. In this study we evaluated the technical feasibility of cardiac bypass in the nonhuman primate fetus and the efficacy of different anesthetic approaches.Twelve baboon fetuses, average gestation 146 +/- 8 days and weight 696 +/- 184 g, were used. Three fetuses were excluded from the study because of nuchal cord presentations. The animals were separated into two anesthesia groups: isoflurane (n = 6) and fentanyl and midazolam (n = 3). A miniature roller pump circuit without oxygenator was used for fetal bypass for 30 minutes. No blood transfusion was performed. Fetal blood gas samples were collected before bypass, during bypass, and at 15 and 60 minutes after bypass.All fetuses in the isoflurane group were successfully placed on the cardiac bypass circuit. However, 2 animals in the fentanyl and midazolam group were not placed on the bypass circuit because of sustained elevation in maternal uterine tone. All maternal baboons survived. Of the 6 fetuses in the isoflurane group, 5 survived for 60 minutes; however, placental function continued to deteriorate after bypass (Pa o 2 33 +/- 3 mm Hg before bypass, 23 +/- 6 mm Hg 15 minutes after, and 18 +/- 9 mm Hg 60 minutes after).The technical feasibility of cardiac bypass in nonhuman primate fetuses weighing less than 1000 g was confirmed. Isoflurane anesthesia appears to be superior to fentanyl and midazolam anesthesia for fetal cardiac surgery because of adequate uterine relaxation.

  View details for DOI 10.1016/j.jtcvs.2004.09.003

  View details for Web of Science ID 000226216600024

  View details for PubMedID 15632840

• Pulmonary arteriovenous shunting in the normal fetal lung JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY McMullan, D. M., Hanley, F. L., Cohen, G. A., Portman, M. A., Riemer, R. K. 2004; 44 (7): 1497-1500

  Abstract

  We hypothesized that pulmonary arteriovenous shunting (PAVS) is normally present in fetal lungs and that cavopulmonary anastomosis-induced PAVS may represent a return to an earlier morphologic stage of development.The surgical superior cavopulmonary anastomosis is performed as part of the staged Fontan pathway to treat univentricular forms of congenital heart disease; PAVS is a known sequela after superior cavopulmonary anastomosis and may have important clinical consequences. Although the etiology and true morphology of the structures responsible for PAVS are unknown, a leading theory is that PAVS is caused by absence of normal hepatic venous drainage to the pulmonary circulation.To determine whether normal fetal lungs demonstrate PAVS, we performed contrast echocardiograms on 13 fetal lambs, 8 neonatal lambs, 4 juvenile lambs, and 4 adult sheep using a blended mixture of saline and blood injected directly into the proximal pulmonary artery.Pulmonary arteriovenous shunting was detected by direct epicardial echocardiography in all fetal lambs (n = 13) and neonatal animals studied at one and three days of life (n = 4) and in two of four animals studied at six to nine days of life. Pulmonary arteriovenous shunting was not present in animals studied at four weeks of life (n = 2) and in adult sheep (n = 5).These studies demonstrate that PAVS is normally present in late gestation fetal and early neonatal lambs but then disappears during the later neonatal period. Furthermore, these findings suggest that PAVS associated with cavopulmonary anastomosis or other processes affecting the developing pulmonary circulation may represent a return to an earlier morphologic stage of development.

  View details for DOI 10.1016/j.jacc.2004.06.064

  View details for Web of Science ID 000224225600025

  View details for PubMedID 15464334

• Pulmonary expression of the hepatocyte growth factor receptor c-Met shifts from medial to intimal layer after cavopulmonary anastomosis 83rd Annual Meeting of the American-Association-for-Thoracic-Surgery Ikai, A., Riemer, R. K., Ma, X. Y., Reinhartz, O., Hanley, F. L., Reddy, V. M. MOSBY-ELSEVIER. 2004: 1442–49

  Abstract

  Pulmonary arteriovenous malformations occur in up to 60% of patients after cavopulmonary anastomosis. We compared the effects of cavopulmonary anastomosis and pulmonary artery banding on lung gene expression in an ovine model to study the abnormal pulmonary vascular remodeling after the exclusion of inferior vena caval blood independent of reduced pulmonary blood flow. We previously demonstrated by contrast echocardiography that pulmonary arteriovenous malformations develop by 8 weeks after cavopulmonary anastomosis but not after pulmonary artery banding. Hepatocyte growth factor, a pleiotropic factor with morphogenic, mitogenic, and angiogenic activities, signals via its specific receptor c-Met to induce the antiapoptotic factor Bcl-2. In this study, we examined pulmonary artery expression of these factors and their potential role in pulmonary artery remodeling after cavopulmonary anastomosis and pulmonary artery banding.Eighteen lambs aged 35 to 45 days were placed into 3 groups: cavopulmonary anastomosis, pulmonary artery banding, and control (n = 6/group). In the cavopulmonary anastomosis group, the superior vena cava was anastomosed to the right pulmonary artery in an end-to-end fashion. In the pulmonary artery banding group, the left pulmonary artery was banded to reduce blood flow to 20% of control. The control group had a simple right pulmonary artery clamp for 30 minutes. Lung was harvested for Western blot, reverse transcriptase-polymerase chain reaction, and immunostaining at 2 weeks (n = 3/group) and 5 weeks (n = 3/group) after surgery.The expression of c-Met mRNA after cavopulmonary anastomosis was increased by twofold compared with the control or pulmonary artery banding group. The total lung expression of c-Met by Western blot was also up regulated at 2 weeks (P <.05). However, total lung expression of hepatocyte growth factor and Bcl-2 by Western and reverse transcriptase-polymerase chain reaction was not different from the control and pulmonary artery banding groups at both 2 and 5 weeks after surgery. Immunohistochemical analysis revealed that c-Met expression was localized to the intimal layer of the pulmonary artery in the cavopulmonary anastomosis, while its expression in the control and pulmonary artery banding lungs was localized to the medial layer. Localization of Bcl-2 on the intimal layer in lambs with cavopulmonary anastomosis followed the same pattern as c-Met.After cavopulmonary anastomosis, pulmonary artery expression of the hepatocyte growth factor receptor c-Met and one of its downstream effectors, Bcl-2, had increased in the intimal layer and decreased in the medial layer. Because the hepatocyte growth factor signaling promotes increased endothelial cell survival, it may have a role in pulmonary artery remodeling following cavopulmonary anastomosis. In addition, the change of c-Met expression in the medial layer after cavopulmonary anastomosis suggests a possible mechanism for the smooth muscle cell alteration related to abnormal angiogenesis.

  View details for DOI 10.1016/j.jtcvs.2003.09.009

  View details for Web of Science ID 000221134600031

  View details for PubMedID 15116006

• A method for selectively limiting lumen diameter in corrosion casting MICROVASCULAR RESEARCH McMullan, D. M., Hanley, F. L., Riemer, R. K. 2004; 67 (3): 215-217

  Abstract

  Corrosion casting is a technique frequently used to evaluate the form and spatial relationship of three dimensional biological structures, such as vascular networks, in vitro [Scann. Microsc. 5 (1991) 1097; Schraufnagel, D.E. The lung microstructure. In: Motta, P.M., Murakami, T. Fujita, H. eds. Scanning Electron Microscopy of Vascular Casts: Methods and Applications. Boston: Kluwer Academic Publishers; 1992:123-137]. However, because corrosion casts tend to reproduce the complexity of surrounding vascular structures as well as the structures of interest, the use of this technique in highly complex vascular systems may obscure important changes such as A-V shunts because they may get lost among the myriad of capillaries. We developed a novel modification for creating vascular corrosion casts by utilizing polystyrene microspheres to selectively embolize normal capillary networks and thereby reduce the overall complexity of the cast. his technique may aid investigators in the evaluation of a variety of vascular beds and is useful in demonstrating non-capillary arteriovenous communications.

  View details for Web of Science ID 000221443700002

  View details for PubMedID 15121445

• Electrical resychronization of failing right ventricle - Response CIRCULATION Dubin, A. M., Feinstein, J. A., Van Hare, G. F., Rosenthal, D. N., Reddy, V. M., Hanley, F. L. 2004; 109 (2): E5-E5

  View details for Web of Science ID 000188250500030

• Effect of enalaprilat on postoperative hypertension after surgical repair of coarctation of the aorta. Pediatric critical care medicine Rouine-Rapp, K., Mello, D. M., Hanley, F. L., Mohan Reddy, V., Soifer, S. 2003; 4 (3): 327-332

  Abstract

  Hypertension in pediatric patients after surgical repair of coarctation of the aorta can be difficult to control and may lead to morbidity. The renin-angiotensin system mediates at least part of this hypertension. Enalaprilat, the only intravenous angiotensin-converting enzyme inhibitor, is used to treat hypertension in pediatric patients in other settings. However, its effect on postoperative hypertension during the early postoperative period in patients undergoing surgical repair of coarctation of the aorta is unknown.Prospective, randomized, double-blind study.Operating room and the pediatric intensive care unit.Fourteen consecutive pediatric patients between the ages of 1 and 18 yrs scheduled to undergo surgical repair of coarctation of the aorta.Patients were randomized to receive enalaprilat or saline placebo. Infusions were begun intraoperatively within 15 mins of aortic repair and repeated every 6 hrs.Plasma renin activity was measured at baseline and on postoperative day 1. Blood pressure was determined at 30 mins and at 2, 4, and 6 hrs after infusion and scored relative to the preoperative blood pressure. The blood pressure in the enalaprilat group was consistently lower at 30 mins, 2 hrs, and 4 hrs after infusion (p <.05), but not at 6 hrs. Plasma renin activity was significantly lower in the placebo group on postoperative day 1. Length of stay in the pediatric intensive care unit trended shorter in the treated group.Conclusions are limited by a small cohort. Angiotensin-converting enzyme inhibitor therapy resulted in improved blood pressure control after coarctation repair. Further improvement of blood pressure control may be achievable by use of a larger dose of enalaprilat or a 4-hr enalaprilat-dosing interval.

  View details for PubMedID 12831415

• Induced fibrillation is equally effective as crystalloid cardioplegia in the protection of fetal myocardial function 82nd Annual Meeting of the American-Association-for-Thoracic-Surgery Malhotra, S. P., Thelitz, S., Riemer, R. K., Reddy, V. M., Suleman, S., Hanley, F. L. MOSBY-ELSEVIER. 2003: 1276–82

  Abstract

  Fetal cardiac intervention represents a potential advance in the treatment of congenital cardiac lesions that increase in complexity during development. Prenatal repair of a primary defect might prevent pathologic blood-flow patterns that can result in hypoplasia of a cardiac chamber or great vessel. However, strategies to optimize fetal myocardial protection have not been studied. A biventricular working fetal heart preparation was used to evaluate the cardioprotective properties of induced fibrillation and crystalloid cardioplegia.Hearts from 16 fetal lambs at 115 to 125 days' gestation were harvested and perfused with Krebs-Henseleit solution. The descending aorta was ligated distal to the ductal insertion and the branch pulmonary arteries were ligated to simulate the parallel circulation of the fetus. Hearts were arrested with normothermic fibrillation (n = 8) or hypothermic crystalloid cardioplegia (n = 8) before reperfusion with Krebs-Henseleit solution. Baseline and postarrest myocardial function measurements were obtained from analysis of pressure-dimension relationships.Fibrillatory and cardioplegic arrest were equally effective at preserving postarrest systolic function (left ventricle, 70% +/- 5% vs 68% +/- 15%, P =.52; right ventricle, 68% +/- 4.5% vs 65% +/- 4.5%, P =.26) and preventing increased diastolic stiffness (left ventricle, 32% +/- 5.3% vs 38% +/- 11%, P =.24; right ventricle, 25% +/- 3.3% vs 27% +/- 2.1%, P =.46). Myocardial water content was unchanged in hearts arrested with fibrillation and cardioplegia (84% +/- 1.5% vs 83.7% +/- 0.9%, P =.71).Normothermic fibrillation and hypothermic crystalloid cardioplegia provide equal protection of the fetal myocardium. In the setting of diminished fetal myocardial reserve and because of the limited ability to manipulate the surrounding temperature in the fetus, normothermic fibrillation may be preferable for in utero repairs of selected congenital heart defects.

  View details for DOI 10.1016/S0022-5223(02)73245-5

  View details for Web of Science ID 000183864700012

  View details for PubMedID 12830044

• Fetal myocardial protection is markedly improved by reduced cardioplegic calcium content ANNALS OF THORACIC SURGERY Malhotra, S. P., Thelitz, S., Riemer, R. K., Reddy, V. M., Suleman, S., Hanley, F. L. 2003; 75 (6): 1937-1941

  Abstract

  Fetal cardiac surgery holds a clear therapeutic benefit in the treatment of lesions that increase in complexity due to pathologic blood flow patterns during development. Fetal and neonatal myocardial physiology differ substantially, particularly in the regulation of myocardial calcium concentration. To examine issues of calcium homeostasis and fetal myocardial protection, a novel isolated biventricular working fetal heart preparation was developed.Hearts from 20 fetal lambs, 115 to 125 days gestation, were harvested and perfused with standard Krebs-Henseleit (K-H) solution. The descending aorta was ligated distal to the ductal insertion and the branch pulmonary arteries were ligated to mimic fetal cardiovascular physiology. Hearts were arrested for 30 minutes with normocalcemic (n = 8), hypocalcemic (n = 6), or hypercalcemic (n = 6) cold crystalloid cardioplegia before reperfusion with K-H solution.Compared with normocalcemic cardioplegia, hypocalcemic cardioplegia improved preservation of left ventricular (LV) systolic function (88% +/- 2.2% vs 64% +/- 15% recovery of end-systolic elastance, p = 0.02), diastolic function (12% +/- 21% vs 38% +/- 11% increase in end-diastolic stiffness, p = 0.04), and myocardial contractility (97% +/- 9.6% vs 75.2% +/- 13% recovery of preload recruitable stroke work [PRSW], p = 0.04). In contrast, the fetal myocardium was sensitive to hypercalcemic arrest with poor preservation of LV systolic function (37.5% +/- 8.4% recovery of elastance), diastolic function (86% +/- 21% increased stiffness), and overall contractility (32% +/- 13% recovery of PRSW). Myocardial water content was reduced in hearts arrested with hypocalcemic cardioplegia (79% +/- 1.8% vs 83.7% +/- 0.9%, p = 0.0006).This study demonstrates the sensitivity of the fetal myocardium to cardioplegic calcium concentration. Hypocalcemic cardioplegia provides superior preservation of systolic, diastolic, and contractile function of the fetal myocardium.

  View details for Web of Science ID 000183311400054

  View details for PubMedID 12822639

• Electrical resynchronization - A novel therapy for the failing right ventricle CIRCULATION Dubin, A. M., Feinstein, J. A., Reddy, V. M., Hanley, F. L., Van Hare, G. F., Rosenthal, D. N. 2003; 107 (18): 2287-2289

  Abstract

  Many patients with congenital heart disease develop right ventricular (RV) failure due to anatomy and prior therapy. RV problems may include right bundle-branch block (RBBB), volume loading, and chamber enlargement. Because the failing RV may have regional dyskinesis, we hypothesized that resynchronization therapy might augment its performance.We studied 7 patients with RV dysfunction and RBBB, using a predefined pacing protocol. QRS duration, cardiac index (CI), and RV dP/dt were measured in 4 different pacing states. Atrioventricular pacing improved CI and RV dP/dtmax and decreased QRS duration as compared with atrial pacing or sinus rhythm.Atrioventricular pacing in patients with RBBB and RV dysfunction augments RV and systemic performance. RV resynchronization is a promising novel therapy for patients with RV failure.

  View details for DOI 10.1161/01.CIR.0000070930.33499.9F

  View details for PubMedID 12732607

• Reliability of intraoperative contrast transesophageal echocardography for detecting interatrial communications in patients with other congenital cardiovascular malformations AMERICAN JOURNAL OF CARDIOLOGY Cassorla, L., Miller-Hance, W. C., Rouine-Rapp, K., Reddy, V. M., Hanley, F. L., Silverman, N. H. 2003; 91 (8): 1027-1031

  View details for DOI 10.1016/S0002-9149(03)00134-6

  View details for Web of Science ID 000182215300030

  View details for PubMedID 12686358

• Robotic-assisted endoscopic thoracic aortic anastomosis in juvenile lambs 5th Annual Meeting of the International-Society-for-Minimally-Invasive-Cardiac-Surgery Malhotra, S. P., Le, D., Thelitz, S., Hanley, F. L., Riemer, R. K., Suleman, S., Reddy, V. M. FORUM MULTIMEDIA PUBLISHING, LLC. 2003: 38–42

  Abstract

  Advances in robotic technology have enabled a wider range of applications for minimally invasive techniques in cardiac surgery, including mitral valve repair and coronary artery bypass grafting. With increased technical sophistication, robotic-assisted techniques can be developed for the endoscopic repair of certain congenital cardiac lesions.The purpose of this study was to assess the feasibility of closed chest thoracic aortic anastomosis in a juvenile ovine model.Lambs, aged 45 to 55 days, underwent surgery that was performed using the da Vinci robotic surgical system. Using 3 ports, the surgeon dissected the descending thoracic aorta and mobilized it free from attachments, using single-lung ventilation and CO2 insufflation. Snares were introduced through 2 stab wounds for aortic occlusion proximally and distally. In 4 lambs, the aorta was completely transected and reanastomosed using interrupted nitinol sutures. One lamb underwent longitudinal aortotomy, and patch aortoplasty was performed with the placement of a Gore-Tex patch. Snares were released and the animals were recovered once hemodynamically stable. Animals were sacrificed at 6 to 12 hours after surgery and the descending aorta was harvested. Burst-pressure testing was performed on the anastomoses.All 5 lambs survived the procedure with stabilization of hemodynamic parameters following surgery. The mean aortic clamp time was 47 +/- 17 minutes, and the anastomosis was completed in 26 +/- 5 minutes. The mean burst pressure was 163 +/- 9 mm Hg.Endoscopic thoracic aortic anastomosis can be performed safely and with adequate exposure in a juvenile large-animal model using computer-assisted surgical techniques. With further refinements, these approaches could be applied to the repair of congenital anomalies of the aorta, including interrupted aortic arch and aortic coarctation.

  View details for Web of Science ID 000181762100006

• The evolving management of aortic arch obstruction with ventricular septal defect. Outcomes of two-stage, circulatory arrest, and low-flow cerebral perfusion approaches American-Heart-Association Abstracts From Scientific Sessions Malhotra, S. P., Suleman, S., Hanley, F. L., Reddy, V. M. LIPPINCOTT WILLIAMS & WILKINS. 2002: 395–95

  View details for Web of Science ID 000179142702003

• Intermediate term follow-up of the end-to-side aortic anastomosis for coarctation of the aorta ANNALS OF THORACIC SURGERY Younoszai, A. K., Reddy, V. M., Hanley, F. L., Brook, M. M. 2002; 74 (5): 1631-1634

  Abstract

  Classic techniques for repairing coarctation of the aorta, especially in neonates, have a significant incidence of recurrent obstruction. By connecting the descending aorta to the proximal aortic arch, the end-to-side aortic anastomosis isolates hypoplastic distal arch and encroaching ductal tissue from the anastomotic site.Follow-up data were available for 88 patients (54 male) who underwent an end-to-side aortic anastomosis from November 1992 until November 1999. The median postoperative follow-up was 1.9 years (range, 0.1 to 6.3 years). Fifty-four patients were corrected as neonates. Thirty-four patients were operated on out of the neonatal period (> 1 month of age). A systolic blood pressure gradient > or = 20 mm Hg and a Doppler flow velocity > or = 2.5 ms across the area of repair were considered a recurrent obstruction.No patients in the pediatric group had a recurrent obstruction. In the neonatal group, 3 patients (5.5%) had recurrent obstruction. Of those, 2 patients had a reintervention performed; one reintervention was a balloon angioplasty and the other one was a reoperation. Kaplan-Meier analysis of the neonatal group revealed a 95.8% freedom from reintervention at 1 and 2 years.The end-to-side aortic anastomosis is an effective repair for coarctation of the aorta. Even when performed in the neonatal period, recurrence of coarctation is rare.

  View details for Web of Science ID 000179262300044

  View details for PubMedID 12440621

• The role of oxidative stress in the development of pulmonary arteriovenous malformations after cavopulmonary anastomosis JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY Malhotra, S. P., Reddy, V. M., Thelitz, S., He, Y. P., McMullan, D. M., Hanley, F. L., Riemer, R. K. 2002; 124 (3): 479-485

  Abstract

  Cavopulmonary anastomosis is used for palliation of cyanotic heart disease. Clinically significant pulmonary arteriovenous malformations occur in up to 25% of patients after surgical intervention. Cavopulmonary anastomosis creates several modifications to pulmonary physiology that may contribute to the development of pulmonary arteriovenous malformations, including reduced pulmonary blood flow and the exclusion of inferior vena caval effluent.By comparing the expression of angiogenic and stress-related proteins after cavopulmonary anastomosis and pulmonary artery banding, we sought to determine which genes were upregulated independent of reduced pulmonary blood flow.Lambs aged 35 to 45 days were placed into 1 of 3 groups: cavopulmonary anastomosis (n = 6), pulmonary artery banding (n = 6), and sham control (n = 6) animals. In our model pulmonary arteriovenous malformations are detectable by means of bubble-contrast echocardiography 8 weeks after cavopulmonary anastomosis. Lung tissue was harvested for Western blotting at 2 and 5 weeks after surgery.Cavopulmonary anastomosis and pulmonary artery banding both increased angiogenic gene expression, but only cavopulmonary anastomosis induced the expression of endothelial stress-related genes. Vascular endothelial growth factor was upregulated 2.5-fold after both cavopulmonary anastomosis (P =.002) and pulmonary artery banding (P =.007). Only cavopulmonary anastomosis upregulated 2 stress-related genes, HO1 and GLUT1, 2.7-fold (P =.002) and 3.8-fold (P =.03), respectively. Hypoxia-inducible factor was upregulated 4-fold (P =.003) after cavopulmonary anastomosis. Pulmonary artery banding failed to induce the increased expression of any of these proteins.Reduced pulmonary blood flow induces a pulmonary angiogenic response but not an endothelial stress response. These results suggest that oxidative stress is more relevant to the formation of pulmonary arteriovenous malformations than angiogenic signaling alone because pulmonary artery banding does not result in pulmonary arteriovenous malformations. Oxidative stress of the pulmonary endothelium resulting from cavopulmonary anastomosis may predispose the affected vasculature to arteriovenous shunting.

  View details for DOI 10.1067/mtc.2002.120346

  View details for Web of Science ID 000177840600009

  View details for PubMedID 12202863

• Value of clinical and echocardiographic features in predicting outcome in the fetus, infant, and child with tetralogy of Fallot with absent pulmonary valve complex AMERICAN JOURNAL OF CARDIOLOGY Moon-Grady, A. J., Tacy, T. A., Brook, M. M., Hanley, F. L., Silverman, N. H. 2002; 89 (11): 1280-1285

  Abstract

  We describe clinical and echocardiographic features of tetralogy of Fallot with absent pulmonary valve complex (TOF/APVC) and hypothesized that outcome might be related to pulmonary artery enlargement or severity of illness. We examined the clinical records of all 23 patients evaluated at our institution before death or surgical correction of TOF/APVC between 1990 and 2000. Echocardiograms for 16 patients (including 5 fetuses) were also reviewed, and measurements of the semilunar valves and pulmonary arteries were obtained and compared with patient's aortic annulus size and with established normal subjects. Actuarial survival was 15 of 23 patients (68%) at 4 years. Four fetuses were hydropic and none survived; 7 patients were ventilator dependent at operation and only 3 survived. No difference was noted in pulmonary artery diameters in survivors versus nonsurvivors. Pulmonary valve annulus size was larger in nonsurvivors (103 +/- 25% vs 71 +/- 24% of normal, p = 0.03); however, when fetal examinations were excluded, this difference did not persist. Thus, only hydrops and ventilator dependence at diagnosis predicted mortality. There was no correlation between postnatal measurements of pulmonary arteries and outcome. Larger pulmonary annulus size in hydropic fetuses and poor survival among patients diagnosed in utero suggests that the pathophysiology in TOF/APVC is not due entirely to the aneurysmal dilation of the pulmonary arteries but may be related to right-sided cardiac dysfunction.

  View details for Web of Science ID 000175985300008

  View details for PubMedID 12031728

• Surgical creation of aortopulmonary window in selected patients with pulmonary atresia with poorly developed aortopulmonary collaterals and hypoplastic pulmonary arteries 81st Annual Meeting of the American-Association-for-Thoracic-Surgery Rodefeld, M. D., Reddy, V. M., Thompson, L. D., Suleman, S., Moore, P. C., Teitel, D. F., Hanley, F. L. MOSBY-ELSEVIER. 2002: 1147–54

  Abstract

  The morphologic characteristics of the pulmonary circulation vary widely in patients with pulmonary atresia, ventricular septal defect, and major aortopulmonary collaterals. Although we favor single-stage unifocalization and complete repair as the procedure of choice, a subgroup of patients who meet specific criteria have been treated with initial surgical creation of an aortopulmonary window.Eighteen patients who were considered unsuitable candidates for single-stage repair underwent surgical creation of an aortopulmonary window. Selection criteria included the following: (1) presence of centrally confluent true pulmonary arteries 1.0 to 2.5 mm in diameter, with a well-developed peripheral arborization pattern; (2) multiple small aortopulmonary collateral vessels, most of which communicated with the true pulmonary arterial system; and (3) the presence of marked cyanosis.There were no early deaths, and the 2 late deaths were both unrelated to the procedure. Follow-up angiography (n = 17) demonstrated good growth of true pulmonary arteries in 15 of 17 cases (88%). Mean pulmonary artery diameter increased from 1.76 mm to 3.45 mm. Subsequent operations have been performed in 15 of 18 cases (83%). Intracardiac repair with or without additional unifocalization was achieved in 8 of these 15 (53%). Seven patients (47%) have undergone staged unifocalization as the next procedure; of these, 3 were able to have intracardiac repair. Thus 11 of the 15 patients who have undergone second operations (73%) have had complete intracardiac repair.The initial surgical creation of an aortopulmonary window in carefully selected patients can increase the size of the true pulmonary arteries, making these patients better candidates for eventual intracardiac repair. The procedure should be avoided in patients with pulmonary overcirculation, a predominance of isolated supply collaterals, or true pulmonary arteries larger than 2.5 mm in diameter, and it is not applicable without a true pulmonary artery central confluence.

  View details for DOI 10.1067/mtc.2002.121685

  View details for Web of Science ID 000176384000020

  View details for PubMedID 12063462

• Cavopulmonary anastomosis induces pulmonary expression of the angiotensin II receptor family 73rd Annual Scientific Sessions of the American-Heart-Association Malhotra, S. P., Reddy, V. M., Thelitz, S., He, Y. P., Hanley, F. L., Suleman, S., Riemer, R. K. MOSBY-ELSEVIER. 2002: 655–60

  Abstract

  Cavopulmonary anastomosis is used for palliation of cyanotic cardiac lesions. Postoperative development of pulmonary arteriovenous malformations can be significant in 10% to 25% of patients. To study the basis for formation of arteriovenous malformations, we developed an ovine model that reliably induces their development 8 weeks after cavopulmonary anastomosis. Previously, we found that cavopulmonary anastomosis inhibits the expression of pulmonary angiotensin-converting enzyme and suppresses angiotensin II production.This study examines the role of the angiotensin II receptors, type 1 and type 2, in this setting of pulmonary vascular remodeling.Lambs, aged 40 to 50 days, underwent cavopulmonary anastomosis. In age-matched control animals, a sham operation was performed. Messenger RNA and protein expression in lung specimens was measured at successive time points after cavopulmonary anastomosis or sham operations (n = 3 at each time point).Angiotensin type 1 mRNA was maximally upregulated 2-fold at 5 weeks after cavopulmonary anastomosis (P =.006). Expression of angiotensin type 1 protein was increased at least 2-fold at 2, 5, and 15 weeks after cavopulmonary anastomosis (P =.005). Cavopulmonary anastomosis also increased angiotensin type 2 mRNA and protein expression at least 2-fold at 2 and 5 weeks (P =.02) after surgical intervention. At 15 weeks, expression of angiotensin type 2 mRNA and protein was unchanged from that seen in control animals. Immunolocalization in pulmonary tissue sections 2 weeks after cavopulmonary anastomosis revealed markedly enhanced staining of angiotensin II receptor type 1 in vascular smooth muscle and angiotensin II receptor type 2 in the endothelium of pulmonary arteries.Rapid elevation in the expression of the type 1 and 2 angiotensin II receptors in the affected pulmonary vasculature after cavopulmonary anastomosis suggests their involvement in the pathologic vascular remodeling that occurs after cavopulmonary anastomosis.

  View details for DOI 10.1067/mtc.2002.119699

  View details for Web of Science ID 000175400100008

  View details for PubMedID 11986592

• Management of neonatal bronchovenous fistula after cardiopulmonary bypass ANNALS OF THORACIC SURGERY Reinhartz, O., DeSilva, A. M., Hanley, F. L. 2002; 73 (4): 1320-1322

  Abstract

  Bronchovenous fistula is occasionally encountered after traumatic lung injury or, in neonates, due to ventilation injuries with high ventilatory pressures. We report a case of massive air embolism associated with a bronchopulmonary venous communication in an infant post-repair of truncus arteriosus. Selective ventilation of the opposite lung for 3 days sealed the fistula.

  View details for Web of Science ID 000174807400080

  View details for PubMedID 11996288

• Neonatal truncus arteriosus repair: surgical techniques and clinical management. Seminars in thoracic and cardiovascular surgery. Pediatric cardiac surgery annual Rodefeld, M. D., Hanley, F. L. 2002; 5: 212-217

  Abstract

  Truncus arteriosus is now ideally repaired in the neonatal period with low morbidity and mortality. Published reports have documented mortality rates in the range of 4% to 5% with mean age at repair as low as 11 days. The physiologic basis for improved outcomes with earlier repair is the avoidance of damaging sequelae of pulmonary overcirculation and heart failure. Data show that baseline mean pulmonary artery pressure is lower in infants undergoing earlier repair. Improved operative outcomes also have been achieved with aggressive truncal valve repair versus replacement in the presence of truncal valve dysfunction, right ventricular outflow tract reconstructive techniques that are patient anatomy-specific, and use of regional perfusion techniques for repair of associated interrupted aortic arch. In addition, a heightened awareness of anomalies of coronary artery ostial location, number, angle of takeoff, and degree of patency can result in avoidance of inadvertent injury to the artery and associated myocardial insult.

  View details for PubMedID 11994881

• Arch reconstruction without circulatory arrest: current clinical applications and results of therapy. Seminars in thoracic and cardiovascular surgery. Pediatric cardiac surgery annual MacDonald, M. J., Hanley, F. L., Reddy, V. M. 2002; 5: 95-103

  Abstract

  Laboratory and clinical data have confirmed the deleterious effects of deep hypothermic circulatory arrest. Long-term data and rigorous neuropsychiatric data are currently sparse, but that which are available show adverse outcomes following circulatory arrest, and therefore support the use of continuous perfusion techniques. There are no available long-term data on continuous perfusion techniques with respect to neurologic outcomes, but using these techniques the incidence of postoperative seizures or other neurologic events is rare. Currently available bypass systems and microsurgical techniques have allowed continuous flow and regional perfusion to become practical alternatives. Several innovative techniques for avoiding circulatory arrest during neonatal aortic arch reconstruction for univentricular and biventricular hearts are described. It would appear prudent and desirable to provide continuous perfusion now that long-term survival after repair of even the most complex cardiac anomalies including single ventricle defects is commonplace.

  View details for PubMedID 11994869

• Robotic-assisted endoscopic thoracic aortic anastomosis in juvenile lambs. heart surgery forum Malhotra, S. P., Le, D., Thelitz, S., Hanley, F. L., Riemer, R. K., Suleman, S., Reddy, V. M. 2002; 6 (1): 38-42

  Abstract

  Advances in robotic technology have enabled a wider range of applications for minimally invasive techniques in cardiac surgery, including mitral valve repair and coronary artery bypass grafting. With increased technical sophistication, robotic-assisted techniques can be developed for the endoscopic repair of certain congenital cardiac lesions.The purpose of this study was to assess the feasibility of closed chest thoracic aortic anastomosis in a juvenile ovine model.Lambs, aged 45 to 55 days, underwent surgery that was performed using the da Vinci robotic surgical system. Using 3 ports, the surgeon dissected the descending thoracic aorta and mobilized it free from attachments, using single-lung ventilation and CO2 insufflation. Snares were introduced through 2 stab wounds for aortic occlusion proximally and distally. In 4 lambs, the aorta was completely transected and reanastomosed using interrupted nitinol sutures. One lamb underwent longitudinal aortotomy, and patch aortoplasty was performed with the placement of a Gore-Tex patch. Snares were released and the animals were recovered once hemodynamically stable. Animals were sacrificed at 6 to 12 hours after surgery and the descending aorta was harvested. Burst-pressure testing was performed on the anastomoses.All 5 lambs survived the procedure with stabilization of hemodynamic parameters following surgery. The mean aortic clamp time was 47 +/- 17 minutes, and the anastomosis was completed in 26 +/- 5 minutes. The mean burst pressure was 163 +/- 9 mm Hg.Endoscopic thoracic aortic anastomosis can be performed safely and with adequate exposure in a juvenile large-animal model using computer-assisted surgical techniques. With further refinements, these approaches could be applied to the repair of congenital anomalies of the aorta, including interrupted aortic arch and aortic coarctation.

  View details for PubMedID 12611730

• Pulmonary artery translocation: A surgical option for complex anomalous coronary artery anatomy ANNALS OF THORACIC SURGERY Rodefeld, M. D., Culbertson, C. B., Rosenfeld, H. M., Hanley, F. L., Thompson, L. D. 2001; 72 (6): 2150-2152

  Abstract

  Rather than perform a difficult and potentially high risk coronary reimplantation in a patient with an aberrant right coronary artery coursing between the aorta and pulmonary artery, the main pulmonary artery was translocated toward the left pulmonary hilum to create additional space between the aortic and pulmonic trunks.

  View details for Web of Science ID 000172584500084

  View details for PubMedID 11789826

• Hemidiaphragmatic paralysis increases postoperative morbidity after a modified Fontan operation 81st Annual Meeting of the American-Association-for-Thoracic-Surgery Amin, Z., McElhinney, D. B., Strawn, J. K., Kugler, J. D., Duncan, K. F., Reddy, V. M., Petrossian, E., Hanley, F. L. MOSBY-ELSEVIER. 2001: 856–62

  Abstract

  After a Fontan procedure, forward pulmonary blood flow is augmented during inspiration because of negative intrathoracic pressure. Total pulmonary blood flow is higher during inspiration. With hemidiaphragmatic paralysis, inspiratory augmentation of pulmonary flow is lost or diminished. The objective of this study was to compare early postoperative morbidity after the modified Fontan operation in patients with and without hemidiaphragmatic paralysis.A case-control analysis was performed comparing 10 patients with documented hemidiaphragmatic paralysis against 30 patients without paralysis who were matched for diagnosis, fenestration, and age. The following early postoperative outcomes were assessed: duration of ventilator support, duration of hospital stay, incidence of ascites, prolonged effusions, and readmission.Preoperatively, there were no significant differences between the 2 groups. However, among the postoperative outcomes, the duration of hospital stay (25.4 +/- 16.6 days vs 10.8 +/- 6.3 days; P =.03), incidence of ascites (70% vs 3%; P <.001), prolonged pleural effusions (60% vs 13%; P =.007), and readmission (50% vs 7%; P =.007) were significantly greater in patients with hemidiaphragmatic paralysis than in those without hemidiaphragmatic paralysis.Hemidiaphragmatic paralysis after the modified Fontan operation is associated with an increase in early morbidity. Care should be taken to avoid injury to the phrenic nerve. Patients with prolonged effusions should be evaluated for hemidiaphragmatic paralysis.

  View details for Web of Science ID 000172244900004

  View details for PubMedID 11689788

• Superior cavopulmonary anastomosis suppresses the activity and expression of pulmonary angiotensin-converting enzyme 80th Annual Meeting of the American-Association-for-Thoracic-Surgery Malhotra, S. P., Riemer, R. K., Thelitz, S., He, Y. P., Hanley, F. L., Reddy, V. M. MOSBY-ELSEVIER. 2001: 464–69

  Abstract

  Superior cavopulmonary anastomosis is widely used for palliation of various forms of univentricular heart defects. However, clinically significant pulmonary arteriovenous malformations develop in 15% to 25% of patients after surgery.To assess altered regulation of pulmonary vascular tone caused by superior cavopulmonary anastomosis in an ovine model.Lambs, aged 35 to 45 days, underwent an end-to-end anastomosis of the superior vena cava to the right pulmonary artery. In age-matched controls, a sham operation was performed. Arteriovenous malformations were detectable by contrast echocardiography by 8 weeks after surgery. Animals (n = 24) were studied at various time points after the operations. Expression of angiotensin-converting enzyme messenger RNA, protein levels, and enzyme activity were measured in lung homogenates. Levels of angiotensin II were measured by enzyme-linked immunosorbent assay.Expression of angiotensin-converting enzyme messenger RNA and protein was significantly reduced at 1 to 5 weeks after superior cavopulmonary anastomosis. Angiotensin-converting enzyme activity in the right lung of animals subjected to superior cavopulmonary anastomosis was reduced 86% +/- 1% (standard deviation) compared with control values at 1 week (P =.003) and 77% +/- 8.5% at 2 weeks (P <.001) after surgery. This correlated with a 59% +/- 3.5% (P =.007) reduction in angiotensin II levels up to 5 weeks after cavopulmonary anastomosis. By 15 weeks after the operations, angiotensin II levels were equivalent to control levels (P =.19).Superior cavopulmonary anastomosis causes an early reversible reduction in activity and expression of angiotensin-converting enzyme, resulting in decreased circulating levels of the vasoconstrictor angiotensin II. These results suggest that the ability of the pulmonary endothelium to regulate vascular tone is inhibited after superior cavopulmonary anastomosis. Dilation of the affected vasculature induced by cavopulmonary anastomosis may contribute to the disordered vascular remodeling observed in this setting.

  View details for DOI 10.1067/mtc.2001.115698

  View details for Web of Science ID 000171027100010

  View details for PubMedID 11547295

• Neonatal repair of truncus arteriosus: Continuing improvement in outcomes ANNALS OF THORACIC SURGERY Thompson, L. D., McElhinney, D. B., Reddy, V. M., Petrossian, E., Silverman, N. H., Hanley, F. L. 2001; 72 (2): 391-395

  Abstract

  Repair of truncus arteriosus in the neonatal and early infant periods has become standard practice at many centers. We reviewed our recent experience with repair of truncus arteriosus in neonates, with a focus on early and intermediate outcomes.From July 1992 to December 1999, 65 patients 1 month of age or less underwent primary complete repair of truncus arteriosus. Median age was 10 days, and median weight was 3.2 kg. Major associated anomalies included moderate or severe truncal valve regurgitation in 15 patients (23%), interrupted aortic arch in 8 (12%), coronary artery abnormalities in 12 (18%), and nonconfluence of the pulmonary arteries in 3 (5%). Median durations of cardiopulmonary bypass and cardioplegic arrest were 172 minutes and 90 minutes, respectively. Circulatory arrest was employed only in 7 patients undergoing concomitant repair of interrupted arch. Reconstruction of the right ventricular outflow tract was achieved with an aortic (n = 39) or pulmonary (n = 26) allograft valved conduit (median diameter, 12 mm). Replacement (n = 6) or repair (n = 5) of a regurgitant truncal valve was performed in 11 patients, and interrupted arch was repaired in 8.There were three early deaths (5%). Early reoperations included reexploration for bleeding in 3 patients, emergent replacement of a pulmonary outflow conduit that failed acutely in 1 patient, and placement of a permanent pacemaker in 1. Mechanical circulatory support was required in 1 patient. During the median follow-up of 32 months, there were two deaths. The Kaplan-Meier estimate of survival was 92% at 1 year and beyond. The only demographic, diagnostic, or operative factors significantly associated with poorer survival over time were operative weight of 2.5 kg or less (p = 0.01) and truncal valve replacement (p = 0.009). Actuarial freedom from conduit replacement among early survivors was 57% at 3 years.Repair of truncus arteriosus in the neonatal period can be performed routinely with excellent survival, even in patients with major associated abnormalities.

  View details for Web of Science ID 000170437300018

  View details for PubMedID 11515872

• A prospective randomized study comparing volume-standardized modified and conventional ultrafiltration in pediatric cardiac surgery 80th Annual Meeting of the American-Association-for-Thoracic-Surgery Thompson, L. D., McElhinney, D. B., Findlay, P., Miller-Hance, W., Chen, M. J., Minami, M., Petrossian, E., Parry, A. J., Reddy, V. M., Hanley, F. L. MOSBY-ELSEVIER. 2001: 220–28

  Abstract

  Modified ultrafiltration has been touted as superior to conventional ultrafiltration for attenuating the consequences of hemodilution after cardiac surgery with cardiopulmonary bypass in children. We conducted a prospective randomized study to test the hypothesis that modified and conventional ultrafiltration have similar clinical effects when a standardized volume of fluid is removed.From October 1998 to September 1999, 110 children weighing 15 kg or less (median weight 6.1 kg, median age 6.3 months) undergoing surgery with cardiopulmonary bypass for functionally biventricular congenital heart disease were randomized to conventional (n = 67) or arteriovenous modified ultrafiltration (n = 43) for hemoconcentration. The volume of fluid removed with both methods was standardized as a percentage of effective fluid balance (the sum of prime volume and volume added during cardiopulmonary bypass minus urine output): in patients weighing less than 10 kg, 50% of effective fluid balance was removed, whereas 60% was removed in patients weighing 10 to 15 kg. Hematocrit, hemodynamics, ventricular function, transfusion of blood products, and postoperative resource use were compared between groups.There were no significant differences between groups in age, weight, or duration of cardiopulmonary bypass. The total volume of fluid added in the prime and during bypass was greater in patients undergoing conventional ultrafiltration than in those receiving modified ultrafiltration (205 +/- 123 vs 162 +/- 74 mL/kg; P =.05), although the difference was due primarily to a greater indexed priming volume in patients having conventional ultrafiltration. There was no difference in the percentage of effective fluid balance that was removed in the 2 groups. Accordingly, the volume of ultrafiltrate was greater in patients receiving conventional than modified ultrafiltration (95 +/- 63 vs 68 +/- 28 mL/kg; P =.01). Preoperative and postoperative hematocrit levels were 35.6% +/- 6.6% and 36.3% +/- 5.6% in patients having conventional ultrafiltration and 34.4% +/- 6.7% and 38.7% +/- 7.5% in those having modified ultrafiltration. By repeated-measures analysis of variance, patients receiving modified and conventional ultrafiltration did not differ with respect to hematocrit value (P =.87), mean arterial pressure (P =.85), heart rate (P =.43), or left ventricular shortening fraction (P =.21) from baseline to the postbypass measurements. There were no differences between groups in duration of mechanical ventilation, stay in the intensive care unit, or hospitalization.When a standardized volume of fluid is removed, hematocrit, hemodynamics, ventricular function, requirement for blood products, and postoperative resource use do not differ between pediatric patients receiving conventional and modified ultrafiltration for hemoconcentration after cardiac surgery.

  View details for Web of Science ID 000170254000005

  View details for PubMedID 11479493

• The safety and efficacy of sevoflurane anesthesia in infants and children with congenital heart disease ANESTHESIA AND ANALGESIA Russell, I. A., Hance, W. C., Gregory, G., Balea, M. C., Cassorla, L., DeSilva, A., Hickey, R. F., Reynolds, L. M., Rouine-Rapp, K., Hanley, F. L., Reddy, V. M., Cahalan, M. K. 2001; 92 (5): 1152-1158

  Abstract

  We tested the hypothesis that sevoflurane is a safer and more effective anesthetic than halothane during the induction and maintenance of anesthesia for infants and children with congenital heart disease undergoing cardiac surgery. With a background of fentanyl (5 microg/kg bolus, then 5 microg. kg(-1). h(-1)), the two inhaled anesthetics were directly compared in a randomized, double-blinded, open-label study involving 180 infants and children. Primary outcome variables included severe hypotension, bradycardia, and oxygen desaturation, defined as a 30% decrease in the resting mean arterial blood pressure or heart rate, or a 20% decrease in the resting arterial oxygen saturation, for at least 30 s. There were no differences in the incidence of these variables; however, patients receiving halothane experienced twice as many episodes of severe hypotension as those who received sevoflurane (P = 0.03). These recurrences of hypotension occurred despite an increased incidence of vasopressor use in the halothane-treated patients than in the sevoflurane-treated patients. Multivariate stepwise logistic regression demonstrated that patients less than 1 yr old were at increased risk for hypotension compared with older children (P = 0.0004), and patients with preoperative cyanosis were at increased risk for developing severe desaturation (P = 0.049). Sevoflurane may have hemodynamic advantages over halothane in infants and children with congenital heart disease.In infants and children with congenital heart disease, anesthesia with sevoflurane may result in fewer episodes of severe hypotension and less emergent drug use than anesthesia with halothane.

  View details for Web of Science ID 000168335900013

  View details for PubMedID 11323338

• Placental compliance during fetal extracorporeal circulation JOURNAL OF APPLIED PHYSIOLOGY Assad, R. S., Lee, F. Y., Hanley, F. L. 2001; 90 (5): 1882-1886

  Abstract

  The fetus requires large amounts of volume when weaning from cardiac bypass. This suggests that placental vasculature can act as a large capacitor in the fetal circulation. To assess placental compliance of fetal lambs, seven isolated in situ lamb placentas were placed on extracorporeal circulation. Umbilical artery blood flow was varied from 0 to 350 ml. min(-1). kg fetal wt(-1). Because the extracorporeal circuit is a closed system, volume changes in the placenta induced by umbilical artery pressure changes were measured from reciprocal volume changes in the reservoir. There was a wide range of change in absolute volume of blood within the fetal placental compartment (216.4 +/- 29.3 ml). Placental compliance was linear over the entire range of pressure changes exerted on the placental vasculature (r(2) = 0.83, P = 0.0001). This indicates that the placenta is a unique and sensitive capacitor in the fetal circulation. This information is important clinically because it establishes that aggressive resuscitation of the fetus using volume may be necessary when weaning the fetus from cardiac bypass.

  View details for Web of Science ID 000168227600034

  View details for PubMedID 11299282

• Improved surgical outcome after fetal diagnosis of hypoplastic left heart syndrome 47th Annual Scientific Session of the American-College-of-Cardiology Tworetzky, W., McElhinney, D. B., Reddy, V. M., Brook, M. M., Hanley, F. L., Silverman, N. H. LIPPINCOTT WILLIAMS & WILKINS. 2001: 1269–73

  Abstract

  Hypoplastic left heart syndrome (HLHS) is frequently diagnosed prenatally, but this has not been shown to improve surgical outcome.We reviewed patients with HLHS between July 1992 and March 1999 to determine the influence of prenatal diagnosis on preoperative clinical status, outcomes of stage 1 surgery, and parental decisions regarding care. Of 88 patients, 33 were diagnosed prenatally and 55 after birth. Of 33 prenatally diagnosed patients, 22 were live-born, and pregnancy was terminated in 11. Of 22 prenatally diagnosed patients who were live-born, 14 underwent surgery, and parents elected to forego treatment in 8. Of 55 patients diagnosed postnatally, 38 underwent surgery, and 17 did not because of parental decisions or clinical considerations. Prenatally diagnosed patients were less likely to undergo surgery than patients diagnosed after birth (P:=0.008). Among live-born infants, there was a similar rate of nonintervention. Among patients who underwent surgery, survival was 75% (39/52). All patients who had a prenatal diagnosis and underwent surgery survived, whereas only 25 of 38 postnatally diagnosed patients survived (P:=0.009). Patients diagnosed prenatally had a lower incidence of preoperative acidosis (P:=0.02), tricuspid regurgitation (P:=0.001), and ventricular dysfunction (P:=0.004). They were also less likely to need preoperative inotropic medications or bicarbonate (P:=0.005). Preoperative factors correlating with early mortality included postnatal diagnosis (P:=0.009), more severe acidosis (P:=0.03), need for bicarbonate or inotropes (P:=0.008 and 0.04), and ventricular dysfunction (P:=0.05).Prenatal diagnosis of HLHS was associated with improved preoperative clinical status and with improved survival after first-stage palliation in comparison with patients diagnosed after birth.

  View details for Web of Science ID 000167562200017

  View details for PubMedID 11238272

• Late thrombosis of the native aortic root after Norwood reconstruction for hypoplastic left heart syndrome JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY Brennan, T. V., Rodefeld, M. D., Tacy, T. A., Reddy, V. M., Hanley, F. L. 2001; 121 (3): 580-582

  View details for Web of Science ID 000167721100024

  View details for PubMedID 11241094

• Perioperative administration of angiotensin converting enzyme inhibitors decreases the severity and duration of pleural effusions following bidirectional cavopulmonary anastomosis CARDIOLOGY IN THE YOUNG Thompson, L. D., McElhinney, D. B., Culbertson, C. B., Hardy, C. E., Brook, M. M., Reddy, V. M., Hanley, F. L. 2001; 11 (2): 195-200

  Abstract

  Pleural effusions after bidirectional cavopulmonary anastomosis remain a significant cause of morbidity. Prolonged effusions in such patients have been associated with persistent elevations in plasma renin and angiotensin II.We conducted a controlled study in 36 patients (median age 8 months) undergoing bidirectional cavopulmonary anastomosis. Enalapril (5 mcg/kg) was administered intravenously within 1 hour of surgery and every 12 hours thereafter in 18 patients; when these patients were tolerating feeds, enalapril was switched to enteral captopril (3 mg/kg/day) every 8 hours. The other 18 patients did not receive perioperative angiotensin converting enzyme inhibitors. Using standardized criteria for discontinuation of chest tubes (< 2 mL/kg/day), volume and duration of pleural drainage were compared between groups. RESULTS. There were no differences between groups in demographic, diagnostic, or hemodynamic factors. There was no difference in cardiopulmonary bypass time between groups and no difference in postoperative pulmonary arterial pressures. The duration of pleural drainage was shorter (2.2+/-1.4 vs 5.9+/-1.4 days, p<0.001) and the volume less during the first 24 hours (4.7+/-1.2 vs 7.7+/-2.1 mL/kg, p<0.001) and overall (10.6+/-2.4 vs 19.6+/-4.5 mL/kg, p<0.001) in patients who received angiotensin converting enzyme inhibitors than those who did not. Readmission for persistent effusions was required in 3 patients who did not receive angiotensin converting enzyme inhibitors and none who did (p=0.11).Perioperative administration of angiotensin converting enzyme inhibitors is associated with decreased severity and duration of pleural effusions following bidirectional cavopulmonary anastomosis.

  View details for Web of Science ID 000167755900009

  View details for PubMedID 11293738

• Elective primary repair of acyanotic tetralogy of fallot in early infancy: Overall outcome and impact on the pulmonary valve JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY Parry, A. J., McElhinney, D. B., Kung, G. C., Reddy, V. M., Brook, M. M., Hanley, F. L. 2000; 36 (7): 2279-2283

  Abstract

  We sought to determine if early primary repair of acyanotic tetralogy of Fallot (ToF) can be performed safely with low requirement for transannular patching (TAP) and thereafter allow normal right ventricular outflow tract (RVOT) growth.Early primary repair of ToF normalizes intracardiac flow patterns, which may allow subsequent normal RVOT growth. Traditionally repair is deferred until symptoms occur or children are deemed of adequate size for operative risk to be acceptable because of a perceived increased requirement for TAP in small infants.Between July 1992 and March 1999, 42 acyanotic infants aged 4 to 87 days (median 62) and weight 2.6 to 6.6 kg (median 4.55) underwent complete repair of ToF. Pulmonary annulus measured 4 to 10.5 mm (median 6.5) with "z-value" of-5.6 to +3.0 (median -1.9). RVOT reconstruction was tailored to each patient; pulmonary valvotomy was performed in 26, main pulmonary arterioplasty in 22, and infundibular patching in 2. Only 10 (24%) required TAP.Postoperative RVOT gradient was 0 to 30 mm Hg (median 10) and pRV/pLV ratio 0.3 to 0.6 (median 0.44). Pulmonary insufficiency was trivial/mild. There were no deaths. Junctional ectopic tachycardia developed in seven; only one required treatment. ICU stay was 2 to 14 days (median 4) and hospital stay 4 to 22 days (median 7). At follow-up 12 to 64 months later (median 38) there were no deaths. One child required reoperation for recurrent RVOT obstruction and two required balloon pulmonary arterioplasty. Follow-up RVOT gradient was 0 to 36 mm Hg (median 12), unchanged from early postoperative condition, and median z-value was -1.2 (-2.8 to +2.5); pulmonary insufficiency remained trivial/mild.Complete repair of acyanotic ToF can be performed in early infancy with low morbidity and mortality and low requirement for TAP. Though results are not statistically significant, early repair may allow normal RVOT growth thereafter.

  View details for Web of Science ID 000165600400039

  View details for PubMedID 11127473

• Coronary arterial size late after the atrial inversion procedure for transposition of the great arteries: Implications for the arterial switch operation 80th Annual Meeting of the American-Association-for-Thoracic-Surgery Amin, Z., McElhinney, D. B., Moore, P., Reddy, V. M., Hanley, F. L. MOSBY-ELSEVIER. 2000: 1047–52

  Abstract

  Coronary flow reserve in the hypertrophied ventricle is reduced. One contributing factor may be the size of the proximal coronary arteries. In patients who undergo atrial inversion procedures for transposition of the great arteries, the left coronary artery supplies the pulmonary ventricle and may be smaller than the right coronary artery. We hypothesized that the dimensions of the coronary arteries may correlate with symptomatic status after atrial inversion and may be an important factor when these patients are considered for the arterial switch operation.The proximal left and right coronary arteries were measured in 9 patients with transposition and failure of the systemic right ventricle after atrial inversion, 10 asymptomatic patients after atrial inversion, and 10 patients with normal hearts. The diameters of the coronary arteries were indexed to body surface area and compared.The absolute and indexed diameters of the right coronary artery were greater in symptomatic patients than in asymptomatic patients (indexed: 3.1 +/- 0.6 vs 2.4 +/- 0.4 mm/m(2), P <.001) or control patients (2.0 +/- 0.3, P <.001), and the absolute diameter of the left coronary artery was smaller (2.9 +/- 0. 7 vs 3.6 +/- 0.5 mm, P =.003 [asymptomatic], 3.6 +/- 0.5 mm, P =.01 [control]). In symptomatic patients, the absolute and indexed diameters of the left coronary artery were smaller than those of the right (indexed: 2.1 +/- 0.6 vs 3.1 +/- 0.6 mm/m(2), P <.001). By contrast, there was no difference in asymptomatic patients (2.2 +/- 0.5 vs 2.4 +/- 0.4 mm/m(2), P =.44), and the left coronary artery was larger in normal control patients (2.2 +/- 0.4 vs 2.0 +/- 0.3 mm/m(2), P <.001).Differences in the sizes of the proximal coronary arteries may be related to symptomatic status in patients with transposition of the great arteries who have undergone an atrial inversion procedure, as well as to the efficacy of ventricular retraining. When pulmonary artery banding and subsequent arterial switch are considered for patients with a Mustard or Senning procedure and a failing systemic right ventricle, the size of the proximal coronary arteries may be an important factor and should be evaluated with preoperative imaging studies.

  View details for Web of Science ID 000165748800006

  View details for PubMedID 11088025

• Familial absent pulmonary valve syndrome without deletions of chromosome 22q11 CARDIOLOGY IN THE YOUNG McElhinney, D. B., Hanley, F. L., Stanger, P. 2000; 10 (6): 618-620

  Abstract

  Deletions of chromosome 22q11 are common in patients with tetralogy of Fallot, and in those with absent pulmonary valve syndrome. In this report, we describe a pair of siblings with absent pulmonary valve syndrome, neither of whom had deletions of chromosome 22q11. The finding of familial absent pulmonary valve syndrome without deletion of 22q11 in our patients suggests an alternative genetic basis for this rare condition.

  View details for Web of Science ID 000165218100013

  View details for PubMedID 11117395

• Infradiaphragmatic totally anomalous pulmonary venous return with two separate descending veins in association with right atrial isomerism ANNALS OF THORACIC SURGERY Thompson, L. D., McElhinney, D. B., Reddy, V. M., Jue, K. L., Hanley, F. L. 2000; 70 (4): 1400-1402

  Abstract

  We present the case of a 1-month-old infant with right atrial isomerism and complex functionally univentricular heart disease, in whom totally anomalous pulmonary venous return drained below the diaphragm via two separate vertical veins. One of the descending vertical veins drained the entire right lung and the left upper lobe, while the other drained the remainder of the left lung. Only one similar case has been described previously.

  View details for Web of Science ID 000089925600064

  View details for PubMedID 11081910

• Neutrophil degranulation and complement activation during fetal cardiac bypass ANNALS OF THORACIC SURGERY Parry, A. J., Petrossian, E., McElhinney, D. B., Reddy, V. M., Hanley, F. L. 2000; 70 (2): 582-589

  Abstract

  Fetal cardiac bypass results in dysfunction of the fetoplacental unit (FPU) characterized by increased placental vascular resistance and respiratory acidosis. However the mechanisms of this dysfunction are not completely understood. To test the hypothesis that complement activation and neutrophil degranulation may contribute to the placental dysfunction associated with fetal bypass, we compared placental hemodynamics, complement activation, and neutrophil degranulation among fetuses exposed to cardiac bypass with a miniaturized bypass circuit including an in-line axial flow pump (Hemopump), fetuses undergoing bypass with a conventional roller pump circuit, and control fetuses that were similarly exposed but did not undergo bypass.Twenty-six Western Cross sheep fetuses (median 122 days gestation) were randomly assigned to undergo cardiac bypass for 30 minutes with the Hemopump circuit (n = 8), to undergo bypass for 30 minutes with the conventional (roller pump) circuit (n = 10), or to undergo identical exposure and cannulation but not bypass (n = 8, controls). Blood samples were collected to measure white cell count and differential, and C3a and lactoferrin levels prior to bypass, at the end of bypass, and 1 and 2 hours after bypass. Hemodynamics and blood gases were also monitored.There was a fall in white cell count over time that continued after bypass in all groups; neutrophils and lymphocytes were affected similarly. C3a levels rose significantly from prebypass to postbypass in the roller pump group (p<0.0001) but not in either of the other groups. Lactoferrin levels rose significantly from start of bypass in both bypass groups (Hemopump p = 0.01; roller pump p<0.0001) but not in controls. The elevation in lactoferrin level coincided with worsening placental gas exchange and deteriorating cardiac function.Complement and neutrophil activation occurred with fetal cardiac bypass but only neutrophil activation mirrored the FPU and cardiac dysfunction, suggesting that products of neutrophil activation may be important contributing factors. Improved FPU function with a bypass circuit that has less extracorporeal surface and does not require a large priming volume may be due in part to a reduction in the magnitude of this inflammatory response.

  View details for Web of Science ID 000088886300051

  View details for PubMedID 10969684

• Transthoracic intracardiac catheters in pediatric patients recovering from congenital heart defect surgery: Associated complications and outcomes CRITICAL CARE MEDICINE Flori, H. R., Johnson, L. D., Hanley, F. L., Fineman, J. R. 2000; 28 (8): 2997-3001

  Abstract

  To characterize transthoracic intracardiac catheter uses and associated morbidities in pediatric patients recovering from congenital heart defect surgery and to identify potential risk factors associated with their use.Prospective data collection and review.An 18-bed pediatric intensive care unit (PICU) in a tertiary care university hospital.All pediatric patients between October 1, 1996, and September 30, 1997, who were recovering from congenital heart defect surgery and had transthoracic intracardiac catheters in place.Catheter use, associated morbidity, necessary interventions, and risk factors for complications of catheter use were identified. During this period, 523 catheters (276 right atrial, 155 left atrial, 68 common atrial, and 24 right ventricular or pulmonary artery catheters) in 351 PICU patients were studied. Mean age was 23.1+/-45.1 months (median, 4.98 months); 138 patients (39.3%) were <3 months old. The rate of occurrence of bleeding with catheter removal (mediastinal output in the hour after removal that was more than twice the previous average hourly output) was 36.7%, and bleeding occurred more frequently with left atrial catheters (47%; odds ratio, 2.0; p < .05). However, interventions after catheter removal were required for only 8.3% (42/504) of catheters removed, and hemodynamic compromise occurred with the removal of only 2.6% (13/504) of catheters. Interventions included fluid resuscitation (35 cases), pleural drainage (three cases), catheter wiring for retention (one case), chest tube suctioning (two cases), and surgical removal (one case). No associated deaths occurred. In a multivariate logistic regression analysis, age <3 months (odds ratio, 4.74), catheter location (left atrial: odds ratio, 4.97; pulmonary artery: odds ratio, 12.48), and platelet count of <50,000 (odds ratio, 8.59) were identified as risk factors associated with a need for intervention after catheter removal (p < .05). Other complications included blood cultures positive for organisms (1.5%), thrombus (0.6%), and catheter nonfunction (10.9%). Prematurity was a risk factor for thrombus and nonfunction.Use of transthoracic intracardiac catheters in pediatric patients is safe. Young infants and pediatric patients with thrombocytopenia or with catheters in the left atrial or pulmonary artery position have a greater need for interventions after catheter removal, warranting added precautions.

  View details for Web of Science ID 000088867300050

  View details for PubMedID 10966285

• Coronary to pulmonary artery collaterals in patients with pulmonary atresia and ventricular septal defect ANNALS OF THORACIC SURGERY Amin, Z., McElhinney, D. B., Reddy, V. M., Moore, P., Hanley, F. L., Teitel, D. F. 2000; 70 (1): 119-123

  Abstract

  The frequency, distribution, and surgical importance of coronary artery to pulmonary artery (CAPA) collaterals have not been established. The aim of this study was to establish prevalence, anatomical pattern, and significance of CAPA in patients with pulmonary atresia and ventricular septal defect (PA/VSD).We reviewed cardiac catheterization and operative data of 87 consecutive, unselected patients who underwent one-stage complete unifocalization for PA/VSD and assessed major systemic to pulmonary collaterals from July 1992 to June 1998.CAPA collaterals were diagnosed in 9 of 87 patients (10%). The collaterals originated from the left coronary artery system in 7 patients and the right in 2. Collaterals from the left coronary system arose from the left main coronary artery in 3 patients and the circumflex in 4. All collaterals joined the central pulmonary artery, which bifurcated and supplied both lungs. One collateral from the right coronary system joined the stump of the main pulmonary artery and the other gave origin to a true left pulmonary artery, which was the sole supply to 75% of the left lung. Coronary artery enlargement was seen in 2 patients only. No patient had evidence of myocardial ischemia. Coronary collaterals comprised a dual source of pulmonary blood flow in all but 1 patient. During unifocalization, the CAPA collaterals were ligated at its origin in all cases, and the collateral from the right coronary to the left pulmonary artery was unifocalized.The prevalence of CAPA collaterals in patients with PA/VSD is approximately 10%. The diagnosis may be missed without appropriate angiograms. We recommend selective ascending aortogram or selective coronary angiogram in all patients.

  View details for Web of Science ID 000088318100029

  View details for PubMedID 10921694

• Surgical approach to complicated cervical aortic arch: anatomic, developmental, and surgical considerations CARDIOLOGY IN THE YOUNG McElhinney, D. B., Thompson, L. D., Weinberg, P. M., Jue, K. L., Hanley, F. L. 2000; 10 (3): 212-219

  Abstract

  Abnormalities of brachiocephalic arterial branching and arch laterality are common in patients with a cervical aortic arch. In addition, structural anomalies of the arch such as obstruction, aneurysms, and tortuosity are found in a significant number of cases.Between 1990 and 1998, 6 patients underwent surgery for an obstructed right cervical arch. A significant obstruction was present at the transverse or distal arch in all patients, and was recurrent after previous repair in 2. In 1 patient, there was also a multi-lobed aneurysm of the aortic segment contiguous to the obstruction, and in 2 there was marked tortuosity of the arch. In all cases, the order of origin of the head and neck vessels was abnormal, and obstruction of 1 or more brachiocephalic vessels was found in 3. A vascular ring was present in all patients, with a right aortic arch and aberrant left subclavian artery in 4 patients and a double aortic arch with a dominant right cervical arch in 2. The descending aorta was circumflex (left-sided) in 3 patients. Three patients were repaired through a standard right posterolateral thoracotomy, and 3 through a median sternotomy. Patch augmentation aortoplasty was used in 2 patients, a tube graft from the ascending to descending aorta in 2, end to side anastomosis of the descending aorta to the proximal arch in 1, and direct anastomosis to reconstruct an atretic left-sided component of a double arch in 1.Repair was successful in all cases, with no perioperative complications. At follow-up ranging from 1 to 9 years, all patients were alive and well, with no recurrence of arch obstruction or other significant complications. Fluorescent in situ hybridization revealed microdeletion of chromosome 22q11 in 1 patient (not performed in the others).Structural anomalies of the arch are relatively common in patients with a cervical aortic arch. Such abnormalities may be the result of hemodynamic conditions and/or abnormal vascular tissue related either to the cervical position of the arch or its embryologic precursors. Given the highly variable anatomy of patients with a complicated cervical aortic arch, surgical considerations will vary in kind.

  View details for Web of Science ID 000086998800007

  View details for PubMedID 10824901

• Early and intermediate outcomes after repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries - Experience with 85 patients 70th Scientific Session of the American-Heart-Association Reddy, V. M., McElhinney, D. B., Amin, Z., Moore, P., Parry, A. J., Teitel, D. F., Hanley, F. L. LIPPINCOTT WILLIAMS & WILKINS. 2000: 1826–32

  Abstract

  Pulmonary atresia with ventricular septal defect (VSD) and major aortopulmonary collaterals (MAPCAs) is a complex lesion with marked heterogeneity of pulmonary blood supply. Traditional management has involved staged unifocalization of pulmonary blood supply. Our approach has been to perform early 1-stage complete unifocalization in almost all patients.Since 1992, 85 patients with pulmonary atresia, VSD, and MAPCAs have undergone unifocalization (median age, 7 months). Complete 1-stage unifocalization and intracardiac repair were performed through a midline approach in 56 patients, whereas 23 underwent unifocalization in a single stage with the VSD left open, and 6 underwent staged unifocalization through sequential thoracotomies. There were 9 early deaths. During follow-up (1 to 69 months), there were 7 late deaths. Actuarial survival was 80% at 3 years. Among early survivors, actuarial survival with complete repair was 88% at 2 years. Reintervention on the neo-pulmonary arteries was performed in 24 patients.Early 1-stage complete unifocalization can be performed in >90% of patients with pulmonary atresia and MAPCAs, even those with absent true pulmonary arteries, and yields good functional results. Complete repair during the same operation is achieved in two thirds of patients. There remains room for improvement; actuarial survival 3 years after surgery is 80%, and there is a significant rate of reintervention. These results must be appreciated within the context of the natural history of this lesion: 65% of patients survive to 1 year of age and slightly >50% survive to 2 years even with surgical intervention.

  View details for Web of Science ID 000086812900013

  View details for PubMedID 10769284

• Reinterventions after repair of common arterial trunk in neonates and young infants JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY McElhinney, D. B., Rajasinghe, H. A., Mora, B. N., Reddy, V. M., Silverman, N. H., Hanley, F. L. 2000; 35 (5): 1317-1322

  Abstract

  To determine rates of reintervention after repair of common arterial trunk in the neonatal and early infant periods.With improving success in the early treatment of common arterial trunk, the need for reinterventional procedures in older children, adolescents and adults will become an increasingly widespread concern in the treatment of these patients.We reviewed our experience with 159 infants younger than four months of age who underwent complete primary repair of common arterial trunk at our institution from 1975 to 1998, with a focus on postoperative reinterventions.Of 128 early survivors, 40 underwent early reinterventions for persistent mediastinal bleeding or other reasons. During a median follow-up of 98 months (range, 2 to 235 months), 121 reinterventions were performed in 81 patients. Actuarial freedom from reintervention was 50% at four years, and freedom from a second reintervention was 75% at 11 years. A total of 92 conduit reinterventions were performed in 75 patients, with a single reintervention in 61 patients, 2 reinterventions in 11 patients and 3 reinterventions in 3 patients. Freedom from a first conduit reintervention was 45% at five years. The only independent variable predictive of a longer time to first conduit replacement was use of an allograft conduit at the original repair (p = 0.05), despite the significantly younger age of patients receiving an allograft conduit (p < 0.001). Reintervention on the truncal valve was performed on 22 occasions in 19 patients, including 21 valve replacements in 18 patients and repair in 1, with a freedom from truncal valve reintervention of 83% at 10 years. Surgical (n = 29) or balloon (n = 12) reintervention for pulmonary artery stenosis was performed 41 times in 32 patients. Closure of a residual ventricular septal defect was required in 13 patients, all of whom underwent closure originally with a continuous suture technique. Eight of 16 late deaths were related to reintervention.The burden of reintervention after repair of common arterial trunk in early infancy is high. Although conduit reintervention is inevitable, efforts should be made at the time of the initial repair to minimize factors leading to reintervention, including prevention of branch pulmonary artery stenosis and residual interventricular communications.

  View details for Web of Science ID 000086265900028

  View details for PubMedID 10758975

• Management and outcomes of delayed sternal closure after cardiac surgery in neonates and infants CRITICAL CARE MEDICINE McElhinney, D. B., Reddy, V. M., Parry, A. J., Johnson, L., Fineman, J. R., Hanley, F. L. 2000; 28 (4): 1180-1184

  Abstract

  To investigate the efficacy, safety, and patterns of management of open sternotomy and delayed sternal closure in infants who were left with an open sternum after cardiac surgery and to assess these patterns for possible correlation with outcome.Retrospective chart review with statistical analysis.Pediatric cardiac surgery service at a regional referral center based in an urban university teaching hospital.All 128 patients <1 yr of age who were left with an open sternum after cardiac surgery with cardiopulmonary bypass during the 4-yr period from July, 1992 to June, 1996.Procedures for managing open sternotomy and delayed sternal closure were analyzed retrospectively. No interventions were undertaken for the study.Of the 128 patients, 14 (11%) died before sternal closure; delayed sternal closure was performed in the remaining 114. Of these 114, 13 died in the early postoperative period. During sternal closure, significant increases were noted in pulmonary arterial (from 21.1+/-7.6 mm Hg to 26.1+/-6.5 mm Hg; p = .006), left atrial (from 8.4+/-3.4 mm Hg to 11.5+/-3.7 mm Hg; p < .001), and right atrial pressures (from 7.3+/-2.5 mm Hg to 9.8+/-2.5 mm Hg; p < .001). In addition, mean airway pressure (from 7.4+/-2.0 mm Hg to 8.6+/-2.4 mm Hg; p < .001) and peak inspiratory pressure (from 29.3+/-5.4 mm Hg to 31.3+/-5.6 mm Hg; p = .004) increased. Sternal wound infection occurred in one patient.Delayed sternal closure is an effective approach to the management of neonates and infants at risk for hemodynamic, respiratory, or hemostatic instability early after cardiac surgery. Significant changes in hemodynamics and respiratory variables occur during sternal closure, often requiring adjustment of inotropic and ventilatory management. (Crit Care Med 2000; 28: 1180-1184)

  View details for Web of Science ID 000086862800044

  View details for PubMedID 10809302

• Incidence and implications of systemic to pulmonary collaterals after bidirectional cavopulmonary anastomosis ANNALS OF THORACIC SURGERY McElhinney, D. B., Reddy, V. M., Tworetzky, W., Petrossian, E., Hanley, F. L., Moore, P. 2000; 69 (4): 1222-1228

  Abstract

  Systemic to pulmonary arterial collaterals often develop after bidirectional cavopulmonary anastomosis (BCPA). It has been proposed that such collaterals may be related to perioperative outcome and duration of effusions after the modified Fontan procedure. However, the incidence and significance of collaterals after BCPA remain uncertain.To evaluate risk factors for, and significance of, such collaterals, we reviewed angiographic and clinical data for all 76 patients who underwent BCPA between January 1990 and June 1996 and had follow-up catheterization during or before 1997.The median age at BCPA was 10 months, and the median duration from BCPA to follow-up catheterization was 18 months. Arterial collaterals were detected on follow-up catheterization in 45 patients (59%). Factors associated with collateral development included a prior right-sided systemic-to-pulmonary arterial shunt, lower pre-BCPA end-diastolic ventricular pressure and pulmonary vascular resistance, and use and duration of cardiopulmonary bypass during the BCPA operation. Fourteen of the 45 patients (30%) underwent coil embolization of the collaterals. Forty-three patients have undergone extracardiac conduit Fontan, with 1 early and 1 late death. Collaterals were present in 22 of these patients, 7 of whom underwent pre-Fontan embolization. The duration from BCPA to Fontan was longer in patients with collaterals, but these patients were not more likely to have prolonged effusions than those without, and the duration of tube thoracostomy was significantly shorter in patients with collaterals. Embolization of collaterals did not affect the duration of effusions.Systemic-to-pulmonary arterial collaterals are common after BCPA. In contrast to prior reports, collaterals were not associated with a higher incidence of prolonged effusions after the Fontan procedure in our experience, and did not correlate with poor outcome.

  View details for Web of Science ID 000086808500059

  View details for PubMedID 10800823

• Surgical intervention for complications of transcatheter dilation procedures in congenital heart disease ANNALS OF THORACIC SURGERY McElhinney, D. B., Reddy, V. M., Moore, P., Brook, M. M., Hanley, F. L. 2000; 69 (3): 858-864

  Abstract

  Transcatheter interventions have assumed an important role in the management of many forms of congenital heart disease. While complications of transcatheter interventions are uncommon and usually minor, significant complications requiring operation do occur on occasion. The purpose of this report is to present our experiences with seven such complications, and to review the literature on this topic.Seven patients who required operation after a transcatheter dilation procedure between 1992 and 1998 are described. Three patients required retrieval of retained foreign bodies (stents or balloons), and repair of the underlying abnormality. Two patients underwent repair of fistulas between 2 great vessels, or a great vessel and a cardiac chamber. One patient required operation for a postdilation aneurysm. One patient underwent urgent repair of severe aortic regurgitation after balloon aortic valvuloplasty.All patients survived and are doing well, with no further need for catheter or operative intervention, from 8 months to 6 years after operation. Additional reported complications requiring operation are discussed as well.Operation for complications of catheter interventions in congenital heart disease is seldom necessary. Though uncommon, a variety of such complications may occur, including vascular, valvar, intracardiac, and foreign body complications. When operation is required, results are typically very good.

  View details for Web of Science ID 000086022000044

  View details for PubMedID 10750773

• Issues and outcomes in the management of supravalvar aortic stenosis ANNALS OF THORACIC SURGERY McElhinney, D. B., Petrossian, E., Tworetzky, W., Silverman, N. H., Hanley, F. L. 2000; 69 (2): 562-567

  Abstract

  Supravalvar stenosis of the aorta is an uncommon congenital cardiac anomaly that involves not only the supravalvar aorta but the entire aortic root. Despite considerable attention to the importance of maintaining the integrity of the aortic root during supravalvar reconstruction, there has been little focus on the management of other components of the aortic root and left ventricular outflow tract, including the aortic valve, subvalvar region, and coronary arteries.We reviewed the records of 36 consecutive patients with supravalvar aortic stenosis who underwent repair from 1992-1998 (median age, 4 years). Discrete stenosis was present in 29 patients, whereas the remaining 7 had the diffuse form of the disease. Associated anomalies of the aortic root and adjacent structures were present in 23 patients. The median pressure gradient across the left ventricular outflow tract was 70 mm Hg. Supravalvar stenosis was relieved by extended aortoplasty with a Y-shaped patch in 18 patients, resection of the stenotic segment of ascending aorta at the sinotubular junction with end-to-end anastomosis of the ascending aorta in 7, the Ross procedure in 4, and other techniques in 7. Additional procedures included aortic valvuloplasty in 10 patients, resection of subvalvar stenosis in 11, and procedures on the coronary arteries in 2.There was 1 perioperative death, and no reoperations or other significant complications. During follow-up (median 33 months), there were no deaths and 3 reoperations for replacement of the aortic valve with a pulmonary autograft (n = 1) or mechanical prosthesis (n = 2). The median pressure gradient across the left ventricular outflow tract was 10 mm Hg.In patients with supravalvar aortic stenosis, abnormalities of the aortic valve, subaortic region, and coronary arteries are frequently present as well. Management of these issues is as critical to the long-term outcome of these patients as reconstruction of the supravalvar aorta. Aggressive valvuloplasty may help decrease the incidence of late aortic valve replacement, whereas the Ross procedure may be a preferable approach in some patients with complex outflow tract obstruction.

  View details for Web of Science ID 000085382200061

  View details for PubMedID 10735699

• Revision of previous Fontan connections to total extracardiac cavopulmonary anastomosis: A multicenter experience 79th Annual Meeting of the American-Association-for-Thoracic-Surgery Marcelletti, C. F., Hanley, F. L., Mavroudis, C., McElhinney, D. B., Abella, R. F., Marianeschi, S. M., Seddio, F., Reddy, V. M., Petrossian, E., de la Torre, T., Colagrande, L., Backer, C. L., Cipriani, A., Iorio, F. S., FONTAN, F. MOSBY-ELSEVIER. 2000: 340–44

  Abstract

  Conversion to total extracardiac cavopulmonary anastomosis is an option for managing patients with dysfunction of a prior Fontan connection.Thirty-one patients (19.9 +/- 8.8 years) underwent revision of a previous Fontan connection to total extracardiac cavopulmonary anastomosis at four institutions. Complications of the previous Fontan connection included atrial tachyarrhythmias (n = 20), progressive heart failure (n = 17), Fontan pathway obstruction (n = 10), effusions (n = 10), pulmonary venous obstruction by an enlarged right atrium (n = 6), protein-losing enteropathy (n = 3), right atrial thrombus (n = 2), subaortic stenosis (n = 1), atrioventricular valve regurgitation (n = 3), and Fontan baffle leak (n = 5). Conversion to an extracardiac cavopulmonary connection was performed with a nonvalved conduit from the inferior vena cava to the right pulmonary artery, with additional procedures as necessary.There have been 3 deaths. Two patients died in the perioperative period of heart failure and massive effusions. The third patient died suddenly 8 months after the operation. All surviving patients were in New York Heart Association class I (n = 20) or II (n = 7), except for 1 patient who underwent heart transplantation. Early postoperative arrhythmias occurred in 10 patients: 4 required pacemakers, and medical therapy was sufficient in 6. In 15 patients, pre-revision arrhythmias were improved. Effusions resolved in all but 1 of the patients in whom they were present before revision. The condition of 2 patients with protein-losing enteropathy improved within 30 days.Conversion of a failing Fontan connection to extracardiac cavopulmonary connection can be achieved with low morbidity and mortality. Optimally, revision should be undertaken early in symptomatic patients before irreversible ventricular failure ensues.

  View details for Web of Science ID 000085509800031

  View details for PubMedID 10649210

• Extracardiac conduit variation of the Fontan procedure. Advances in cardiac surgery Petrossian, E., Thompson, L. D., Hanley, F. L. 2000; 12: 175-198

  Abstract

  The ECF operation is designed to improve postoperative outcome by enhancing factors that are critical in optimal functioning of the Fontan circulation, including preservation of ventricular and pulmonary vascular function, avoidance of dysrhythmias, and prevention of stasis and flow turbulence in the Fontan circuit. Preoperative strategies include an early bidirectional Glenn procedure, and avoiding ancillary intracardiac procedures at the time of the Fontan by performing them at the time of the Glenn operation. Operative strategies include minimizing the duration of CPB by performing the conduit to pulmonary artery anastomosis off bypass, using partial instead of full CPB by cannulating the IVC alone, avoiding hypothermia, avoiding cross-clamping of the aorta, avoiding atrial incisions and suture lines, using a tubular conduit to construct the Fontan pathway, making a large conduit to pulmonary artery anastomosis, incorporating the conduit into aggressive pulmonary arterioplasties, and offsetting of the superior and inferior cavopulmonary anastomoses.

  View details for PubMedID 10949650

• A 5-year experience with surgical repair of atrial septal defect employing limited exposure CARDIOLOGY IN THE YOUNG Khan, J. H., McElhinney, D. B., Reddy, V. M., Hanley, F. L. 1999; 9 (6): 572-576

  Abstract

  There has been a trend in recent years towards less invasive therapy for many congenital cardiac malformations. For the past 5 years, we have employed a technique of limited surgical exposure when repairing atrial defects within the oval fossa.Over the 5-year period from July 1992 to August 1997, 115 consecutive patients underwent surgical repair of an isolated atrial septal defect in the region of the oval fossa by a single surgeon. The patients had a limited midline skin incision starting at the line of the nipples and extending inferiorly across 2 to 3 intercostal spaces. A partial sternotomy was performed, sparing the manubrium. Standard instruments and cannulation techniques were used for cardiopulmonary bypass and fibrillatory arrest.There were no deaths and no major complications. The median time to extubation after leaving the operating room was 3 hours (30 minutes to 8 days). Mediastinal drains were removed the morning after surgery. The median stay in the intensive care unit was 7 hours (3 hours to 10 days), and patients were discharged from the hospital a median of 4 days postoperatively (2 to 23 days).This approach using limited exposure can be applied safely without any new instruments and without peripheral incisions or sites of vascular access, while providing a comfortable exposure for the surgeon and achieving a cosmetically superior result for the patient.

  View details for Web of Science ID 000085038300007

  View details for PubMedID 10593266

• Is it necessary to routinely fenestrate an extracardiac fontan? JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY Thompson, L. D., Petrossian, E., McElhinney, D. B., Abrikosova, N. A., Moore, P., Reddy, V. M., Hanley, F. L. 1999; 34 (2): 539-544

  Abstract

  This study was conducted to assess the need for, and use of, fenestration of an extracardiac conduit Fontan.Fenestration of a Fontan connection has been proposed as a means of improving outcomes of single ventricle palliation. The benefit of fenestration is likely to be greatest in the early postoperative period when patients may experience increased pulmonary vascular resistance and decreased ventricular function due to the effects of cardiopulmonary bypass, aortic cross-clamping and positive pressure ventilation. However, there are potential drawbacks to fenestration. The utility of fenestration with extracardiac Fontan operation has not been determined.Since 1992, 81 patients have undergone a modification of the Fontan procedure in which an extracardiac inferior cavopulmonary conduit is used in combination with a previously staged bidirectional Glenn anastomosis. We conducted a retrospective review of these patients.Fenestration was performed selectively in 32 patients (39%), including only 2 of the last 38 (5%). In seven patients, a fenestration was placed or clipped in the early postoperative period without cardiopulmonary bypass. There were two operative deaths. Prolonged (>2 weeks) pleural drainage occurred in 13 patients, 8 with fenestration and 5 without. In addition to undergoing earlier Fontan in our experience, patients who had a fenestration placed had significantly higher preoperative pulmonary vascular resistance, significantly higher common atrial pressure after Fontan and significantly lower post-Fontan systemic arterial oxygen saturation. Fontan pressure did not differ between nonfenestrated and fenestrated patients. At follow-up ranging to five years, there were two late deaths and no patients developed protein losing enteropathy.Fenestration is not necessary in most Fontan patients when an extracardiac conduit technique is performed as described in this article, and therefore, should not be performed routinely with the extracardiac conduit Fontan. The need for fenestration should be assessed after cardiopulmonary bypass when hemodynamics can be evaluated accurately. Fenestration can be placed and revised easily without bypass and with minimal intervention in patients with an extracardiac conduit Fontan.

  View details for Web of Science ID 000083039200033

  View details for PubMedID 10440170

• Asymmetrically short tendinous cords causing congenital tricuspid regurgitation: improved understanding of tricuspid valvar dysplasia in the era of color flow echocardiography CARDIOLOGY IN THE YOUNG McElhinney, D. B., Silverman, N. H., Brook, M. M., Hanley, F. L., Stanger, P. 1999; 9 (3): 300-304

  Abstract

  Tricuspid regurgitation as a manifestation of an isolated congenital anomaly of the tricuspid valve is rare. Cross-sectional and color Doppler echocardiography allow improved evaluation of tricuspid valvar function. As a result, the heterogeneous category of congenital tricuspid valvar dysplasia may be better understood from a functional point of view. We are reporting a distinct entity in which tricuspid valvar regurgitation results from failure of coaptation due to short tendinous cords tethering the septal leaflet.Three children with significant primary tricuspid regurgitation were evaluated, treated, and followed. On echocardiographic evaluation, a central regurgitant jet of moderate or severe degree was directed toward the atrial septum through poorly coapting tricuspid valvar leaflets, which did not approximate due to tethering of the septal leaflet by abnormally short cords. In one patient, the tricuspid valve was otherwise normal; in the other two the leaflets and cords were also thickened. Two patients underwent surgery at 9 and 11 years of age. The cords tethering the septal leaflet were augmented by interposing appropriate lengths of expanded polytetrafluoroethylene suture and performing commissural annuloplasty. Both patients are asymptomatic 33 and 42 months postoperatively, with mild residual tricuspid regurgitation that has not changed since surgery. The other patient, an 8 month-old infant, has not yet undergone surgery.Asymmetric tendinous cords of the tricuspid valve causing tethering of the septal leaflet is a distinct cause of tricuspid regurgitation that can be recognized with echocardiography. Although rare, the importance of recognizing this lesion lies in its being readily amenable to surgical repair.

  View details for Web of Science ID 000083408600010

  View details for PubMedID 10386700

• Resection of subaortic stenosis; can a more aggressive approach be justified? EUROPEAN JOURNAL OF CARDIO-THORACIC SURGERY Parry, A. J., Kovalchin, J. P., Suda, K., McElhinney, D. B., Wudel, J., Silverman, N. H., Reddy, V. M., Hanley, F. L. 1999; 15 (5): 631-638

  Abstract

  Discrete subaortic stenosis causes left ventricular outflow tract (LVOT) obstruction and often produces aortic regurgitation (AR) which alone may precipitate surgical intervention. Conventional resection relieves the obstruction, but the recurrence rate is high, and the AR is little changed as the thick fibrous membrane which extends onto the valve leaflets remains. We studied whether an aggressive surgical approach could reduce both the severity of AR and rate of recurrence of obstruction associated with discrete subaortic stenosis, and whether this aggressive approach could be justified.Between June 1992 and April 1996, 37 patients aged 0.5-35 years (median 7.5) underwent resection of a discrete subaortic membrane. Ten underwent re-operation for recurrent obstruction and eight followed previous ventricular septal defect closure. LVOT gradient was measured using the modified Bernoulli equation and AR was graded on a scale of 0-4 (0 = none, 4 = severe). Postoperative assessment was performed early (<7 days) and at mid-term (27.0 months; range 2-59 months).There was significant improvement in AR from mild/moderate to none/trivial (P = 0.019) immediately postoperatively and LVOT gradient from 66.9+/-30.4 to 15.1+/-12.2 mmHg (P < 0.0001). By stepwise logistic regression preoperative gradient correlated significantly with postoperative mild/moderate AR (P = 0.015) and LVOT gradient (P = 0.0036). Preoperative mild/moderate AR also correlated with postoperative mild/moderate AR (P = 0.034). Five patients developed complete heart block, four undergoing reoperation for recurrent obstruction, and one preoperatively had right bundle branch block from previous ventricular septal defect repair. At mid-term follow-up there was no increase in AR or LVOT gradient (14.8+/-12.8 mmHg). Early post-operative AR was the strongest predictor of late mild/moderate AR (P = 0.02). Early post-operative gradient was a weaker predictor (P = 0.04). Pre-operative and early post-operative gradient were significant predictors of late gradient (P = 0.0038; <0.0001, respectively). No patient required reoperation for recurrent obstruction; one underwent late aortic valve replacement for severe AR.An aggressive surgical approach to discrete subaortic stenosis produces excellent relief of obstruction and frees the valve leaflets, significantly reducing associated AR at early and mid-term follow-up with low morbidity for primary operation. Long-term follow-up is required to confirm whether this early benefit is maintained.

  View details for Web of Science ID 000080692000021

  View details for PubMedID 10386409

• Compression of the central airways by a dilated aorta in infants and children with congenital heart disease ANNALS OF THORACIC SURGERY McElhinney, D. B., Reddy, V. M., Pian, M. S., Moore, P., Hanley, F. L. 1999; 67 (4): 1130-1136

  Abstract

  Children with congenital heart disease often experience respiratory symptoms in the preoperative and perioperative periods, which can complicate their management. An uncommon but important cause of respiratory insufficiency in such children is external airway compression.We operated on 5 patients (median age, 6 months) with significant respiratory distress attributable to compression of the central airways by a dilated ascending aorta before or after repair of concomitant cardiovascular defects. Four of these patients had right aortic arch and 3 had pulmonary atresia with a ventricular septal defect and major aortopulmonary collaterals. In all patients, aortopexy was performed at the time of operation for the cardiovascular defects (n = 3) or after symptoms developed in the postoperative period (n = 2). The 3 patients in whom airway compression produced symptoms preoperatively also underwent reduction ascending aortoplasty.Symptoms resolved immediately after operation in 3 patients, whereas symptoms persisted in the other 2 patients and tracheostomy was required. At follow-up of 20 months to 5 years, all patients are alive and well, with mild or moderate respiratory symptoms in the 2 patients who required tracheostomy, both of whom were decannulated within 13 months.External airway compression can cause significant morbidity in patients with congenital heart defects other than vascular rings. In patients with respiratory symptoms in the context of a lesion that involves increased aortic outflow during intrauterine life and consequently, an enlarged ascending aorta, such as tetralogy of Fallot with pulmonary atresia, airway compression should be considered as a cause, especially if a right aortic arch is present or the patient also has pulmonary atresia with a ventricular septal defect and collaterals. Attempts to address this problem surgically may provide substantial relief, but increasing duration of airway compression is likely to lead to tracheal or bronchial malacia and persistent symptoms even after the compression is relieved.

  View details for Web of Science ID 000080115300050

  View details for PubMedID 10320262

• Congenital obstructive lesions of the right aortic arch ANNALS OF THORACIC SURGERY McElhinney, D. B., Tworetzky, W., Hanley, F. L., Rudolph, A. M. 1999; 67 (4): 1194-1202

  Abstract

  In the setting of normal cardiac situs, a right-sided aortic arch is uncommon. When a right arch does occur, it is typically in conjunction with other congenital cardiovascular anomalies, especially defects with abnormal right ventricular outflow. Congenital obstruction of a right arch, caused by coarctation, interruption, or cervical arch, is extremely rare.We reviewed our experience and all reported cases of right aortic arch with coarctation of the aorta, interrupted arch, or obstruction of a cervical arch in the setting of normal cardiac situs and topology.Since 1992, 4 such patients have undergone repair at our institution, including 1 with interrupted arch, 1 with coarctation of a mirror image arch, and 2 with obstruction of a cervical arch. In addition to these 4 patients, 38 others have been described in the published reports: 15 with interrupted arch, 19 with coarctation, and 4 with obstruction of a cervical arch. Associated cardiac defects were uncommon, except for ventricular septal defect in patients with interrupted arch, but abnormalities of the brachiocephalic vessels were frequent. Except for most of the patients with interrupted right arch, the majority of patients described have undergone successful surgical repair.Although obstructive arch lesions are often grouped together, the etiologies of coarctation of the aorta, interrupted arch, and cervical arch with obstruction almost certainly differ. The rarity of such lesions among patients with right aortic arch may be explained in part by the fact that the fetal hemodynamic conditions associated with persistence of a right arch do not facilitate flow-related arch obstruction. In this review, we discuss these issues in detail, along with specific surgical considerations in the management of obstruction lesions of the right aortic arch.

  View details for Web of Science ID 000080115300078

  View details for PubMedID 10320289

• Early results of the extracardiac conduit Fontan operation JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY Petrossian, E., Reddy, V. M., McElhinney, D. B., Akkersdijk, G. P., Moore, P., Parry, A. J., Thompson, L. D., Hanley, F. L. 1999; 117 (4): 688-695

  Abstract

  Among the modifications of the Fontan operation, the extracardiac approach may offer the greatest potential for optimizing early postoperative ventricular and pulmonary vascular function, insofar as it can be performed with short periods of normothermic partial cardiopulmonary bypass and without cardioplegic arrest in most cases. In this study, we reviewed our experience with the extracardiac conduit Fontan operation, with a focus on early postoperative outcomes.Between July 1992 and April 1997, 51 patients (median age 4.9 years) underwent an extracardiac conduit Fontan operation. Median cardiopulmonary bypass time was 92 minutes and has decreased significantly over the course of our experience. Intracardiac procedures were performed in only 5 patients (10%), and the aorta was crossclamped in only 11 (22%). Intraoperative fenestration was performed in 24 patients (47%). There were no early deaths. Fontan failure occurred in 1 patient who was a poor candidate for the Fontan procedure. Transient supraventricular tachyarrhythmias occurred in 5 patients (10%). Median duration of chest tube drainage was 8 days. Factors significantly associated with prolonged resource use (mechanical ventilation, inotropic support, intensive care unit stay, and hospital stay) included longer bypass time and higher Fontan pressure. At a median follow-up of 1.9 years, there was 1 death from bleeding at reoperation.The extracardiac conduit Fontan procedure can be performed with minimal mortality and morbidity. Improved results may be related to advantages of the extracardiac approach and improved preservation of ventricular and pulmonary vascular function.

  View details for Web of Science ID 000079520000008

  View details for PubMedID 10096963

• Endothelin receptor blockade prevents the rise in pulmonary vascular resistance after cardiopulmonary bypass in lambs with increased pulmonary blood flow 24th Annual Meeting of the Western-Thoracic-Surgical-Association Petrossian, E., Parry, A. J., Reddy, V. M., Akkersdijk, G. P., McMullan, D. M., Thompson, L., Hendricks-Munoz, K. D., Hallak, H., Hanley, F. L., Fineman, J. R. MOSBY-ELSEVIER. 1999: 314–22

  Abstract

  Children with increased pulmonary blood flow may experience morbidity as the result of increased pulmonary vascular resistance after operations in which cardiopulmonary bypass is used. Plasma levels of endothelin-1, a potent vasoactive substance implicated in pulmonary hypertension, are increased after cardiopulmonary bypass.In a lamb model of increased pulmonary blood flow after in utero placement of an aortopulmonary shunt, we characterized the changes in pulmonary vascular resistance induced by hypothermic cardiopulmonary bypass and investigated the role of endothelin-1 and endothelin-A receptor activation in postbypass pulmonary hypertension.In eleven 1-month-old lambs, the shunt was closed, and vascular pressures and blood flows were monitored. An infusion of a selective endothelin-A receptor blocker (PD 156707; 1.0 mg/kg/h) or drug vehicle (saline solution) was then begun 30 minutes before cardiopulmonary bypass and continued for 4 hours after bypass. The hemodynamic variables were monitored, and plasma endothelin-1 concentrations were determined before, during, and for 6 hours after cardiopulmonary bypass.After 90 minutes of hypothermic cardiopulmonary bypass, both pulmonary arterial pressure and pulmonary vascular resistance increased significantly in saline-treated lambs during the 6-hour study period (P <.05). In lambs pretreated with PD 156707, pulmonary arterial pressure and pulmonary vascular resistance decreased (P <. 05). After bypass, plasma endothelin-1 concentrations increased in all lambs; there was a positive correlation between postbypass pulmonary vascular resistance and plasma endothelin-1 concentrations (P <.05).This study suggests that endothelin-A receptor-induced pulmonary vasoconstriction mediates, in part, the rise in pulmonary vascular resistance after cardiopulmonary bypass. Endothelin-A receptor antagonists may decrease morbidity in children at risk for postbypass pulmonary hypertension. This potential therapy warrants further investigation.

  View details for Web of Science ID 000078353700024

  View details for PubMedID 9918974

• Role of the endothelium in placental dysfunction after fetal cardiac bypass 78th Annual Meeting of the American-Association-for-Thoracic-Surgery Reddy, V. M., McElhinney, D. B., Rajasinghe, H. A., Liddicoat, J. R., Hendricks-Munoz, K., Fineman, J. R., Hanley, F. L. MOSBY-ELSEVIER. 1999: 343–51

  Abstract

  Fetal cardiac bypass causes placental dysfunction, characterized by increased placental vascular resistance, decreased placental blood flow, hypoxia, and acidosis. Vasoactive factors produced by the vascular endothelium, such as nitric oxide and endothelin 1, are important regulators of placental vascular tone and may contribute to this placental dysfunction.To investigate the role of the vascular endothelium in placental dysfunction related to fetal cardiac bypass, we studied 3 groups of fetal sheep. In the first group (n = 7) we determined placental hemodynamic responses before and after bypass to an endothelium-dependent vasodilator (acetylcholine), an endothelium-independent vasodilator (nitroprusside), and endothelin 1. In the second group (n = 8) a nonspecific endothelin receptor blocker (PD 145065) was administered and placental hemodynamic values were measured before and after bypass. In the third group (n = 5) endothelin 1 levels were measured before and after bypass.Before fetal cardiac bypass exogenous endothelin 1 decreased placental blood flow by 9% and increased placental resistance by 9%. After bypass endothelin 1 decreased placental flow by 47% and increased resistance by 106%. There was also a significant attenuation of the placental vascular relaxation response to acetylcholine after bypass, whereas the response to nitroprusside was not significantly altered. In fetuses that received the PD 145065, placental vascular resistance increased significantly less than in control fetuses (28% versus 62%). Similarly, placental blood flow decreased significantly more (from 6. 3 +/- 3.1 to 28.3 +/- 10.4 pg/mL; P =.01) in control fetuses than in fetuses receiving PD 145065 (33% versus 20%). Umbilical venous endothelin 1 levels increased significantly in fetuses exposed to fetal bypass but did not change in control fetuses.The basal endothelial regulatory mechanisms of placental vascular tone were deranged after fetal cardiac bypass. Endothelin receptor blockade, which substantially reduced postbypass placental dysfunction, and other interventions aimed at preserving endothelial function may be effective means of optimizing fetal outcome after cardiac bypass.

  View details for Web of Science ID 000078353700030

  View details for PubMedID 9918976

• The use of 'pericardial hoods' for maintaining exact coronary artery geometry in the arterial switch operation with complex coronary anatomy EUROPEAN JOURNAL OF CARDIO-THORACIC SURGERY Parry, A. J., Thurm, M., Hanley, F. L. 1999; 15 (2): 159-164

  Abstract

  Complex coronary artery anatomy is the major risk factor for the arterial switch operation. Of the many approaches described the 'trap door' technique for coronary reimplantation is most flexible and allows safer transfer in complex arterial configurations. However, we have occasionally been concerned regarding torsion of the vessels with this approach. We therefore explored the role of trap-door augmentation with pericardial hoods to maintain exact coronary geometry during coronary transfer.Between February 1992 and December 1997, 80 patients underwent an arterial switch procedure at our institution. Sixty-seven patients underwent direct coronary reimplantation. In ten, coronary/great vessel anatomy was considered unfavourable and the trap-door approach was adopted primarily. In two an augmented trap-door was performed as the primary procedure and in the last patient Aubert's approach was used. In five patients during rewarming, ischaemic changes were noted on the electrocardiogram and/or regional wall motion abnormalities on transoesophageal echocardiography. This prompted revision of the appropriate coronary anastomosis. In three it was considered the anastomosis was kinked due to angulation of the button; in two the coronary was overstretched. In four, revision of the anastomosis was by pericardial hood augmentation.In all patients there was normalization of the electrocardiogram and immediate improvement in cardiac function documented by transoesophageal echocardiography. No early or late death occurred in the pericardial hood group nor were there any readmissions for any reason.Pericardial augmentation of trap-door aortic anastomoses allows for the maintenance of exact coronary artery geometry during the arterial switch procedure and minimizes the risk of myocardial ischaemia. We believe it broadens the application of the arterial switch procedure to even the most complex coronary anatomy and is a useful adjunct to the other techniques of coronary transfer.

  View details for Web of Science ID 000080762600009

  View details for PubMedID 10219548

• Results of 102 cases of complete repair of congenital heart defects in patients weighing 700 to 2500 grams 78th Annual Meeting of the American-Association-for-Thoracic-Surgery Reddy, V. M., McElhinney, D. B., Sagrado, T., Parry, A. J., Teitel, D. F., Hanley, F. L. MOSBY-ELSEVIER. 1999: 324–30

  Abstract

  Published data suggest that low birth weight is a risk factor for poor outcome in corrective surgery for many cardiac defects. Congenital heart defects in low birth weight infants are typically managed with supportive therapy or palliative operations, with definitive repair delayed. The morbidity associated with such approaches is high.Since 1990 complete repair of congenital heart defects (other than patent ductus arteriosus) has been performed in 102 infants no larger than 2500 g (median 2100 g, range 700-2500 g), including 16 no larger than 1500 g. Defects included ventricular septal defect (n = 22), tetralogy of Fallot complexes (n = 20), transposition complexes (n = 13), aortic coarctation (n = 12), interrupted arch (n = 10), truncus arteriosus (n = 8), atrioventricular septal defect (n = 6), total anomalous pulmonary venous return (n = 5), and other (n = 6).Preoperative morbidity was more common among patients referred late for surgical correction. There were 10 early deaths (10%) attributable to cardiac failure (n = 4), arrhythmia (n = 1), multiorgan failure (n = 1), sepsis (n = 1), idiopathic coronary artery intimal necrosis (n = 1), foot gangrene (n = 1), and pulmonary hemorrhage (n = 1). No patient had postbypass intracerebral hemorrhage. At follow-up (median 36 months) there were 8 late deaths, and 8 patients underwent 10 reinterventions. There was no evidence of neurologic sequelae attributable to the operation.In general, delaying repair of congenital heart defects in low birth weight infants does not confer a benefit and is associated with higher preoperative morbidity. Complete repair of both simple and complex lesions can be achieved in such cases with good results. Growth after repair approximates the normal curve for low birth weight infants without heart disease. It is recommended that such infants, especially when they have symptoms, undergo early surgical repair rather than prolonged medical management or other forms of palliation.

  View details for Web of Science ID 000078353700026

  View details for PubMedID 10049033

• Echocardiographic diagnosis alone for the complete repair of major congenital heart defects JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY Tworetzky, W., McElhinney, D. B., Brook, M. M., Reddy, V. M., Hanley, F. L., Silverman, N. H. 1999; 33 (1): 228-233

  Abstract

  The study was done to determine the diagnostic accuracy of echocardiography alone in the preoperative diagnosis of children with major congenital heart defects undergoing primary complete repair.Although echocardiography is well established as the first-line imaging technique for the diagnosis of all forms of congenital heart disease, most institutions continue to perform cardiac catheterization prior to complete repair of more complex defects.To determine the diagnostic accuracy of echocardiography alone and echocardiography plus catheterization, we reviewed the records of 503 children with major congenital heart defects who underwent primary complete repair at our institution between July 1992 and June 1997. We included children with transposition of the great arteries, tetralogy of Fallot, double-chamber right ventricle, interrupted aortic arch, aortic coarctation, atrioventricular septal defect, truncus arteriosus, aortopulmonary septal defect, and totally anomalous pulmonary venous return. We excluded children with less complex defects such as isolated shunt lesions, as well as those with the most complex defects that would require surgical palliation (e.g., functional univentricular heart). We defined major errors as those that increased the surgical risk and minor errors as those that did not. Errors in diagnosis were determined at surgery.Eighty-two percent of children (412 of 503) underwent surgery after preoperative diagnosis by echocardiography alone. There were 9 major (2%) and 10 minor errors in the echocardiography alone group and 7 major and 5 minor errors in the echocardiography plus catheterization group. The most common type of error was misidentification of coronary artery anatomy in patients with transposition of the great arteries. No error in either group resulted in surgical morbidity or mortality.This study suggests that echocardiography alone is an accurate tool for the preoperative diagnosis of major congenital heart defects in most children undergoing primary complete repair, and may obviate the need for routine diagnostic catheterization.

  View details for Web of Science ID 000078088300036

  View details for PubMedID 9935035

• Multiple ventricular septal defects: How and when should they be repaired? 78th Annual Meeting of the American-Association-for-Thoracic-Surgery Seddio, F., Reddy, V. M., McElhinney, D. B., Tworetzky, W., Silverman, N. H., Hanley, F. L. MOSBY-ELSEVIER. 1999: 134–39

  Abstract

  Congenital heart lesions with multiple ventricular septal defects remain a surgical challenge. Traditional approaches often rely on either ventriculotomy for exposure or palliation with pulmonary artery banding. However, indications for repair versus palliation and for various approaches to surgical exposure are not clearly defined.From July 1992 to January 1998, 45 patients with multiple (>/=2) ventricular septal defects (37 with associated lesions) underwent surgery. Median age was 86 days; all but 4 patients were infants. The mean number of defects was 3.7, and almost half of the patients had more than 3 defects. Apical muscular defects were present in 62% of patients. Thirty-one patients underwent primary complete repair through a right atriotomy or trans-semilunar valve approach (group 1), 8 had palliation (group 2), and 6 underwent complete repair after prior palliation elsewhere (group 3). No patient had a ventriculotomy.One early death occurred in a group 1 patient. Four patients who had had palliation (50%) underwent early reoperation for pulmonary artery band revision because of failure to thrive or band removal after spontaneous closure of the defects. At follow-up (median 22 months), there was 1 death in a group 2 patient (palliation) and 1 other group 2 patient required cardiac transplantation. The only late reoperation was for removal of the pulmonary artery band and closure of multiple apical defects in a group 2 (palliation) patient. No patients who underwent repair have hemodynamically significant residual defects.In our experience, palliation of multiple ventricular septal defects is associated with greater morbidity than primary repair. Multiple defects can almost always be repaired adequately in early infancy without ventriculotomy, although "Swiss-cheese" septum may be an indication for palliation.

  View details for Web of Science ID 000077898200027

  View details for PubMedID 9869767

• Accessory and anomalous atrioventricular valvar tissue causing outflow tract obstruction - Surgical implications of a heterogeneous and complex problem JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY McElhinney, D. B., Reddy, V. M., Silverman, N. H., Hanley, F. L. 1998; 32 (6): 1741-1748

  Abstract

  The purpose of this study was to determine the effect of accessory or anomalous atrioventricular valvar apparatus on relief of outflow tract obstruction.Outflow tract obstruction due to accessory tissue or anomalous attachments of the atrioventricular valvar apparatus is an unusual but well-recognized problem. In addition to obstruction, anomalous attachments of the atrioventricular valvar apparatus may interfere with procedures to relieve outflow tract obstruction or perform outflow tract reconstruction.Since 1992, we have operated on 21 patients (median age 4 years) with systemic (n=13), pulmonary (n=5) or bilateral (n=3) outflow tract obstruction due to accessory atrioventricular valvar tissue and/or anomalous attachments of the subvalvar apparatus. Primary diagnoses were isolated obstruction of the systemic outflow tract or aortic arch (n=7), transposition complexes (n=6), previously repaired atrioventricular septal defect (n=3), functionally single ventricle (n=3) and ventricular septal defect with pulmonary outflow obstruction (n=2). Outflow tract gradients ranged from 20-110 mm Hg (median 58 mm Hg).Complete relief of obstruction due to atrioventricular valvar anomalies was possible in 14 patients. In six patients, the planned procedure either had to be modified or only partial relief of the obstruction was achieved. In the remaining patient, who had borderline functionally single ventricle heart disease (unbalanced atrioventricular septal defect) and systemic outflow obstruction due to accessory and functional valvar apparatus, support was withdrawn because the parents refused univentricular palliation and the valvar anomalies precluded a Ross-Konno procedure. There were two early deaths. At follow-up ranging from 1 to 66 months (median 27 months), there was one death, and there has been no recurrence of outflow tract obstruction or residual atrioventricular valvar tissue.Outflow tract obstruction caused by accessory or anomalous atrioventricular valvar structures is an uncommon and heterogeneous group of conditions that can have significant surgical implications. In the majority of cases, tailoring of surgical techniques will permit complete relief of obstruction. However, such anomalies may limit standard surgical options and necessitate an innovative approach in some patients.

  View details for Web of Science ID 000076981500022

  View details for PubMedID 9822104

• Extracardiac conduit Fontan procedure without cardiopulmonary bypass ANNALS OF THORACIC SURGERY McElhinney, D. B., Petrossian, E., Reddy, V. M., Hanley, F. L. 1998; 66 (5): 1826-1828

  Abstract

  There are a number of potential advantages of extracardiac conduit cavopulmonary anastomosis for palliation of functional single ventricle heart disease, including the ability to perform the operation with no aortic cross-clamping and with minimal duration of extracorporeal circulation. In many patients, it may be possible to perform the procedure without cardiopulmonary bypass altogether. In this report, we present our technique for performing the extracardiac conduit Fontan operation without cardiopulmonary bypass.

  View details for Web of Science ID 000077703300095

  View details for PubMedID 9875809

• Intramyocardial hematoma causing cardiac tamponade after repair of Ebstein malformation: Erroneous echocardiographic diagnosis as intracavitary thrombus JOURNAL OF THE AMERICAN SOCIETY OF ECHOCARDIOGRAPHY Momenah, T. S., McElhinney, D. B., Brook, M. M., Teitel, D. F., Hanley, F. L., Silverman, N. H. 1998; 11 (11): 1087-1089

  Abstract

  Intramyocardial hematoma is an uncommon lesion, most often occurring after chest trauma, aortic valve disease, acute myocardial infarction, or coronary artery bypass surgery. We describe a 17-year-old patient who experienced cardiac tamponade after repair of Ebstein malformation. The malformation was caused by an enlarging intramyocardial hematoma that extended from the right atrium to the atrialized right ventricle. The hematoma was incorrectly diagnosed by echocardiography as an intracavitary thrombus, and the correct diagnosis was recognized only at the time of surgical intervention.

  View details for Web of Science ID 000077038900014

  View details for PubMedID 9812104

• Alternative approach to the repair of Ebstein's malformation: Intracardiac repair with ventricular unloading ANNALS OF THORACIC SURGERY Marianeschi, S. M., McElhinney, D. B., Reddy, V. M., Silverman, N. H., Hanley, F. L. 1998; 66 (5): 1546-1550

  Abstract

  Moderate to severe Ebstein's malformation remains a surgical challenge. Although the various approaches that have been used are appropriate and successful in many patients, there are many for which these approaches are suboptimal. To improve the prognosis for patients across the full spectrum of Ebstein's malformation, alternative surgical approaches are necessary.From December 1995 to October 1997, 10 patients (median age, 9 years) with moderate or severe Ebstein's malformation and mild to severe tricuspid regurgitation had partial biventricular repair with reduction of right ventricular volume load. All patients were symptomatic in New York Heart Association functional class II (n = 9) or III (n = 1). In addition to bidirectional cavopulmonary anastomosis and closure of intracardiac defects in all patients, 6 underwent tricuspid valve repair using a variety of procedures, most often simple horizontal annuloplasty.There were no deaths. Early reoperation was required in 1 patient (atrial septostomy on the day after operation for right ventricular failure) and another required revision of the tricuspid valve repair 10 months postoperatively for recurrent regurgitation. At follow-up ranging from 2 to 24 months, all patients are in New York Heart Association class I and have trivial tricuspid regurgitation, including the 4 who had no tricuspid valvuloplasty performed.We have presented an alternative approach to the management of severe Ebstein's malformation that focuses on both the tricuspid valve and the right ventricle. Just as tricuspid valve repair and reduction of regurgitation will likely improve right ventricular performance, reducing the volume load on the ventricle may improve both ventricular (right and left) and tricuspid valve function. All patients have demonstrated improved exercise tolerance and right heart function at follow-up ranging to 24 months. Additional experience will be necessary to evaluate this strategy more completely.

  View details for Web of Science ID 000077703300014

  View details for PubMedID 9875749

• Repair of secundum atrial septal defect: Limiting the incision without sacrificing exposure ANNALS OF THORACIC SURGERY Khan, J. H., McElhinney, D. B., Reddy, V. M., Hanley, F. L. 1998; 66 (4): 1433-1435

  Abstract

  A simple and effective technique for repair of secundum atrial septal defect is described. The heart is exposed through a limited midline skin incision and partial sternotomy, and the atrial septal defect is closed through a right atriotomy with ascending aortic and dual venous cannulation. This approach achieves a cosmetically superior result with standard instrumentation and cardiopulmonary bypass techniques, without compromising exposure or using peripheral incisions.

  View details for Web of Science ID 000076692900087

  View details for PubMedID 9800859

• Atrioventricular septal defect with common valvar orifice and tetralogy of Fallot revisited: making a case for primary repair in infancy CARDIOLOGY IN THE YOUNG McElhinney, D. B., Reddy, V. M., Silverman, N. H., Brook, M. M., Hanley, F. L. 1998; 8 (4): 455-461

  Abstract

  Atrioventricular septal defect with common valvar orifice and tetralogy of Fallot is a rare combination of congenital cardiac anomalies. Approaches to this lesion have tended to emphasize either staged repair or complete repair beyond infancy. Between July 1992 and August 1997, nine patients underwent repair of complete atrioventricular septal defect with tetralogy of Fallot. One patient, aged 9.6 years at the time of repair, had previously undergone construction of a modified Blalock-Taussig shunt. Primary complete repair was performed in the other 8 patients at ages ranging from 2.5 to 16 months (median 4.6 months), and all but one were infants. All patients had a Rastelli type C defect, a single ventricular septal defect with inlet and outlet components, and malalignment of the muscular outlet septum with subpulmonary stenosis. A single patch technique, with closure of the zone of apposition ('cleft') in the left atrioventricular valve, was used in all eight patients undergoing primary repair, while a double patch was employed in the previously palliated older patient. In all cases of repair using a single patch, the anterosuperior bridging leaflet was divided obliquely to the right, following the malaligned outlet septum, in order to avoid subaortic obstruction. Repair of the right ventricular outflow tract included infundibular myectomy in eight, pulmonary valvotomy in four, infundibular or transannular patching in three and one, respectively, and reconstruction with a valved allograft conduit in two patients. There was no early mortality or significant morbidity. At a median follow-up of 45 months, there had been one death related to non-cardiac causes and no reinterventions. Left atrioventricular valvar regurgitation was moderate or mild in two patients, and right atrioventricular valvar regurgitation was mild in one patient. No patient had more than mild pulmonary regurgitation or a gradient across the right ventricular outflow tract in excess of 18 mm Hg. Our results demonstrate that primary repair of atrioventricular septal defect with tetralogy of Fallot can be performed with excellent early and mid-term results in young infants. Although it has been suggested that a technique utilizing oblique division of the anterosuperior bridging leaflet may lead to high rates of atrioventricular valvar regurgitation, medium-term atrioventricular valvar function in the present cohort of patients has been excellent.

  View details for Web of Science ID 000077254300010

  View details for PubMedID 9855099

• Tetralogy of Fallot with major aortopulmonary collaterals: Early total repair PEDIATRIC CARDIOLOGY McElhinney, D. B., Reddy, V. M., Hanley, F. L. 1998; 19 (4): 289-296

  Abstract

  Tetralogy of Fallot with pulmonary atresia and major aortopulmonary collaterals is a complex lesion distinguished by marked heterogeneity of pulmonary blood supply. Over the past two decades, investigators have developed various approaches to the management of this anomaly generally based on the concept of staged unifocalization of pulmonary blood supply. Although such approaches may represent an improvement on the natural history of this lesion, they remain inadequate for a substantial portion of patients born with tetralogy of Fallot and major aortopulmonary collaterals. Since 1992, our approach has been to perform one-stage complete unifocalization through a midline approach in all but a few extremely complicated patients. We aim to repair these patients early in infancy, with an emphasis on native tissue-tissue reconstruction, in order to optimize prospects for survival with a good functional outcome in as many patients as possible. In this review, we present our philosophy and our experience with unifocalization and repair in 72 patients.

  View details for Web of Science ID 000074204200004

  View details for PubMedID 9636252

• The effects of inhaled nitric oxide on postoperative pulmonary hypertension in infants and children undergoing surgical repair of congenital heart disease 46th Annual Meeting of the American-Society-of-Anesthesiologists Russell, I. A., Zwass, M. S., Fineman, J. R., Balea, M., Rouine-Rapp, K., Brook, M., Hanley, F. L., Silverman, N. H., Cahalan, M. K. LIPPINCOTT WILLIAMS & WILKINS. 1998: 46–51

  Abstract

  The role of inhaled nitric oxide in the immediate post-bypass period after surgical repair of congenital heart disease is uncertain. In a controlled, randomized, double-blind study, we tested the hypothesis that inhaled nitric oxide (NO) would reduce pulmonary hypertension immediately after surgical repair of congenital heart disease in 40 patients with preoperative evidence of pulmonary hypertension (mean pulmonary arterial pressure [MPAP] exceeding 50% of mean systemic arterial pressure [MSAP]). Patients were then followed in the intensive care unit (ICU) to document the incidence of severe pulmonary hypertension. Of the patients, 36% (n = 13) emerged from bypass with MPAP > 50% MSAP. In these patients, inhaled NO reduced MPAP by 19% (P = 0.008) versus an increase of 9% in the placebo group. No effect on MPAP was observed in patients emerging from bypass without pulmonary hypertension (n = 23). Inhaled NO was required five times in the ICU, always in the patients who had emerged from cardiopulmonary bypass with pulmonary hypertension (5 of 13 [38%] versus 0 of 23). We conclude that, in infants and children undergoing congenital heart surgery, inhaled NO selectively reduces MPAP in patients who emerge from cardiopulmonary bypass with pulmonary hypertension and has no effect on those who emerge without it.In a randomized double-blind study, inhaled nitric oxide selectively reduced pulmonary artery pressures in pediatric patients who developed pulmonary hypertension (high blood pressure in the lungs) immediately after cardiopulmonary bypass and surgical repair.

  View details for Web of Science ID 000074557500011

  View details for PubMedID 9661544

• Partial biventricular repair for complex congenital heart defects: An intermediate option for complicated anatomy or functionally borderline right complex heart 23rd Annual Meeting of the Western-Thoracic-Surgical-Association Reddy, V. M., McElhinney, D. B., Silverman, N. H., Marianeschi, S. M., Hanley, F. L. MOSBY-ELSEVIER. 1998: 21–27

  Abstract

  Partial biventricular repair consists of bidirectional cavopulmonary anastomosis in the presence of separated systemic and pulmonary circulations, with antegrade flow of inferior caval return through an intact or reconstructed pulmonary outflow tract. This versatile procedure may be used as a definitive repair for patients with a functional right heart complex incapable of supporting an entire cardiac output or in patients with complicated anatomy.From July 1992 to April 1997, 23 patients (median age 5.2 years) underwent partial biventricular repair. In 15 of these cases the entire repair, including bidirectional cavopulmonary anastomosis, intracardiac repair, and right ventricular outflow reconstruction, was performed as a planned procedure at our institution. The other eight patients had previously been placed on a Fontan track and had undergone bidirectional cavopulmonary anastomosis; their circulations were converted to a partial biventricular circulation.There were no early deaths. Complete atrioventricular block developed in two patients with straddling tricuspid valve. At a median follow-up of 17 months, there were no late deaths and three patients had undergone reintervention. Partitioning of the pulmonary arteries to create a classic Glenn anastomosis with antegrade flow to the left lung was performed in one case; another patient underwent an atrial septectomy, and the third patient required revision of tricuspid valve repair. All patients are in New York Heart Association functional class I.Partial biventricular repair is a versatile strategy that can be used to manage a variety of forms of complex congenital heart disease. Cases for which the repair is useful include those in which complete biventricular repair is unlikely to be achieved because of limited size or function of the right side of the heart and those in which a patient with a ventricle capable of supporting inferior vena caval return was previously placed on a Fontan track.

  View details for Web of Science ID 000074833500002

  View details for PubMedID 9671893

• Rare forms of isolation of the subclavian artery: echocardiographic diagnosis and surgical considerations CARDIOLOGY IN THE YOUNG McElhinney, D. B., Silverman, N. H., Brook, M. M., Reddy, V. M., Hanley, F. L. 1998; 8 (3): 344-351

  Abstract

  Isolation of the subclavian artery is an unusual anomaly in which the subclavian artery arises not from the aortic arch but from a pulmonary artery via an arterial duct. Such isolation most often occurs with a right aortic arch, and in lesions frequently associated with a right arch, such as tetralogy of Fallot. Since 1994, we have undertaken surgery in four young infants with isolated subclavian arteries and unusual associated anomalies, including one with atrioventricular septal defect and common valvar orifice, two with interruption of a left aortic arch and one with interruption of a right aortic arch. In both patients with interrupted left arch, the isolated subclavian artery was diagnosed preoperatively by echocardiography. We emphasize the significant surgical issues.

  View details for Web of Science ID 000075607500012

  View details for PubMedID 9731649

• Repair of congenital tricuspid valve abnormalities with artificial chordae tendineae ANNALS OF THORACIC SURGERY Reddy, V. M., McElhinney, D. B., Brook, M. M., Silverman, N. H., Stanger, P., Hanley, F. L. 1998; 66 (1): 172-176

  Abstract

  Congenital abnormalities of the tricuspid valve, including Ebstein's malformation, dysplasia, straddling, and those found in pulmonary atresia with intact septum and congenitally corrected transposition, are an uncommon cause of tricuspid regurgitation. Congenital tricuspid valve anomalies are found as a spectrum of disease in which both the leaflets and the subvalvar apparatus are often involved. Tricuspid valve repair is complicated in such patients because the chordae tendineae are often abnormally short and thick. Replacement or augmentation of chordae tendineae has proved to be a useful component of mitral valve repair. In the present report, we describe the techniques and results of chordal augmentation in the repair of congenital tricuspid valve abnormalities.Since July 1992, tricuspid valve repair has been performed in 5 children with severe tricuspid regurgitation secondary to congenital abnormalities of the tricuspid valve with significant chordal pathology. As a component of the repair, chordal replacement or augmentation was performed using expanded polytetrafluoroethylene suture.Intraoperative and postoperative echocardiographic assessment showed good mobility of the tricuspid valve leaflets and trivial to mild tricuspid regurgitation. There were no complications and no early or late mortality. At follow-up of 34 to 60 months (median, 49 months), tricuspid valve function has remained excellent in 4 of the 5 patients. In the remaining patient, progressive regurgitation of the right ventricle to pulmonary artery allograft conduit has led to right ventricular dilatation, with a secondary increase in tricuspid regurgitation from trivial to moderate.Chordal replacement or augmentation with expanded polytetrafluoroethylene suture is a useful technique in the repair of congenitally dysplastic tricuspid valves with abnormal chordal structures.

  View details for Web of Science ID 000074992000038

  View details for PubMedID 9692459

• Congenital cardiac anomalies with vein of Galen malformations in infants ARCHIVES OF DISEASE IN CHILDHOOD McElhinney, D. B., Halbach, V. V., Silverman, N. H., Dowd, C. F., Hanley, F. L. 1998; 78 (6): 548-551

  Abstract

  Published reports and personal experience are reviewed relating to patients under 1 year of age diagnosed with a vein of Galen malformation and congenital heart disease. Including five patients from this institution, a total of 23 patients (12 neonates) with congenital heart disease and a vein of Galen malformation have been reported. Six of these had sinus venosus atrial septal defect and nine had aortic coarctation.

  View details for Web of Science ID 000074060300011

  View details for PubMedID 9713012

• Geometric mismatch of pulmonary and aortic anuli in children undergoing the Ross procedure: Implications for surgical management and autograft valve function 77th Annual Meeting of the American-Association-for-Thoracic-Surgery Reddy, V. M., McElhinney, D. B., Phoon, C. K., Brook, M. M., Hanley, F. L. MOSBY-ELSEVIER. 1998: 1255–62

  Abstract

  There is often substantial mismatch between the diameters of the pulmonary and aortic anuli in young patients with systemic outflow tract disease. To implant the autologous pulmonary valve in the aortic position under such circumstances, it is necessary to adapt the geometry of the systemic outflow tract. The effects of such adaptations on autograft function in children are not well known.To determine factors predictive of autograft regurgitation, we analyzed 41 cases of children who have undergone the Ross procedure. The diameter of the pulmonary valve was greater (by at least 3 mm) than that of the aortic valve in 20 cases, equal (within 2 mm) in 12 cases, and less (by at least 3 mm) in nine cases, with differences ranging from +10 to -12 mm. In 12 patients with a larger pulmonary anulus, aortoventriculoplasty was used to correct the mismatch. In patients with a larger aortic anulus, the mismatch was corrected by gradual adjustment along the circumference of the autograft, rather than by tailoring of the native aortic anulus.At follow-up (median 31 months), two patients had undergone reoperation on the neoaortic valve for moderate regurgitation. In the remaining 38 cases, autograft regurgitation was as follows: none or trivial in 30, mild in seven, and moderate in one. There was no correlation between regurgitation and age, geometric mismatch, or previous or concurrent procedures.Subtle technical factors that may result in distortion of the valve complex are probably more important determinants of autograft regurgitation than are indication for repair, geometric mismatch, or previous or concomitant outflow tract procedures. Significant mismatch of the semilunar anuli is not a contraindication to the Ross procedure in children.

  View details for Web of Science ID 000074093600004

  View details for PubMedID 9628666

• Atrioventricular valve function after single patch repair of complete atrioventricular septal defect in infancy: How early should repair be attempted? 77th Annual Meeting of the American-Association-for-Thoracic-Surgery Reddy, V. M., McElhinney, D. B., Brook, M. M., Parry, A. J., Hanley, F. L. MOSBY-ELSEVIER. 1998: 1032–38

  Abstract

  Though repair of complete atrioventricular septal defect in infancy has become routine at most centers, it is not unusual for very young infants to be managed medically because of concerns about the fragility of the atrioventricular valve tissue.Since July 1992, seventy-two infants have undergone primary repair of complete atrioventricular septal defects at a median age of 3.9 months (40% < 3 months). A single-patch technique was used in all patients. The cleft was closed completely in 61 patients and partially (n = 10) or not at all (n = 1) in select patients at risk for valve stenosis. Left atrioventricular valve annuloplasty was performed in 18 patients. On the basis of transesophageal echocardiographic findings, 10 patients were returned to bypass for revision of the valve repair.There was one early death in a patient with single left papillary muscle, no early reoperations, and no new permanent arrhythmias. Only three patients had moderate left atrioventricular valve regurgitation at discharge. During a median follow-up of 24 months, there was one late death and five reoperations for left atrioventricular valve regurgitation (n = 2) and/or systemic outflow obstruction (n = 4). Follow-up left atrioventricular valve regurgitation was moderate in three patients, mild in 14, and none/trace in 54. Age had no relation to postoperative atrioventricular valve regurgitation, death, or reoperation.Despite concerns about fragility of valve tissue in very young patients, excellent results can be achieved with meticulous techniques. From neonates to older infants, age at repair does not influence outcome or valve function.

  View details for Web of Science ID 000073609200011

  View details for PubMedID 9605072

• Left pulmonary artery kinking caused by outflow tract dilatation after transannular patch repair of tetralogy of fallot ANNALS OF THORACIC SURGERY McElhinney, D. B., Parry, A. J., Reddy, V. M., Hanley, F. L., Stanger, P. 1998; 65 (4): 1120-1126

  Abstract

  Stenosis of the branch pulmonary arteries after tetralogy of Fallot repair can result from several mechanisms. In patients with free pulmonary regurgitation and right ventricular dilatation after transannular patch repair, we have observed that the pulmonary outflow tract can dilate and elongate craniad and rotate to the left, resulting in kinking and obstruction of the previously normal left pulmonary artery.Ten patients referred for reoperation after tetralogy repair with severe pulmonary regurgitation and right ventricular outflow tract dilatation were found to have left pulmonary artery kinking. In 8 of these patients kinking was the sole or partial cause of left pulmonary artery obstruction, whereas there was no documented obstruction in the other 2. All patients underwent right ventricular outflow tract reconstruction and left pulmonary artery repair with removal of the redundancy at the kink point. Patching alone is not an effective method of repair in this condition, because the mechanism of obstruction is not corrected and the patch material can become redundant and lead to recurrent obstruction.All patients underwent successful pulmonary outflow tract reconstruction. Left pulmonary artery kinking was corrected in all patients, and relief of obstruction was attained in the 8 patients with stenosis. At follow-up ranging from 9 to 58 months, no patient has evidence of recurrent left pulmonary artery stenosis.Left pulmonary artery kinking should be suspected at long-term follow-up after tetralogy repair in patients with significant pulmonary regurgitation and right-sided dilatation, even if previous evaluations showed no evidence of left pulmonary arterial abnormality. Because unilateral obstruction caused by kinking may lead to asymmetric pulmonary flow, it can exacerbate pulmonary regurgitation and right ventricular dilatation, in effect accelerating the processes that led to kinking in the first place. Kinking can be relieved successfully with the techniques described in this report.

  View details for Web of Science ID 000073160100049

  View details for PubMedID 9564939

• Trends in the management of truncal valve insufficiency 69th Annual Scientific Session of the American-Heart-Association McElhinney, D. B., Reddy, V. M., Rajasinghe, H. A., Mora, B. N., Silverman, N. H., Hanley, F. L. ELSEVIER SCIENCE INC. 1998: 517–24

  Abstract

  The single semilunar valve in patients with truncus arteriosus frequently is dysplastic and dysfunctional. Truncal valve insufficiency has been associated with poor outcome. Although the management of truncal valve insufficiency has evolved over the years, approaches to this problem vary considerably and remain a serious dilemma in many cases.We reviewed the records of 89 patients with unrepaired truncus arteriosus and mild (n = 37), moderate (n = 33), or severe (n = 19) truncal valve insufficiency who were admitted to our institution between 1975 and 1995. Eight patients (7 neonates) with moderate or severe insufficiency died before surgical intervention, and 4 patients underwent palliative pulmonary artery banding. The remaining 77 patients underwent repair. The median age at repair was 3.2 months (range, 2 days to 15 years; 83% infants), and it decreased from 4 months between 1975 and 1985 to 1 month between 1986 and 1995. Truncal valve replacement (mechanical = 6, allograft = 4) was performed in 10 patients, and 5 patients underwent valve repair.All 4 patients who underwent pulmonary artery banding died either early or late. The hospital (or 30-day) mortality rate after repair was 34% (26/77). At a median follow-up of 10 years, 11 hospital survivors had died, with overall 1- and 10-year actuarial survival rates of 56% and 48%, respectively, and poorer survival among patients with severe truncal valve insufficiency (p = 0.02). Late truncal valve replacement (n = 24) had been performed in 21 patients. Freedom from truncal valve replacement was better in patients with mild truncal insufficiency than in those with moderate or severe preoperative insufficiency (p < 0.001). Four late deaths were related directly to reoperation for truncal valve replacement or to prosthetic valve dysfunction. Three of the 4 neonates who received allograft root replacements died within 7 months of repair, and severe allograft valve insufficiency requiring replacement 1 year after operation developed in the fourth.The prospects for patients with truncal valve insufficiency have been improving over time. Nevertheless, the results in patients with severe insufficiency continue to be poor.

  View details for Web of Science ID 000072128100046

  View details for PubMedID 9485257

• Early and late results after repair of aortopulmonary septal defect and associated anomalies in infants < 6 months of age AMERICAN JOURNAL OF CARDIOLOGY McElhinney, D. B., Reddy, V. M., Tworetzky, W., Silverman, N. H., Hanley, F. L. 1998; 81 (2): 195-201

  Abstract

  The Richardson classification system for aortopulmonary septal defect (APSD) includes simple defects between the ascending aorta and pulmonary trunk (type I), defects extending distally to include the origin of the right main pulmonary artery (type II), and anomalous origin of the right main pulmonary artery from the ascending aorta with no other aortopulmonary communication (type III). These are rare lesions that must be repaired in early infancy to avoid development of pulmonary vascular disease. Few reports have focused on patients with complex, associated lesions who underwent repair in early infancy. Between 1972 and 1995, 24 patients with Richardson type I (n = 11), II (n = 7), or III (n = 6) defects underwent repair at ages ranging from 2 to 172 days (median 34). Twelve patients had complex, associated anomalies, including interrupted or hypoplastic arch (n = 9), tetralogy of Fallot with (n = 1) or without (n = 1) pulmonary atresia, and transposition of the great arteries (n = 1). The most recent 7 patients were diagnosed by echocardiography without cardiac catheterization. There were no early or late deaths among the 12 patients with simple APSD. Four patients with complex, associated lesions died in the early postoperative period and another died 4 months after surgery. All 6 surviving patients with interrupted arch have had recurrent obstruction at the arch repair site, although reintervention for this reason has been performed in only 2 patients. Altogether, 6 early survivors have required reintervention, and all survivors are in New York Heart Association class I at follow-up ranging from 2 to 25 years. Thus, long-term survival after repair of APSD in early infancy is excellent. Late sequelae are likely to be related either to associated lesions or to obstruction at the APSD repair site. Almost all cases of APSD in young infants can be diagnosed and evaluated by echocardiography without catheterization.

  View details for Web of Science ID 000071617500015

  View details for PubMedID 9591904

• Esophageal compression by the aorta after arterial switch ANNALS OF THORACIC SURGERY McElhinney, D. B., Reddy, V. M., Reddy, G. P., Higgins, C. B., Hanley, F. L. 1998; 65 (1): 246-248

  Abstract

  Extrinsic compression of the esophagus in children most often occurs in the presence of a congenital vascular ring. We recently operated on a patient in whom esophageal compression had developed that was severe enough to require feeding via a gastrostomy tube several years after the arterial switch operation. Aortopexy and extensive mediastinal mobilization were performed twice with transient relief and gradual return of symptoms. Almost 3 years after the first aortopexy, lasting relief was achieved by transposing the esophagus into the right side of the chest.

  View details for Web of Science ID 000071642300056

  View details for PubMedID 9456127

• Cytokine response to fetal cardiac bypass 66th Annual Meeting of the Society-for-Pediatric-Research Reddy, V. M., McElhinney, D. B., Rajasinghe, H. A., Rodriguez, J. L., Hanley, F. L. SPRINGER. 1998: 46–49

  View details for Web of Science ID 000072754700011

• Truncus arteriosus with patent ductus arteriosus and normal aortic arch ANNALS OF THORACIC SURGERY Mello, D. M., McElhinney, D. B., Parry, A. J., Silverman, N. H., Hanley, F. L. 1997; 64 (6): 1808-1810

  Abstract

  In hearts with a common arterial trunk (truncus arteriosus), there is almost always an inverse development of the aortic arch and the ductus arteriosus. Truncus with a normal aortic arch and a patent ductus is a rare echocardiographic and surgical finding. In this report, we describe 2 neonates in whom truncus arteriosus with a normal aortic arch and a medium or large patent ductus was diagnosed by preoperative echocardiography (without catheterization) and confirmed intraoperatively.

  View details for Web of Science ID 000071469400060

  View details for PubMedID 9436579

• Intraoperative discovery of neuroblastoma in an infant with pulmonary atresia ANNALS OF THORACIC SURGERY McElhinney, D. B., Reddy, V. M., Feuerstein, B. G., Marx, G. R., Hanley, F. L. 1997; 64 (6): 1827-1829

  Abstract

  There have been 28 previously reported cases of neuroblastoma associated with congenital heart disease. Because many of these have been defects of the conotruncal region, it has been proposed that abnormal neural crest cell migration or maturation may be a factor that links these normally disparate pathologic conditions. Most neuroblastomas in these cases have been detected at autopsy or by radiologic studies conducted in the evaluation of the cardiac anomalies. Recently, we discovered an occult posterior mediastinal neuroblastoma in a patient undergoing a unifocalization procedure for tetralogy of Fallot with pulmonary atresia and major aortopulmonary collaterals. The tumor was resected, and the patient has demonstrated no evidence of residual or metastatic neuroblastoma.

  View details for Web of Science ID 000071469400069

  View details for PubMedID 9436587

• Isolated left superior vena cava to the left atrium with situs solitus and dextrocardia: Extracardiac repair facilitated by juxtaposition of the atrial appendages AMERICAN JOURNAL OF CARDIOLOGY McElhinney, D. B., Mishaly, D. A., Moore, P., Brook, M. M., Reddy, V. M., Hanley, F. L. 1997; 80 (10): 1379-?

  Abstract

  We describe an extremely unusual case, with isolated left superior vena cava to the left atrium, normal intracardiac anatomy, and left juxtaposition of the atrial appendages. Surgical repair was performed without cardiopulmonary bypass by anastomosing the left superior cava to the superior aspect of the right atrial appendage, and was facilitated by the ectopic location of the appendage.

  View details for Web of Science ID A1997YG63500030

  View details for PubMedID 9388124

• Modified Damus-Kaye-Stansel procedure for single ventricle, subaortic stenosis, and arch obstruction in neonates and infants: Midterm results and techniques for avoiding circulatory arrest 77th Annual Meeting of the American-Association-for-Thoracic-Surgery McElhinney, D. B., Reddy, V. M., Silverman, N. H., Hanley, F. L. MOSBY-YEAR BOOK INC. 1997: 718–25

  Abstract

  A modified Damus-Kaye-Stansel procedure is one of several options for palliation of single ventricle with subaortic obstruction, but results in neonates have been disappointing. In the presence of arch obstruction, this procedure is typically performed with circulatory arrest, which may contribute to neurologic insult.Since 1990, a modified Damus-Kaye-Stansel procedure has been performed in 14 neonates and seven infants with single ventricle and subaortic stenosis, including 15 with arch obstruction. Diagnoses were double-inlet left ventricle (n = 12), tricuspid atresia (n = 2), and other forms of hypoplastic ventricle with subaortic obstruction (n = 7). Three patients underwent concurrent bidirectional Glenn shunt. In the most recent seven patients with arch obstruction, arch repair was achieved with an end-to-side anastomosis of the descending aorta to the ascending aorta with continuous upper body perfusion.One early death occurred among the 14 neonates (7%) and three among the infants, for an early mortality of 19%. At a median follow-up of 33 months, there were no late deaths or neurologic complications. Nine patients underwent subsequent bidirectional Glenn anastomosis, including three who had Fontan completion and one who later underwent conversion to a partial biventricular repair. One patient required a transplant for cardiomyopathy of unknown etiology. The remaining 12 patients are considered good candidates for Fontan completion. No patient has recurrent arch obstruction. Four patients have mild (n = 1) or trivial (n = 3) semilunar valvular regurgitation.The modified Damus-Kaye-Stansel procedure is an effective primary palliation for single ventricle and subaortic stenosis, with or without arch obstruction. Results are especially encouraging in neonates. Arch repair can be achieved without circulatory arrest to the brain.

  View details for Web of Science ID A1997YG20600004

  View details for PubMedID 9375601

• Pulmonary root translocation for biventricular repair of double-outlet left ventricle with absent subpulmonic conus JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY McElhinney, D. B., Reddy, V. M., Hanley, F. L. 1997; 114 (3): 501-503

  View details for Web of Science ID A1997XW44900031

  View details for PubMedID 9305210

• Systemic venous collateral channels causing desaturation after bidirectional cavopulmonary anastomosis: Evaluation and management JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY McElhinney, D. B., Reddy, V. M., Hanley, F. L., Moore, P. 1997; 30 (3): 817-824

  Abstract

  We sought to characterize the frequency, anatomic details and factors associated with the development of collateral channels between the superior and inferior vena caval systems after bidirectional cavopulmonary anastomosis.It is well known that systemic venous collateral channels often develop in patients who have undergone a classic Glenn shunt or bidirectional cavopulmonary anastomosis and that such collateral channels can lead to profound systemic desaturation. However, there have been few reports focusing on this problem.Fifty-four patients (median age 1.4 years) who underwent bidirectional cavopulmonary anastomosis and had preoperative and postoperative angiograms available for review were studied retrospectively. Postoperative connections between the superior and inferior vena caval systems were identified and measured. Sites of collateral origin and entry from the superior and inferior venous systems, as well as the course taken in between, were recorded.At follow-up angiography performed 17 days to 46 months postoperatively, a total of 31 venous collateral channels were observed in 18 patients with a wide variety of primary morphologic diagnoses. The majority of these collateral channels (80%) originated from the brachiocephalic vein or its junction with the superior vena cava, and over half of them drained below the diaphragm. In patients who developed venous collateral channels, the mean transpulmonary pressure gradient early after bidirectional cavopulmonary anastomosis was higher (p = 0.005), and mean arterial oxygen saturation at follow-up was lower (p = 0.009). There were trends toward higher superior vena caval pressure early after the operation and at follow-up in patients with collateral channels and a higher likelihood of absent upper lobe pulmonary blood flow in these patients. Successful coll embolization of 10 collateral channels was performed in six patients, with a median increase in arterial oxygen saturation of 16%.Angiographically detectable systemic venous collateral channels develop after bidirectional cavopulmonary anastomosis in a substantial number of patients (33% in the present series) with a variety of forms of a functional univentricular heart. Patients with venous collateral channels can be treated successfully with coil embolization, but the indications for embolization will depend on individual circumstances.

  View details for Web of Science ID A1997XT43600033

  View details for PubMedID 9283546

• The double switch procedure for anatomical repair of congenitally corrected transposition of the great arteries in infants and children XVIIIth Congress of the European-Society-of-Cardiology Reddy, V. M., McElhinney, D. B., Silverman, N. H., Hanley, F. L. W B SAUNDERS CO LTD. 1997: 1470–77

  Abstract

  To assess outcomes of anatomical repair (double switch procedure) in infants and children with congenitally corrected transposition of the great arteries.Between September 1993 and August 1996, 17 patients with congenitally corrected transposition underwent surgery at UCSF. Anatomical repair was performed in 11 of these patients, at ages ranging from 4.8 months to 7.8 years (median 3.2 years). The remaining six patients did not undergo anatomical repair due to unfavourable anatomy (n = 2), prior conduit repair (n = 2), biventricular dysfunction (n = 1), and isolated complete atrioventricular block (n = 1). The 11 patients who underwent anatomical repair make up the study group for the present report. All 11 patients had a malalignment ventricular septal defect, while pulmonary outflow tract obstruction was present in nine patients and significant tricuspid valve pathology or dysfunction was present in five. Anatomical repair was achieved with a Senning (n = 7) or a Mustard (n = 4) procedure combined with an arterial switch operation plus ventricular septal defect closure (n = 4), or a Rastelli procedure with left ventricle to aortic baffle and right ventricle to pulmonary artery conduit (n = 7). There was one early death and no patients developed surgical complete atrioventricular block. At a median follow-up of 22 months, there were no late deaths. Two patients required a total of three late reoperations, and all patients were asymptomatic on no cardiac medication. Follow-up echocardiography revealed normal biventricular function in all patients.Anatomical repair of corrected transposition can be achieved with low rates of early mortality and surgical heart block, and favourable mid-term results. Long-term follow-up will be necessary to determine if the double switch approach improves the natural history of corrected transposition when compared to less aggressive surgical approaches that leave the right ventricle in the systemic circulation.

  View details for Web of Science ID A1997XV98800020

  View details for PubMedID 9458454

• Hemodynamic effects of chronic prenatal ventricular pacing for the treatment of complete atrioventricular block CIRCULATION Liddicoat, J. R., Klein, J. R., Reddy, V. M., Klautz, R. J., Teitel, D. F., Hanley, F. L. 1997; 96 (3): 1025-1030

  Abstract

  Increasing the heart rate of the fetus with cardiac failure caused by complete AV block (CAVB) may allow delivery of a full-term, stable neonate with preserved ventricular function. Direct fetal pacing may be a feasible method to achieve this, but the effect of pacing on the structure and function of the rapidly developing fetal heart is unknown.CAVB was created in fetal lambs at 80% gestation by cryoablating the AV node. Epicardial ventricular pacing at 130 bpm was achieved by use of a pacemaker placed under the pectoral muscles. The fetus was returned to the uterus and allowed to continue to term. Ventricular function was assessed 1 week after birth in 7 lambs with CAVB and 10 control lambs. By use of the conductance catheter technique, the end-systolic pressure-volume relationship was determined at different heart rates, pacing conditions, and inotropic states. The contractility was not different between the two groups at their baseline heart rates and rhythms or when they were paced synchronously compared with asynchronously. Also, both groups responded significantly and similarly to inotropic manipulation, indicating preserved contractile reserve. Finally, in both groups, increased heart rates were associated with increased contractility, indicating an intact force-frequency relationship.We conclude that chronic epicardial ventricular pacing is well tolerated by the fetus, can be successfully applied as a treatment for CAVB, and does not adversely affect myocardial function in the rapidly developing, immature heart.

  View details for Web of Science ID A1997XP13000045

  View details for PubMedID 9264514

• Percutaneous fetal access and uterine closure for fetoscopic surgery - Lessons learned from 16 consecutive procedures in pregnant sheep SURGICAL ENDOSCOPY-ULTRASOUND AND INTERVENTIONAL TECHNIQUES Kohl, T., Szabo, Z., Suda, K., Quinn, T. M., Petrossian, E., Harrison, M. R., Hanley, F. L. 1997; 11 (8): 819-824

  Abstract

  Maternal morbidity and preterm labor from fetal surgery might be minimized by a percutaneous technique for fetal access and uterine closure.In each of 16 ewes, we inserted three trocars percutaneously into the amniotic cavity using ultrasound and fetoscopic guidance. In six ewes, percutaneous uterine closure after the procedure was attempted. We assessed feasibility and acute complications of our technique during surgery and at autopsy.We achieved percutaneous fetal access in 14 ewes and closed the uterus percutaneously in all six ewes attempted. Fetal injury was related to amnioinfusion or fixation of chorioamniotic membranes. Other complications were trocar dislodgment and damage to uterine wall and chorioamniotic membranes. The latter complication was prevented using balloon-tipped trocars.Percutaneous intraamniotic access and uterine closure for fetoscopic surgery can be achieved reliably with little maternal and fetal morbidity in sheep. Minor modifications are desired to apply this approach in humans.

  View details for Web of Science ID A1997XN75400008

  View details for PubMedID 9266643

• Bilateral branch pulmonary artery obstruction due to kinking at insertion sites of bilateral ductus arteriosus ANNALS OF THORACIC SURGERY McElhinney, D. B., Reddy, V. M., Moore, P., Hanley, F. L. 1997; 64 (2): 537-539

  Abstract

  Bilateral ductus arteriosus (or ligamentum arteriosum) with right aortic arch and isolation of the left subclavian artery is a rare anomaly of the aortic arch system. We report on a patient with complete atrioventricular septal defect, right aortic arch, bilateral ligamentum arteriosum, and isolation of the left subclavian artery in whom kinking at the ductal insertions caused bilateral branch pulmonary artery obstruction. Complete surgical repair was performed when the patient was 4 months of age, and the pulmonary artery obstructions were entirely relieved by ligation and division of both ducts without pulmonary arterioplasty.

  View details for Web of Science ID A1997XR28100063

  View details for PubMedID 9262610

• Repair of truncus arteriosus with intact ventricular septum (Van Praagh type B2) in a neonate JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY McElhinney, D. B., Reddy, V. M., Brook, M. M., Hanley, F. L. 1997; 114 (1): 134-138

  View details for Web of Science ID A1997XM02300021

  View details for PubMedID 9240306

• An institutional experience with the bidirectional cavopulmonary shunt: do we know enough about it? 22nd Annual Meeting of the Western-Thoracic-Surgical-Association Reddy, V. M., McElhinney, D. B., Moore, P., Bristow, J., Haas, G. S., Hanley, F. L. GREENWICH MEDICAL MEDIA LTD. 1997: 284–93

  View details for Web of Science ID A1997XV28900010

• Correction of left superior vena cava draining to the left atrium using extracardiac techniques ANNALS OF THORACIC SURGERY Reddy, V. M., McElhinney, D. B., Hanley, F. L. 1997; 63 (6): 1800-1802

  Abstract

  Intraatrial rerouting techniques have been the most common approaches to correcting left superior vena caval drainage to the left atrium in patients without atrial isomerism and with no connecting vein. Although these techniques have proved reliable and successful, there are cases in which extracardiac methods for managing this form of anomalous systemic drainage may be preferable. In the present report, we describe three extracardiac approaches to the correction of left superior vena cava draining to the left atrium.

  View details for Web of Science ID A1997XH23000067

  View details for PubMedID 9205198

• Bidirectional cavopulmonary shunt in patients with anomalies of systemic and pulmonary venous drainage ANNALS OF THORACIC SURGERY McElhinney, D. B., Reddy, V. M., Moore, P., Hanley, F. L. 1997; 63 (6): 1676-1684

  Abstract

  Bidirectional cavopulmonary shunt and Fontan repair are now commonly performed in patients with a variety of forms of complex single ventricle, including those with anomalies of systemic or pulmonary venous return. These anomalies are ideally dealt with during bidirectional cavopulmonary shunt, thereby minimizing the complexity of the eventual Fontan procedure.Between March 1990 and December 1995, 36 patients with anomalous systemic or pulmonary venous drainage underwent bidirectional cavopulmonary shunt. A combination of anomalous systemic and pulmonary venous drainage was present in 12 patients, whereas 19 patients had anomalous drainage only from the systemic circulation and 5 patients had isolated anomalies of pulmonary venous return. Visceral heterotaxy syndrome was diagnosed in 18 patients. The median age at operation was 11 months, and bidirectional cavopulmonary shunt was the first surgical procedure performed in 10 of these patients. Techniques of repair are described.There were two early deaths and one bidirectional cavopulmonary shunt was taken down, for mortality and failure rates not significantly different than those for all patients undergoing bidirectional cavopulmonary shunt during this time period (n = 117). At a mean follow-up of 19.9 months, there have been three late deaths and 11 patients have undergone Fontan completion. Actuarial survival was 87% at 1 year and 81% at 3 years. Among all patients undergoing bidirectional cavopulmonary shunt during this time period, neither heterotaxy syndrome nor anomalies of systemic or pulmonary venous return were significantly associated with decreased survival or poor outcome.Bidirectional cavopulmonary shunt can be performed in patients with anomalous systemic or pulmonary venous drainage, including those with visceral heterotaxy syndrome, with morbidity and mortality rates that do not differ significantly from those achieved in all patients undergoing bidirectional cavopulmonary shunt. In this report, we describe our experience with this group of patients, primarily focusing on outcomes and technical issues that pertain to the use of bidirectional cavopulmonary shunt as a preparatory procedure for the extracardiac conduit Fontan operation.

  View details for Web of Science ID A1997XH23000029

  View details for PubMedID 9205167

• One-stage complete unifocalization in infants: When should the ventricular septal defect be closed? 76th Annual Meeting of the American-Association-for-Thoracic-Surgery Reddy, V. M., Petrossian, E., McElhinney, D. B., Moore, P., Teitel, D. F., Hanley, F. L. MOSBY-YEAR BOOK INC. 1997: 858–66

  Abstract

  The decision whether to close the ventricular septal defect at the time of unifocalization in patients with pulmonary atresia, ventricular septal defect, and major aortopulmonary collaterals may be difficult. The purpose of this study was to develop morphologic and physiologic methods to aid in deciding whether to close the ventricular septal defect in patients undergoing one-stage unifocalization.Between July 1992 and April 1996, 27 infants with pulmonary atresia, ventricular septal defect, and aortopulmonary collaterals were treated at our institution. Midline complete unifocalization was performed in 25 patients-the ventricular septal defect was closed in 17 and left open in eight. Two patients with severe distal collateral stenoses underwent staged unifocalization. Pulmonary artery and collateral sizes were measured from preoperative angiograms and used to calculate the indexed cross-sectional area of the total neopulmonary artery bed. An intraoperative pulmonary flow study previously validated with experiments in neonatal lambs was performed in six patients: the unifocalized neopulmonary arteries were perfused with a known flow and pulmonary artery pressures were recorded.The neopulmonary artery index was greater in patients who underwent ventricular septal defect closure than in those who did not (p = 0.001), although the values did overlap. This index correlated with the postoperative right ventricular/left ventricular pressure ratio (p = 0.037). Mean pulmonary artery pressures obtained during the intraoperative flow study and after bypass were comparable.The total neopulmonary artery index correlates with postrepair right ventricular/left ventricular pressure ratio and is useful in deciding when to close the ventricular septal defect if it is larger than 200 mm2/m2. The pulmonary flow study is helpful in deciding whether to close the ventricular septal defect in all patients.

  View details for Web of Science ID A1997WZ62100006

  View details for PubMedID 9159619

• Outcomes after bidirectional cavopulmonary shunt in infants less than 6 months old 45th Annual Scientific Session of the American-College-of-Cardiology Reddy, V. M., McElhinney, D. B., Moore, P., Haas, G. S., Hanley, F. L. ELSEVIER SCIENCE INC. 1997: 1365–70

  Abstract

  We sought to assess the results after bidirectional cavopulmonary shunt (BCPS) in infants < 6 months old and to identify risk factors for poor outcome.Although BCPS is a well established procedure for the palliation of patients with a single-ventricle heart, there have been very few reports of outcomes after BCPS in young infants.Since 1990, 42 infants between 0.8 and 6.0 months of age (mean [+/-SD] 3.7 +/- 1.4) have undergone BCPS for primary (n = 16) or secondary (n = 26) palliation of tricuspid atresia (n = 13), hypoplastic left heart syndrome (n = 10) or other forms of functional single-ventricle heart (n = 19). Accessory pulmonary blood flow was included in 18 patients. Preoperative and perioperative data were gathered on retrospective review of patient records, and follow-up was conducted by means of direct physician contact or record review.The overall hospital mortality rate, including that associated with reoperations, was 4.8% (2 of 42 patients). Seven patients (17%) required reoperation related to the BCPS or pulmonary blood flow in the early postoperative period: Procedures included take-down of the BCPS in four patients, with one early death, and procedures to decrease pulmonary blood flow in three patients. Age < 1 month correlated significantly with early death and with early failure of the BCPS (death or take-down). Follow-up of the 37 patients discharged with intact BCPS was obtained at a mean +/-SD of 14.3 +/- 11.3 months postoperatively, during which time three patients died (at 6.5 +/- 2.5 months). The 2-year actuarial survival rate for patients undergoing BCPS at < 6 months of age was 86%. Overall freedom from death or take-down (including early and late events) was significantly lower in patients < 2 months old than in those > 2 months old. Four patients have undergone successful Fontan completion (18.3 +/- 2.9 months postoperatively), and one patient whose BCPS was taken down subsequently underwent successful restoration of a BCPS.Outcomes after BCPS in young infants are comparable to those in older infants and children. However, our current preference is to defer this procedure until after 2 months of age.

  View details for Web of Science ID A1997WW57700033

  View details for PubMedID 9137237

• Long-term follow-up of truncus arteriosus repaired in infancy: A twenty-year experience 76th Annual Meeting of the American-Association-for-Thoracic-Surgery Rajasinghe, H. A., McElhinney, D. B., Reddy, V. M., Mora, B. N., Hanley, F. L. MOSBY-YEAR BOOK INC. 1997: 869–78

  Abstract

  There have been few reports of long-term follow-up after truncus arteriosus repair in infancy.A retrospective review was performed to assess long-term outcomes among 165 patients who survived the initial hospital stay after complete repair of truncus arteriosus since 1975. The median age at truncus repair over this 20-year experience was 3.5 months (range 2 days to 36 years), and 81% of patients were less than 1 year of age. Previous pulmonary artery banding had been performed in 15 patients, and two patients had undergone prior repair of interrupted aortic arch. Significant procedures performed along with truncus repair included truncal valve replacement (n = 10) or repair (n = 5) and repair of interrupted aortic arch (n = 4).Patients were followed up for up to 20.4 years (median 10.5 years). Twenty-five patients were lost at cross-sectional follow-up, with a total of 67 patient-years of follow-up available on these patients. There have been 23 late deaths, eight of which occurred within 6 months of repair and 13 of which occurred within 1 year. Ten of the late deaths were related to reoperations. Actuarial survival among all hospital survivors was 90% at 5 years, 85% at 10 years, and 83% at 15 years and was essentially identical for infants alone. A significant independent risk factor for poorer long-term survival was truncus with moderate to severe truncal valve insufficiency before repair. During the follow-up period, 107 patients underwent 133 conduit reoperations. Median time to conduit reoperation was 5.5 years, and the only factor significantly associated with shorter time to conduit replacement was smaller conduit size at initial repair. In addition, 26 patients underwent 30 truncal valve replacements. Six patients required truncal valve replacement before any conduit-related reintervention, with two associated deaths. Actuarial freedom from truncal valve replacement among patients with no prerepair truncal valve insufficiency was 95% at 10 years. Actuarial freedom from truncal valve replacement was significantly lower among patients with truncal insufficiency before initial repair (63% at 10 years). At follow-up, all patients except three were in New York Heart Association functional class I.Ten- to 20-year survival and functional status are excellent among infants undergoing complete repair of truncus arteriosus. Conduit replacement or revision is almost inevitably necessary in this group of patients.

  View details for Web of Science ID A1997WZ62100008

  View details for PubMedID 9159620

• Measurement of collateral blood flow in a porcine model of aortic coarctation by velocity-encoded cine MRI JOURNAL OF MAGNETIC RESONANCE IMAGING Chernoff, D. M., Derugin, N., Rajasinghe, H. A., Hanley, F. L., Higgins, C. B., Gooding, C. A. 1997; 7 (3): 557-563

  Abstract

  The purpose of this study was to investigate the time course of development of collateral blood flow in an animal model of aortic coarctation. A juxtaductal aortic stenosis (model coarctation) was surgically created in five juvenile pigs. MRI was performed preoperatively, 1 to 2 days postoperatively, and 2 to 10 weeks postoperatively. Aortic blood flow was measured by velocity-encoded cine MR (VENC-MR). The percent change in aortic blood flow (delta BF) from proximal to distal descending thoracic aorta was calculated, and a multiple-comparison paired t test used to assess changes in delta BF over time. Invasive flow measurements were obtained in one animal before sacrifice using an ultrasonic probe. delta BF preoperatively was -2 +/- 8% (mean +/- SE). delta BF increased to 32 +/- 7% (mean +/- SE, P = .022) 2 days postoperatively and 55 +/- 19% (P = .032) 2 to 8 weeks postoperatively. Invasive measurements were in qualitative agreement with the VENC-MR data. VENC-MR is an accurate noninvasive method of measuring collateral blood flow in aortic coarctation. Recruitment and development of collateral flow pathways occur rapidly in an animal model.

  View details for Web of Science ID A1997XA92300017

  View details for PubMedID 9170042

• Total cavopulmonary direct anastomosis - Reply JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY Hanley, F. L., Reddy, V. M., VANSON, J. A. 1997; 113 (4): 808-809

  View details for Web of Science ID A1997WT81200028

• Biventricular repair of lesions with straddling tricuspid valves using techniques of cordal translocation and realignment CARDIOLOGY IN THE YOUNG Reddy, V. M., Liddicoat, J. R., McElhinney, D. B., Brook, M. M., VANSON, J. A., Hanley, F. L. 1997; 7 (2): 147-152

  View details for Web of Science ID A1997XP56200005

• Post-cardiopulmonary bypass pulmonary hypertension in lambs with increased pulmonary blood flow - A role for endothelin I CIRCULATION Reddy, V. M., HENDRICKSMUNOZ, K. D., Rajasinghe, H. A., Petrossian, E., Hanley, F. L., Fineman, J. R. 1997; 95 (4): 1054-1061

  Abstract

  After cardiopulmonary bypass (CPB), pulmonary hypertension and its associated increased vascular reactivity are a major source of morbidity, particularly for children with increased pulmonary blood flow. Although post-CPB pulmonary hypertension is well described, its mechanisms remain incompletely understood. Plasma levels of endothelin 1. a potent vasoactive substance implicated in pulmonary hypertension, are increased after CPB. The purpose of the present study was threefold: to characterize the changes in pulmonary vascular resistance and vascular reactivity induced by hypothermic CPB; to investigate the effects of preexisting increased pulmonary blood flow on these changes; and to better define the role of endothelin 1 in the pathogenesis of post-CPB pulmonary hypertension.Vascular pressures and blood flows were monitored in 14 1-month-old lambs with increased pulmonary blood flow (after in utero placement of an aortopulmonary shunt) and 6 age-matched control lambs. During the 2-hour study period after 105.3 +/- 20.6 minutes of hypothermic CPB the increase in pulmonary vascular resistance was significantly augmented in lambs with increased pulmonary blood flow compared with control lambs (P < .05). Pretreatment with PD 145065 (a nonselective endothelin receptor blocker; 50 micrograms.kg-1.min-1) completely blocked this increase in pulmonary vascular resistance and blocked the increase in pulmonary vascular resistance in response to acute alveolar hypoxia after CPB (96.3 +/- 88.5% versus -9.7 +/- 16.4%; P < .05). Plasma endothelin 1 levels increased after CPB in all lambs.Preexisting increased pulmonary blood flow alters the response of the pulmonary circulation to hypothermic CPB; the increase in pulmonary vascular resistance induced by CPB is augmented in lambs with increased pulmonary blood flow. Pretreatment with endothelin 1 receptor blockers eliminated the increase in pulmonary vascular resistance and the pulmonary vasoconstricting response to alveolar hypoxia, suggesting a role for endothelin 1 in post-CPB pulmonary hypertension. Endothelin 1 receptor blockers may decrease morbidity in children at risk for pulmonary hypertension after surgical repair with CPB and warrants further study.

  View details for Web of Science ID A1997WJ28200049

  View details for PubMedID 9054770

• Fetoscopic and open transumbilical fetal cardiac catheterization in sheep - Potential approaches for human fetal cardiac intervention CIRCULATION Kohl, T., Szabo, Z., Suda, K., Petrossian, E., KO, E. T., Kececioglu, D., Moore, P., Silverman, N. H., Harrison, M. R., Chou, T. M., Hanley, F. L. 1997; 95 (4): 1048-1053

  Abstract

  Shortening the prenatal disease course of severe aortic and pulmonary stenoses by balloon valvuloplasty may diminish their postnatal expression. The purpose of this study in fetal sheep was to assess the feasibility of fetoscopic and open transumbilical fetal cardiac catheterization guided by fetal transesophageal echocardiography to provide alternative approaches for human fetal cardiac intervention.We studied a total of nine fetal sheep (95 to 103 days of gestation; term = 145 to 150 days) and performed transumbilical fetal cardiac catheterization by a minimally invasive fetoscopic (n = 6) or an open fetal surgical approach (n = 3). Monitored by fetal transesophageal echocardiography, with an 8F or 10F, 10-MHz intravascular ultrasound catheter we placed guidewires and interventional catheters via the umbilical arterial route into the fetal heart. In three of the fetuses, we created supravalvar pulmonary artery stenosis by open fetal cardiac surgery After fetal and maternal recovery, we exteriorized these fetuses and performed open transumbilical fetal cardiac catheterization with successful pulmonary arterial angioplasty in two. Three fetuses survived fetoscopic transumbilical catheterization for 1 or 2 days and died most likely of blood loss after sheath dislodgment (n = 1) or removal (n = 2). By securing the sheath insertion site with a suture, we prevented sheath dislodgment and minimized bleeding during sheath removal in three fetuses. These fetuses then survived fetoscopic transumbilical fetal cardiac catheterization for 1 to 2 weeks before being killed.This study in fetal sheep demonstrates that fetoscopic and open transumbilical fetal cardiac catheterization are feasible and, guided by fetal transesophageal echocardiography, provide potential alternative approaches for human fetal cardiac intervention.

  View details for Web of Science ID A1997WJ28200048

  View details for PubMedID 9054769

• Hemodynamic effects of epinephrine, bicarbonate and calcium in the early postnatal period in a lamb model of single-ventricle physiology created in utero JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY Reddy, V. M., Liddicoat, J. R., McElhinney, D. B., Fineman, J. R., Klein, J. R., Chang, R., Hanley, F. L. 1996; 28 (7): 1877-1883

  Abstract

  A reproducible fetal animal model of single-ventricle physiology was created to examine the effects of pharmacologic agents commonly used in the perinatal and perioperative intensive care management of patients with a single ventricle.Single-ventricle physiology is characterized by parallel pulmonary and systemic circulations, with effective blood flow to each determined by the relative resistances in the pulmonary and systemic vascular beds. Perinatal and perioperative management of these patients is largely based on empiric observations and differs considerably between institutions and is further complicated by the transitional physiology of the newborn. The lack of animal models of single-ventricle physiology has hindered the understanding of this problem.A 10-mm, Damus-Kaye-Stansel-type aortopulmonary anastomosis was created in 10 fetal sheep at 140 +/- 1.2 days of gestation. The main pulmonary artery was ligated distally, and pulmonary blood flow (Qp) was provided through a 5-mm aortopulmonary shunt. Eight lambs were delivered at term and placed on cardiopulmonary bypass (30 min) 48 to 72 h after birth. Pharmacologic interventions (0.1 microgram/kg body weight per min of epinephrine, 2 mEq/kg of sodium bicarbonate and 10 mg/kg of calcium chloride) were performed before and after bypass, and hemodynamic responses were observed. The response to the epinephrine bolus was determined only in the postbypass study.Both before and after bypass, epinephrine infusion and calcium and bicarbonate administration increased Qp and systemic blood flow (Qs) (total cardiac output) but produced only small changes in the Qp/Qs ratio (-0.5% to -7.3% change). With the epinephrine bolus, Qp increased enormously, and the Qp/Qs ratio increased by 584% (p < 0.001).In neonatal lambs with single-ventricle physiology created in utero, epinephrine infusion and calcium and bicarbonate administration increased total cardiac output without significantly compromising the Qp/Qs ratio. However, epinephrine bolus seems to be hemodynamically detrimental in circumstances of single-ventricle physiology and should be used with caution and probably in relatively lower doses in the resuscitation of patients with single-ventricle physiology. Further investigation of the dose-dependent effects and the effects of prolonged administration of common pharmacologic agents will enable better management of patients with single-ventricle physiology.

  View details for Web of Science ID A1996VY32500033

  View details for PubMedID 8962579

• Revision of previous fontan connections to extracardiac or intraatrial conduit cavopulmonary anastomosis 32nd Annual Meeting of the Society-of-Thoracic-Surgeons McElhinney, D. B., Reddy, V. M., Moore, P., Hanley, F. L. ELSEVIER SCIENCE INC. 1996: 1276–82

  Abstract

  In patients who have received an atriopulmonary Fontan connection, complications such as right pulmonary vein obstruction, atrial arrhythmias, and thromboembolism are often secondary to right atrial enlargement. When such complications develop despite good ventricular function, there are few management options available. Extracardiac or intraatrial conduit cavopulmonary anastomosis, which improves central systemic venous flow patterns, avoids atrial distention, and does not involve the extensive atrial suturing required by other forms of cavopulmonary anastomosis, may provide relief for this group of patients.Between October 1992 and October 1995, 7 patients presented 8 to 20 years after atriopulmonary connection with severe right atrial dilatation (7), Fontan pathway obstruction (4), progressive congestive heart failure (4), atrial tachydysrhythmias (3), right atrial thrombus (1), obstruction of right pulmonary veins by an enlarged right atrium (1), and subaortic stenosis (1). After evaluation of the options, they underwent revision of the atriopulmonary connection to extracardiac (5) or intraatrial (2) conduit cavopulmonary anastomosis.One patient with severe cachexia, in whom transplantation was contraindicated for social reasons, died in the early postoperative period of massive effusions. Two patients eventually required permanent pacing for atrial dysrhythmias (1) or complete heart block secondary to subaortic fibromuscular resection (1), and 2 demonstrated marked improvement in unstable preoperative rhythm disturbances. At a median follow-up of 17 months, 4 of the 6 survivors were functioning at higher New York Heart Association levels than preoperatively, and 1 had recently undergone heart transplantation.In properly selected patients with atrial complications, revision of a prior Fontan connection to extracardiac or intraatrial conduit cavopulmonary anastomosis appears to be a viable option.

  View details for Web of Science ID A1996VQ16700007

  View details for PubMedID 8893557

• Homografts in congenital heart disease: Current applications and future directions 9th Annual Meeting of the Mediterranean-Association-of-Cardiology-and-Cardiac-Surgery McElhinney, D. B., Reddy, V. M., Hanley, F. L. ISRAEL JOURNAL MED SCIENCES. 1996: 880–85

  Abstract

  The first clinical use of homograft tissue in cardiovascular surgery was in 1948, when Gross used cadaveric arterial grafts to construct systemic to pulmonary artery shunts in patients with tetralogy of Fallot, and to repair coarctation of the aorta. Eighteen years later, a valved homograft was used for the first time in the treatment of congenital heart disease for reconstruction of the right ventricular outflow tract in a child with pulmonary atresia. Since these pioneering advances, valve and vascular homografts have become central to the management of congenital anomalies of the heart and great vessels. The primary use for homografts in congenital heart surgery today is establishment of a valved connection between the right ventricle and pulmonary arteries in children with tetralogy of Fallot with pulmonary atresia or other complicating factors, truncus arteriosus, transposition complexes, and double-outlet right ventricle, and in patients undergoing the Ross procedure. Homograft reconstruction of the left ventricular outflow tract has also been performed for many years in children with aortic insufficiency or recurrent aortic stenosis, but early homograft degeneration in young children has been a significant problem. Many surgeons are turning away from homografts in the systemic circulation in favor of the pulmonary autograft. Homograft is also widely used as a vascular patch material. In the present report, we discuss the various uses of homografts in congenital heart surgery, the benefits and drawbacks of homografts in young patients, and some of the future possibilities for homograft development and application in patients with congenital heart disease.

  View details for Web of Science ID A1996VU63200023

  View details for PubMedID 8950257

• The Ross procedure in children 9th Annual Meeting of the Mediterranean-Association-of-Cardiology-and-Cardiac-Surgery Reddy, V. M., McElhinney, D. B., Hanley, F. L. ISRAEL JOURNAL MED SCIENCES. 1996: 888–91

  Abstract

  Although it has been almost 30 years since Ross described his technique for aortic valve replacement using the pulmonary autograft, only recently has there been widespread interest in the use of the Ross procedure for aortic valve replacement in children with congenital heart disease. Since July 1992 we have performed the Ross procedure in 46 patients, 40 of whom were < or = 18 years of age at the time of surgery. In 10 children the procedure was combined with Konno ventriculoplasty, and in 3 others a Ross-Konno procedure was used for biventricular repair of borderline hypoplastic left heart syndrome. The only early deaths were in two of the patients with borderline hypoplastic left heart syndrome. There has been no late mortality, and two patients have required reoperation: one for recurrent distal aortic arch obstruction, and one for moderate-severe autograft insufficiency. Freedom from reoperation at 30 months is 89%. Aside from one patient with moderate autograft insufficiency and eight with mild regurgitation, all patients have trace or no aortic insufficiency at follow-up ranging from 1 to 38 months (median 22 months). The Ross procedure is an important advance in the treatment of aortic valve disease in pediatric patients. It is the only available replacement for the aortic valve that has growth potential, and there is ample evidence that the pulmonary autograft holds up well in the systemic circulation.

  View details for Web of Science ID A1996VU63200025

  View details for PubMedID 8950259

• Morphologic determinants favoring surgical aortic valvuloplasty versus pulmonary autograft aortic valve replacement in children 21st Annual Meeting of the Western-Thoracic-Surgical-Association VANSON, J. A., Reddy, V. M., Black, M. D., Rajasinghe, H., Haas, G. S., Hanley, F. L. MOSBY-YEAR BOOK INC. 1996: 1149–56

  Abstract

  The pulmonary autograft is being used with increasing frequency to replace the diseased aortic valve in the pediatric population. Attempted surgical aortic valvuloplasty with an unacceptable result and return to cardiopulmonary bypass for aortic valve replacement with a pulmonary autograft results in prolonged bypass time and increased potential for morbidity. Therefore, the ability to predict an unsuccessful outcome for valvuloplasty would be of significant clinical benefit. This issue is addressed in the present study. Methods: Twenty-two patients (median age 5.7 years, range 3 weeks to 14 years) with bicuspid (n = 11), tricuspid (n = 9), or quadricuspid (n = 2) aortic valves underwent valvuloplasty for aortic stenosis (n = 9), aortic regurgitation (n = 7), or a combination (n = 6). Previous related procedures included balloon aortic valvuloplasty (n = 3) and open surgical valvotomy (n = 1). Median pressure gradient across the aortic valve was 80 mm Hg. Surgical valvuloplasty techniques included thinning of leaflets (n = 18), commissurotomy (n = 15), suspension of reconstructed leaflet to the aortic wall (n = 10), closure of leaflet fenestration (n = 5), shortening of free edge of prolapsed cusp (n = 4), repair of torn leaflets (n = 3), and augmentation of scarred leaflets with autologous pericardium (n = 3). Concomitant subvalvular and supravalvular stenosis were repaired in nine and four patients, respectively. In five patients, during the same hospital stay, a failed valvuloplasty was converted into a valve replacement with a pulmonary autograft because of residual or resultant stenosis (n = 3) or regurgitation (n = 2). Results: No early or late deaths occurred. At a median follow-up of 16.3 months the median pressure gradient across the aortic valve in the 15 patients with preoperative stenosis or combined stenosis and regurgitation was 16 mm Hg (p < 0.01 versus preoperative gradient). Of the 22 patients, the aortic valve functioned normally (defined as < or = mild stenosis or regurgitation, or both) in 14 patients (including five patients with valve replacement); four patients had stenosis (gradients 40, 45, 60, and 60 mm Hg), two patients had regurgitation, and two patients had combined stenosis (gradients 40 and 50 mm Hg) and regurgitation. Three of the patients with recurrent stenosis underwent secondary surgical valvuloplasty without improvement. Outcome after valvuloplasty was examined according to valve structure: six of nine tricuspid valves functioned normally, whereas only three of 13 nontricuspid valves functioned normally (P = 0.07). Patients with a nontricuspid aortic valve and regurgitation had a high probability of requiring immediate valve replacement (P = 0.009). The actuarial freedom from significant native valve stenosis or regurgitation at 24 months was 82% for tricuspid valves and 36% for nontricuspid valves (P = 0.007). Conclusions: (1) Surgical aortic valvuloplasty should be the preferred approach when the aortic valve is tricuspid. (2) In contrast, aortic valve replacement with a pulmonary autograft should be the preferred strategy in the presence of a nontricuspid aortic valve (especially when the aortic valve is regurgitant) and after failed surgical valvuloplasty.

  View details for Web of Science ID A1996UQ38300007

  View details for PubMedID 8642815

• Use of autogenous aortic and maim pulmonary artery flaps for repair of anomalous origin of the right pulmonary artery from the ascending aorta JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY VANSON, J. A., Hanley, F. L. 1996; 111 (3): 675-676

  View details for Web of Science ID A1996UB98000033

  View details for PubMedID 8601986

• Extending the limits of the Ross procedure VII Biennial Meeting of the Society-of-Pediatric-Cardiovascular-Surgery Reddy, V. M., Rajasinghe, H. A., McElhinney, D. B., VANSON, J. A., Black, M. D., Silverman, N. H., Hanley, F. L. ELSEVIER SCIENCE INC. 1995: S600–S603

  Abstract

  The potential for growth and the proven long-term durability of the native pulmonary valve make it ideal for replacement of the diseased aortic valve, especially in growing children. The use of the autologous pulmonary valve can be further extended to patients with complex left ventricular outflow tract obstruction and to neonates and infants.Between June 1993 and May 1995, 35 patients underwent the Ross procedure at our center. Of these, 15 (43%) had complex left ventricular outflow tract obstruction and 7 (20%) were infants, including 3 neonates. The autologous pulmonary valve was implanted as a root replacement with coronary reimplantation in all patients. Additional left ventricular outflow tract procedures performed were ventricular myectomy in 7 patients and a Konno type aortoventriculoplasty in 11 patients.There was one early death in a patient with borderline hypoplastic left heart syndrome. At a median follow-up of 9 months (range, 0.2 to 22 months) there were no late deaths or reinterventions. The autologous pulmonary valve function was excellent, with 1 (2.8%) patient having moderate insufficiency.Autologous pulmonary valve is an excellent option for aortic valve replacement in all age groups. Its use can be readily extended to neonates, infants, and patients with complex left ventricular obstruction requiring additional left ventricular outflow tract procedures.

  View details for Web of Science ID A1995TW27300030

  View details for PubMedID 8604945

• Routine primary repair of tetralogy of Fallot in neonates and infants less than three months of age VII Biennial Meeting of the Society-of-Pediatric-Cardiovascular-Surgery Reddy, V. M., Liddicoat, J. R., McElhinney, D. B., Brook, M. M., Stanger, P., Hanley, F. L. ELSEVIER SCIENCE INC. 1995: S592–S596

  Abstract

  Although primary repair of tetralogy of Fallot is increasingly undertaken in infancy, complete repair is generally performed in only selected symptomatic neonates.From July 1992 through March 1995, 30 consecutive neonates and young infants with tetralogy of Fallot underwent routine primary repair. Group I (n = 10) consisted of patients with tetralogy of Fallot and pulmonary atresia (n = 5) or severe pulmonary stenosis (n = 5) who were duct dependent and were repaired in the neonatal period. Group II (n = 11) consisted of patients who were asymptomatic with arterial oxygen saturation between 75% and 90% (adequate pulmonary blood flow). Group III (n = 9) consisted of patients with "pink" tetralogy of Fallot (arterial oxygen saturation > 90%). Patients in groups II and III were electively scheduled for repair at about 2 months of age.The postrepair peak systolic right ventricular-to-peak systolic left ventricular pressure ratio did not correlate (p = 0.96) with the branch pulmonary artery size. One patient died 2 months after operation, despite good hemodynamics, of uncontrollable diffuse subcutaneous edema due to familial distichiasis lymphedema syndrome. There were no late deaths. At a median follow-up of 19 months, 1 patient underwent balloon dilation of branch pulmonary arteries. Follow-up echocardiography revealed a 30 to 60 mm Hg right ventricle-to-pulmonary artery gradient in 3 patients.Excellent early and midterm results can be accomplished with routine primary repair of tetralogy of Fallot in early infancy regardless of age, symptomatic status, coronary anatomy, and the size of branch pulmonary arteries as long as they arborize normally.

  View details for Web of Science ID A1995TW27300028

  View details for PubMedID 8604943

• Performance of right ventricle to pulmonary artery conduits after repair of truncus arteriosus: a comparison of Dacron-housed porcine valves and cryopreserved allografts. Seminars in thoracic and cardiovascular surgery Reddy, V. M., Rajasinghe, H. A., McElhinney, D. B., Hanley, F. L. 1995; 7 (3): 133-138

  Abstract

  Repair of complex congenital heart defects often requires the use of extracardiac conduits. These repairs can be technically accomplished with excellent early results. However, the long-term performance of various conduits is less than optimal. In this report, we examined the long-term outcome of xenograft valved Dacron conduits and cryopreserved allograft valved conduits used for reconstruction of the right ventricular outflow tract in young patients with truncus arteriosus. A retrospective review was performed on 222 patients who underwent primary surgical repair of truncus arteriosus between January 1975 and December 1994 using a xenograft valved synthetic conduit (group I; n = 175) or with a cryopreserved valved allograft conduit (group II; n = 47). Median age at repair was 121 days in group I and 70 days in group II; median weight of patients in both groups was the same (4.2 kg). Conduit-related early deaths occurred in 5.7% (10/175) of patients in group I and none in group II. In a cohort of age-matched patients, actuarial freedom from conduit-related reintervention at 5 years follow-up was significantly better (P = .037) for the cryopreserved valved allograft conduits when compared with xenograft valved synthetic conduits. However, this difference was not apparent by 7 years. Multivariate analysis showed conduit size (P = .0005) as a significant predictor of early conduit-related reintervention. This study shows that mid-term performance of cryopreserved allografts (which have the advantage of technical ease of insertion of availability of small sizes for use in neonates), is better than the xenograft Dacron conduits.(ABSTRACT TRUNCATED AT 250 WORDS)

  View details for PubMedID 7548319

• MIDLINE ONE-STAGE COMPLETE UNIFOCALIZATION AND REPAIR OF PULMONARY ATRESIA WITH VENTRICULAR SEPTAL-DEFECT AND MAJOR AORTOPULMONARY COLLATERALS 74th Annual Meeting of the American-Association-for-Thoracic-Surgery Reddy, V. M., Liddicoat, J. R., Hanley, F. L. MOSBY-YEAR BOOK INC. 1995: 832–45

  Abstract

  Traditionally patients with pulmonary atresia, ventricular septal defect, diminutive or absent central pulmonary arteries, and multiple aortopulmonary collaterals have been managed by staged procedures necessitating multiple operations. We have taken a different approach to this lesion. Between August 1992 and March 1994, ten patients aged 1.43 months to 37.34 years (median 2.08 years) at the severe end of the morphologic spectrum of this lesion underwent a one-stage complete unifocalization and repair from a midline sternotomy approach. The median Nakata index of true pulmonary arteries was 50.0 (range 0 to 103.13) and they provided vascular supply to up to nine lung segments (median 5 segments). The number of collaterals per patient ranged from two to five with a median of four. The collaterals provided vascular supply to a median of 15 lung segments per patient (range 11 to 20). Complete unifocalization was achieved in all patients with emphasis on native tissue-to-tissue connections via anastomosis of collaterals to other collaterals and to the native pulmonary arteries. In only one patient (37.34 years old) was it necessary to use a non-native conduit for peripheral pulmonary artery reconstruction. The ventricular septal defect was left open in one patient (5 years old) because of diffuse distal hypoplasia and stenosis of the pulmonary arteries and the collaterals. The postrepair peak systolic right ventricular/left ventricular pressure ratio ranged from 0.31 to 0.58 (median 0.47). There were no early deaths. Complications were bleeding necessitating reexploration in one patient, phrenic nerve palsy in three patients, and severe bronchospasm in three patients. Follow-up (median 8 months, range 2 to 19 months) was complete in all patients. One patient was reoperated on for pseudoaneurysm of the central homograft conduit and then again for stenosis of the left lower lobe collateral. After this last operation at 13 months after the initial repair she died of a preventable cardiac arrest caused by pneumothorax. The patient with open ventricular septal defect underwent balloon dilation of the unifocalized pulmonary arteries, with a current pulmonary/systemic flow ratio of 1.4 to 1.8:1, and is awaiting ventricular septal defect closure. One other patient underwent balloon dilation of the reconstructed right pulmonary artery, with a good result. All survivors (9/10) are clinically doing well. This approach establishes normal cardiovascular physiology early in life, eliminates the need for multiple systemic-pulmonary artery shunts and use of prosthetic material, and minimizes the number of operations required.(ABSTRACT TRUNCATED AT 400 WORDS)

  View details for Web of Science ID A1995QX36800002

  View details for PubMedID 7739241

• FACTORS INFLUENCING EARLY AND LATE OUTCOME OF THE ARTERIAL SWITCH OPERATION FOR TRANSPOSITION OF THE GREAT-ARTERIES 74th Annual Meeting of the American-Association-for-Thoracic-Surgery Wernovsky, G., Mayer, J. E., Jonas, R. A., Hanley, F. L., Blackstone, E. H., Kirklin, J. W., Castaneda, A. R. MOSBY-ELSEVIER. 1995: 289–302

  Abstract

  Between January 1983 and January 1992, 470 patients underwent an arterial switch operation at our institution. An intact (or virtually intact) ventricular septum was present in 278 of 470 (59%); a ventricular septal defect was closed in the remaining 192. Survivals at 1 month and 1, 5, and 8 years among the 470 patients were 93%, 92%, 91%, and 91%, respectively. The hazard function for death (at any time) had a rapidly declining single phase that approached zero by one year after the operation. Risk factors for death included coronary artery patterns with a retropulmonary course of the left coronary artery (two types) and a pattern in which the right coronary artery and left anterior descending arose from the anterior sinus with a posterior course of the circumflex coronary. The only procedural risk factor identified was augmentation of the aortic arch; longer duration of circulatory arrest was also a risk factor for death. Earlier date of operation was a risk factor for death, but only in the case of the senior surgeon. Reinterventions were performed to relieve right ventricular and/or pulmonary artery stenoses alone in 28 patients. The hazard function for reintervention for pulmonary artery or valve stenosis revealed an early phase that peaked at 9 months after the operation and a constant phase for the duration of follow-up. Incremental risk factors for the early phase included multiple ventricular septal defects, the rapid two-stage arterial switch, and a coronary pattern with a single ostium supplying the right coronary and left anterior descending, with a retropulmonary course of the circumflex. The need for reintervention has decreased with time. The arterial switch operation can currently be performed early in life with a low mortality risk (< 5%) and a low incidence of reintervention (< 10%) for supravalvular pulmonary stenosis. The analyses indicate that both the mortality and reintervention risks are lower in patients with less complex anatomy.

  View details for Web of Science ID A1995QG08100013

  View details for PubMedID 7853882

• FETAL HEART-BLOCK - A NEW EXPERIMENTAL-MODEL TO ASSESS FETAL PACING PACE-PACING AND CLINICAL ELECTROPHYSIOLOGY Assad, R. S., Jatene, M. B., Moreira, L. F., SALES, P. C., Costa, R., Hanley, F. L., Jatene, A. D. 1994; 17 (7): 1256-1263

  Abstract

  Epicardial fetal pacing via thoracotomy has the potential of being a safer and more reliable procedure to treat congenital complete heart block (CHB) associated with fetal hydrops refractory to medical therapy. To assess the acute electrophysiological characteristics of two ventricular epicardial leads, a new experimental model of fetal heart block induced by cryosurgical ablation of the AV node without the need for fetal cardiac bypass was performed in 12 pregnant ewes at 110-115 days gestation. A modified screw-in lead (1 1/2 turns) was used in six fetal lambs and a stitch-on lead in the other six lambs. CHB was achieved in 100% of the fetal lambs, with no ventricular escape rate noticed in any of the lambs. The acute stimulation thresholds were consistently low for both leads, with lower values for the screw-in lead at pulse duration below 0.9 msec (P < 0.03). Current measured at voltage threshold with pulse width below 0.5 msec was lower for the screw-in lead (P < 0.048). Stimulation resistance, measured during constant-voltage pacing, was not statistically different between the two leads (441.8 +/- 13.7 omega for the screw-in lead vs 480.2 +/- 59.2 omega for the stitch-on lead). No significant differences (P > 0.20) were found in R wave amplitude between the two electrodes. Slew rates were significantly higher in the screw-in group than in the stitch-on group (1.40 +/- 0.2 vs 0.62 +/- 0.2 V/sec, P = 0.04). This model of CHB is a simple and reproducible method to assess fetal pacing. We find the screw-in electrode to be a better option when fetal pacing is indicated.

  View details for Web of Science ID A1994NX28800007

  View details for PubMedID 7937231

• LONG-TERM SURVIVORS OF FETAL CARDIAC BYPASS IN LAMBS JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY Fenton, K. N., ZINN, H. E., Heinemann, M. K., Liddicoat, J. R., Hanley, F. L. 1994; 107 (6): 1423-1427

  Abstract

  The initial experience with cardiac bypass in fetal lambs resulted in early fetal death from placental insufficiency. Subsequent work in our laboratory indicated that vasoactive cyclooxygenase products were released as mediators of this response. The placental dysfunction could be blocked by the administration of indomethacin, allowing longer fetal survival. This unmasked a more subacute (but fatal) problem: fetal surgical stress resulted in diminished fetal cardiac output and progressive metabolic acidosis and contributed to the placental vasoconstriction. In acute studies, when indomethacin was given and the stress response was inhibited by the use of total spinal anesthesia, the fetus maintained normal blood gas levels, cardiac output, placental blood flow, and acid-base status for several hours after bypass. We hypothesized that beyond this point, no further fetal or placental compromise would occur and that this management technique would thus allow long-term fetal survival. With the use of total spinal anesthesia and sterile technique for long-term study, 12 fetal lambs at 120 days (80%) gestation underwent exposure, line placement, and cannulation for fetal cardiac bypass. Indomethacin was given intravenously on obtaining venous access. After 20 minutes of normothermic cardiac bypass at flow rates of 250 to 300 ml/kg/min, the fetus was weaned from bypass, the cannulas and lines were removed, the uterus and abdomen were closed, and the ewe and fetus were allowed to recover. There was one maternal death (pneumonia) and one early abortion (of twins); the remaining 10 ewes progressed to term. At term, five healthy lambs that had undergone fetal cardiac bypass were delivered (including one twin), four ewes delivered a mummified study fetus and one or two healthy siblings, and one delivered a dead term fetus. With the use of techniques that inhibit fetal stress and block placental vasoconstriction, cardiac bypass can be performed in single-gestation fetal lambs with a high degree of recovery and survival (80% in this study). The cause of the elevated abortion rate associated with twin gestation is unclear.

  View details for Web of Science ID A1994NQ76900007

  View details for PubMedID 8196383

• INHIBITION OF THE FETAL STRESS-RESPONSE IMPROVES CARDIAC-OUTPUT AND GAS-EXCHANGE AFTER FETAL CARDIAC BYPASS JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY Fenton, K. N., Heinemann, M. K., Hickey, P. R., Klautz, R. J., Liddicoat, J. R., Hanley, F. L. 1994; 107 (6): 1416-1422

  Abstract

  Cardiac bypass in late-gestation fetal lambs causes severe placental vasoconstriction, which leads to fetal death from hypoxemia and respiratory acidosis. This response can be blocked by the administration of indomethacin; however, a fatal metabolic acidosis then gradually develops in the fetus. Because the fetus is known to mount an intensive catecholamine response to stress, and because the fetal myocardium is particularly sensitive to increased afterload, we hypothesized that elevated afterload as a result of fetal stress contributes to diminished cardiac output after bypass. Twenty fetal lambs at 80% gestation underwent 30 minutes of normothermic cardiac bypass at flow rates of 200 to 500 ml/kg per minute. All ewes received general anesthesia with ketamine. In 10 fetuses general anesthesia was specifically designed not to inhibit the release of stress-related catechols (ketamine); the remaining 10 fetuses received a "high" (cisterna magna) total spinal anesthetic with tetracaine, to block the fetal stress response. In each anesthetic group, 5 of the 10 lambs received indomethacin. During operation, normal hemodynamics were preserved in the spinal anesthetic group. Cardiac output, placental blood flow, and arterial carbon dioxide tension were all improved relative to results in the ketamine group. When spinal anesthesia and indomethacin are both given, hemodynamics also approach normal after bypass, and gas exchange is further improved. These data suggest that the inhibition of the stress response by spinal anesthesia improves the hemodynamic status of the fetus during operation and, in combination with indomethacin, allows maintenance of near-normal placental function after fetal cardiac bypass. Similar responses may also be possible in human fetuses with use of a high-dose narcotic technique.

  View details for Web of Science ID A1994NQ76900006

  View details for PubMedID 8196382

• MANAGEMENT AND OUTCOME OF LOW-BIRTH-WEIGHT NEONATES WITH CONGENITAL HEART-DISEASE JOURNAL OF PEDIATRICS Chang, A. C., Hanley, F. L., Lock, J. E., Castaneda, A. R., Wessel, D. L. 1994; 124 (3): 461-466

  Abstract

  In 100 consecutive neonates with birth weights < or = 2500 gm (range, 540 to 2500 gm; median, 2200 gm), major congenital heart disease (excluding patent ductus arteriosus, isolated atrial septal defect, and ventricular septal defect) was diagnosed between January 1987 and January 1991; 46 had ductus-dependent lesions. Of the 100 neonates, 30 had genetic aberrations or significant associated congenital anomalies. The four most common cardiac diagnoses were tetralogy of Fallot with or without pulmonary atresia (n = 16); coarctation of the aorta (n = 12); transposition of the great arteries (n = 11); and common atrioventricular canal (n = 11). The hospital survival rate for the entire group of 100 neonates was 70%. The patients were separated into three groups on the basis of the time of intervention. Group 1 (early intervention) included 62 infants. These neonates (including 31 with ductus-dependent lesions) had surgical or catheter intervention during the initial hospitalization (median age, 9 days), all at weights < or = 2500 gm. The hospital survival rate was 81% (50/62); survival rates for palliation (78%, 18/23) and for correction (82%, 32/39) were similar. There were 26 neonates in group 2 (late intervention). These neonates did not have surgical intervention during the initial hospitalization. All were managed medically; survivors were discharged and had surgical procedures later (at a median age of 4.3 months). Six neonates (23%) died during medical management; all 20 survivors returned and had surgical procedures, with 90% survival. Overall survival rate for this group was 69% (18/26). The remaining 12 patients (group 3) had complicating features that precluded intervention; none survived. On the basis of these results, we conclude that early intervention, even with corrective surgery, can be performed in low birth weight neonates with an acceptable mortality rate. Prolonged medical therapy to achieve further weight gain did not appear to improve the survival rate.

  View details for Web of Science ID A1994NA03500022

  View details for PubMedID 8120722

• TOTAL ANOMALOUS PULMONARY VENOUS DRAINAGE IN NEWBORNS WITH VISCERAL HETEROTAXY 29TH Annual Meeting of the Society-of-Thoracic-Surgeons Heinemann, M. K., Hanley, F. L., VANPRAAGH, S., Fenton, K. N., Jonas, R. A., Mayer, J. E., Castaneda, A. R. ELSEVIER SCIENCE INC. 1994: 88–91

  Abstract

  Children with visceral heterotaxy often present with total anomalous pulmonary venous drainage (TAPVD) associated with univentricular congenital heart disease. We reviewed our experience with the primary surgical management of this lesion under these circumstances. Over a recent 10-year span, 38 patients within the first 3 days of life were admitted to our institution and underwent primary palliation. Twenty-one of them had TAPVD, 18 to a systemic vein. Twelve (67%) of these 18 were seen with obstruction of the anomalous connection and underwent emergency operation. In 7 patients, repair of TAPVD was combined with a systemic-pulmonary artery shunt because of additional obstruction of the pulmonary blood supply, with two deaths. One patient had primary shunting and then repair of TAPVD. Four patients underwent repair of TAPVD alone. Two of them then showed signs of insufficient pulmonary blood flow, received a shunt in a second procedure, and subsequently died. Early mortality in the group with obstructed TAPVD was thus 4 (33%) of 12 patients. Statistical analysis of all 38 patients (univariate analysis, chi 2 testing) showed that neither the presence of TAPVD (p = 0.7) nor TAPVD repair alone (p = 0.8) or with shunting (p = 0.8) was a definite risk factor for early death. The performance of a shunt during the first operation, however, was associated with lower early mortality (p = 0.03). Total anomalous pulmonary venous drainage is a common finding in newborns with visceral heterotaxy. Its presence and its subsequent early repair (requiring cardiopulmonary bypass) do not increase the mortality risk. The need of a concomitant shunt in obstructed TAPVD can initially be underestimated.

  View details for Web of Science ID A1994MR31800016

  View details for PubMedID 8279925

• HIGH-DOSE STEROIDS PREVENT PLACENTAL DYSFUNCTION AFTER FETAL CARDIAC BYPASS JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY Sabik, J. F., Heinemann, M. K., Assad, R. S., Hanley, F. L., Russo, P. 1994; 107 (1): 116-125

  Abstract

  Surgical treatment of certain congenital heart lesions in utero may have a therapeutic advantage over postnatal repair or palliation. For fetal heart surgery to be possible, a method to support the fetal circulation is necessary. Early experimental attempts at fetal cardiac bypass were unsuccessful because of increased placental vascular resistance during and after fetal cardiac bypass, which led to decreased placental flow, fetal asphyxia, and death. Our laboratory has demonstrated that the administration of indomethacin (a cyclooxygenase inhibitor) during fetal cardiac bypass prevents this increase in placental vascular resistance during and after fetal cardiac bypass. The specific mechanism by which indomethacin achieves this effect is likely to be either by inhibiting the production of a placental vasoconstrictive prostaglandin or by diverting substrate from the cyclooxygenase pathway to the lipoxygenase pathway, thereby potentially increasing the production of a placental vasodilating leukotriene. To examine these potential mechanisms in more detail, we inhibited both prostaglandin and leukotriene synthesis at the phospholipase stage with high-dose steroids. Fourteen fetal lambs were used in the study. Six animals received indomethacin (3 mg/kg), four received high-dose steroids (Solu-Medrol 50 mg/kg), and four animals were used as controls. Observations were made during a 1-hour prebypass period, a 30-minute bypass period, and a 2-hour postbypass period. Placental blood flow and placental vascular resistance were calculated at four times during the experiments: before sternotomy; after sternotomy; during bypass at 30 minutes; and 30 minutes after cessation of bypass. Similar to indomethacin, high-dose steroid administration during fetal cardiac bypass prevents the rise in placental vascular resistance and preserves placental blood flow during and after fetal cardiac bypass. This study suggests that the production of a placental vasoconstrictive prostaglandin is responsible for the increase in placental vascular resistance and decrease in placental blood flow observed after fetal cardiac bypass.

  View details for Web of Science ID A1994MR27500017

  View details for PubMedID 8283873

• Intraoperative device closure of ventricular septal defects. Circulation Fishberger, S. B., Bridges, N. D., Keane, J. F., Hanley, F. L., Jonas, R. A., Mayer, J. E., Castaneda, A. R., Lock, J. E. 1993; 88 (5): II205-9

  Abstract

  Surgical repair of muscular ventricular septal defects (VSDs) has been associated with significant morbidity and mortality when the defects are multiple, associated with complex cardiac lesions, or requiring left ventriculotomy. Transcatheter VSD closure may be difficult in patients weighing less than 7 kg or those with limited vascular access. We attempted intraoperative device closure of muscular VSDs during surgical repair of associated lesions.We considered intraoperative umbrella closure in 10 patients, 8 with additional complex heart lesions. "Swiss cheese" defects were present in 4. Median patient age was 4.7 months. Device closure was not attempted in 1 patient due to an inadequate septal rim. Nine umbrellas, 12 to 40 mm in diameter, were positioned to straddle the septum in all 9 patients. There were 3 early deaths, 2 in patients who were moribund preoperatively. The third was due to severe ventricular dysfunction. Among the 6 survivors, 3 had a Qp:Qs < or = 2 and right ventricular or pulmonary artery pressure less than half systemic. These patients had no further intervention. The other 3 had a Qp:Qs > 2. One died unexpectedly 5 weeks after cardiac surgery. The other 2 had subsequent closure of residual VSDs. All 5 survivors are well at follow-up of 8 to 25 months.Intraoperative umbrella VSD closure appears less successful than VSD closure in the catheterization laboratory. In selected patients, particularly very small infants, intraoperative device placement may be advantageous. Completeness of closure is difficult to assess intraoperatively.

  View details for PubMedID 8222155

• Early bidirectional cavopulmonary shunt in young infants. Postoperative course and early results. Circulation Chang, A. C., Hanley, F. L., Wernovsky, G., Rosenfeld, H. M., Wessel, D. L., Jonas, R. A., Mayer, J. E., Lock, J. E., Castaneda, A. R. 1993; 88 (5): II149-58

  Abstract

  Despite the recent wide applicability of the bidirectional cavopulmonary shunt, there is limited reported experience in performing these shunts in infants 6 months or younger.Before October 1992, 17 consecutive infants aged 4.2 to 6.5 months (median, 6.1 months) underwent bidirectional cavopulmonary shunts. The diagnoses were hypoplastic left heart syndrome (n = 7), single right ventricle (n = 5), and single left ventricle (n = 5). All but 2 patients had prior palliative surgery. The bidirectional cavopulmonary shunt was performed early on an elective basis in 9 patients; the remaining patients had progressive cyanosis (6 patients), severe ventricular failure (1 patient), and coexisting restrictive bulboventricular foramen (1 patient). The median preoperative pulmonary arterial pressure and pulmonary vascular resistance were 15 mm Hg and 2.3 U.m2, respectively. One patient died; the overall hospital survival was 94%. The most common postoperative problem was transient systemic hypertension, observed in 14 (88%) of 16 survivors. Systemic arterial oxygen saturation increased from a median of 75% before surgery to a median of 85% after surgery (P < .05). The median hospital stay was 6 days. There were no late deaths during follow-up (median, 12.4 months). At postoperative cardiac catheterization performed in 9 of 16 survivors, there was no evidence of severe hypoxemia, shunt narrowing, or pulmonary arteriovenous fistulas. Of the 16 survivors, 6 have had a subsequent Fontan operation at a median age of 1.9 years; there were 5 survivors.Early bidirectional cavopulmonary shunt in young infants has shown encouraging early results and provides improved oxygenation with low morbidity and mortality. We speculate that an early bidirectional cavopulmonary shunt on an elective basis may reduce the deleterious sequelae of chronic hypoxemia, long-term ventricular volume overload, and repeated palliative procedures, thus yielding a more suitable Fontan candidate.

  View details for PubMedID 7693365

• OUTCOMES IN CRITICALLY ILL NEONATES WITH PULMONARY STENOSIS AND INTACT VENTRICULAR SEPTUM - A MULTIINSTITUTIONAL STUDY JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY Hanley, F. L., Sade, R. M., Freedom, R. M., Blackstone, E. H., Kirklin, J. W. 1993; 22 (1): 183-192

  Abstract

  This study attempted to determine the optimal therapeutic interventions by risk-adjusted comparisons of early and intermediate-term outcomes.The variety of interventions and the small case load at individual institutions have delayed the generation of reliable information concerning therapy for this condition.In this prospective 27-institution study, 101 neonates were consecutively enrolled (between January 1, 1987 and January 1, 1991). Treatment was determined by the physicians. Demographic and morphologic details were tabulated. Dimensions of the pulmonary "anulus" and tricuspid anulus were measured on echocardiograms, and right ventricular cavity size was estimated. Right ventricular-pulmonary trunk pressure gradients were tabulated. Numerous analyses were made.Severe pulmonary valve stenosis and an intact ventricular septum were present in all patients. The right ventricular-pulmonary trunk junction ("anulus") was severely narrowed in 15%. Right ventricular cavity size was severely reduced in 4%. The tricuspid valve was small in 15% of patients; its diameter was poorly correlated with right ventricular cavity size. Eighty-nine percent and 81% of patients survived > or = 1 month and 4 years, respectively, after the initial procedure. Multivariable analysis identified no patient-specific risk factors for death. Only open pulmonary valvotomy without a support technique was uniformly a procedural risk factor; under some circumstances, transannular patching without a shunt was a risk factor. The right ventricular-pulmonary trunk gradient immediately after valvotomy was < 30 mm Hg in 81% of patients and was similar after surgical and balloon valvotomy. In 74% of patients, no intervention was required after the first accomplished intervention.Marked variation in morphology is uncommon in critical pulmonary stenosis in neonates. Percutaneous balloon valvotomy and certain types of surgical valvotomy are optimal initial procedures. The unusual situation of a small pulmonary "anulus" may initially require a transannular patch and a systemic-pulmonary artery shunt.

  View details for Web of Science ID A1993LK56600025

  View details for PubMedID 8509540

• REPAIR OF TRUNCUS ARTERIOSUS IN THE NEONATE 72ND ANNUAL MEETING OF THE AMERICAN ASSOC FOR THORACIC SURGERY Hanley, F. L., Heinemann, M. K., Jonas, R. A., Mayer, J. E., Cook, N. R., Wessel, D. L., Castaneda, A. R. MOSBY-YEAR BOOK INC. 1993: 1047–56

  Abstract

  From September 1986 through December 1991, 63 patients with truncus arteriosus underwent surgical repair. The management approach evolved over the period of the study from elective primary repair at 3 months of age to elective primary repair in the early neonatal period. Thirty variables were examined as potential risk factors for the outcome events of death, reoperation, and presence of pulmonary vascular morbidity. According to both univariate and multivariate techniques, severe truncal valve regurgitation, interrupted aortic arch, coronary artery anomalies, and age at repair greater than 100 days were important risk factors for perioperative death. In the 33 patients without these risk factors, early survival was 100%. In the 30 patients with one or more of these risk factors, survival was 63%. Pulmonary hypertensive episodes were fewer, and duration of ventilator dependence and pulmonary artery pressure were significantly less in patients undergoing the operation before 30 days of age. Seven patients required reoperation for right ventricular outflow tract obstruction at a mean follow-up time of 23.4 months, with no deaths. In conclusion, interrupted aortic arch, severe truncal valve insufficiency, coronary anomalies, and repair later than 100 days of age were risk factors for death after repair of truncus arteriosus. In the absence of these associations, truncus arteriosus can be repaired with excellent surgical outcome in the neonatal and early infancy period. Repair in the early neonatal period reduces the prevalence of postoperative pulmonary vascular morbidity.

  View details for Web of Science ID A1993LG08700011

  View details for PubMedID 8501932

• FATE OF SMALL HOMOGRAFT CONDUITS AFTER EARLY REPAIR OF TRUNCUS ARTERIOSUS 29TH Annual Meeting of the Society-of-Thoracic-Surgeons Heinemann, M. K., Hanley, F. L., Fenton, K. N., Jonas, R. A., Mayer, J. E., Castaneda, A. R. ELSEVIER SCIENCE INC. 1993: 1409–12

  Abstract

  Neonatal repair of truncus arteriosus is being performed in a number of centers, often with the use of small homograft conduits. The fate of the homograft and the risk of replacement were the subjects of this study. Between January 1987 and October 1991, 43 infants aged less than 3 months (range, 3 to 90 days) underwent primary repair of truncus arteriosus including implantation of a valved homograft conduit (diameter, 7 to 12 mm). Twenty-nine had follow-up of more than 6 months (range, 6 to 65 months; mean, 21.9 months). After a mean period of 31 months (range, 8 to 65 months), 7 patients showed obstruction with right ventricular pressures at least 75% systemic and underwent either a conduit change (n = 5) or a patch augmentation (n = 2). Mean cardiopulmonary bypass time at reoperation was 99 minutes; mortality was zero. Five other children are known to have a right ventricular pressure of 50% to 60% systemic, 2 having undergone balloon dilation. Statistical comparison of the patients with conduit reoperation or high right ventricular pressure (n = 12) with the rest of the population (n = 17) revealed an elevated pulmonary artery to right ventricular pullback gradient on postoperative day 1 after the repair (7.7 versus 1.3 mm Hg; p = 0.001) and choice of an aortic over a pulmonary homograft (100% versus 64.7%; p = 0.065) as significant risk factors. Age and weight at repair, postoperative pulmonary artery pressure, length of follow-up, and size of the homograft showed no significant differences between the two groups.(ABSTRACT TRUNCATED AT 250 WORDS)

  View details for Web of Science ID A1993LH44100011

  View details for PubMedID 8512389

• HALOTHANE AS AN ANESTHETIC FOR FETAL SURGERY JOURNAL OF PEDIATRIC SURGERY Sabik, J. F., Assad, R. S., Hanley, F. L. 1993; 28 (4): 542-547

  Abstract

  Halothane has become the preferred anesthetic agent during fetal surgery because it can be administered via maternal inhalation and it improves surgical exposure by relaxing the uterus. However, the effects of halothane anesthesia on fetal cardiovascular homeostasis during fetal surgery have not been documented. In 10 pregnant ewes, inhalation halothane anesthesia was administered and their fetuses were instrumented for cardiovascular evaluation. During a 1-hour period we evaluated the acute effects of halothane anesthesia on fetal hemodynamics, arterial blood gases, cardiac output, placental blood flow, total vascular resistance, systemic vascular resistance, and placental vascular resistance. Fetal cardiac output and placental blood flow were determined by the radiolabelled microsphere technique and resistances were calculated using pressure and flow data. These findings were compared to both the results we obtained in 15 fetal sheep anesthetized with the maternal administration of intravenous ketamine, and to the accepted values found in nonanesthetized, chronically instrumented fetal sheep. Our findings indicate that with halothane anesthesia during fetal surgery fetal cardiac output and placental blood flow significantly decrease, and total vascular resistance increases. Placental vascular resistance increases out of proportion to systemic vascular resistance, resulting in the shunting of blood away from the placenta. The combination of decreased cardiac output and increased shunting of blood away from the placenta causes depressed respiratory gas exchange. These findings are not present with other anesthetic agents. Halothane has significant negative effects on both the fetal heart and the peripheral vasculature which disrupt fetal cardiovascular homeostasis. Halothane is a poor anesthetic during fetal intervention.

  View details for Web of Science ID A1993KY51400006

  View details for PubMedID 8483067

• OUTCOMES IN NEONATAL PULMONARY ATRESIA WITH INTACT VENTRICULAR SEPTUM - A MULTIINSTITUTIONAL STUDY 72ND ANNUAL MEETING OF THE AMERICAN ASSOC FOR THORACIC SURGERY Hanley, F. L., Sade, R. M., Blackstone, E. H., Kirklin, J. W., Freedom, R. M., Nanda, N. C., Foker, J. E., Williams, W. G. MOSBY-ELSEVIER. 1993: 406–27

  Abstract

  A total of 171 neonates with pulmonary atresia and intact ventricular septum were entered into a prospective multiinstitutional study between January 1, 1987, and January 1, 1991. Treatment was not assigned randomly but was selected by the responsible physicians. The Z-value (standard deviation units) of the diameter of the tricuspid valve was less than -2 in 52% of patients and less than -4 in 26%; it was highly correlated with right ventricular cavity size (which was small in 90% of patients and was severely reduced in 54%). Coronary artery-right ventricular fistulas were present in 45% of patients, and right ventricular dependency was severe in 9%; diameter (Z-value) of the tricuspid valve was negatively correlated (P < 0.0001) with the prevalence of both. Survival was 81% at 1 month after the first intervention and 64% at 4 years; the hazard function (instantaneous risk of death) declined rapidly after the initial procedure but remained appreciable for 24 months. Multivariable analysis showed small diameter of the tricuspid valve, severe right ventricular coronary dependency, birth weight, and the date and type of initial procedure to be risk factors for time-related death. Subsequent procedures were performed in 51% of patients. Among patients undergoing an initial procedure that did not include a systemic-pulmonary artery shunt, only 49% did not receive a shunt in the subsequent 1 month; small size of the tricuspid valve was the only risk factor for receiving the subsequent shunt. Ninety-eight percent of living patients whose initial procedure did not include a transannular patch were free of a subsequently placed transannular patch within 1 month, but only 45% were free of it 3 years later; no risk factors were identified. Eighteen percent of living patients had received a one-ventricle repair within 3 years, and 32% had received a two-ventricle repair; the remainder (50%) had incompletely separated pulmonary and systemic circulations. The only patient-specific risk factor for not receiving a two-ventricle repair was the Z-value of the tricuspid valve.

  View details for Web of Science ID A1993KR71500005

  View details for PubMedID 8445920

• Exclusion of the placenta during fetal cardiac bypass augments systemic flow and provides important information about the mechanism of placental injury. journal of thoracic and cardiovascular surgery Fenton, K. N., Heinemann, M. K., Hanley, F. L. 1993; 105 (3): 502-510

  Abstract

  The in utero correction of congenital cardiac malformations requires the availability of fetal cardiac bypass. One difficulty with fetal cardiac bypass is that very high flow rates are necessary when the placenta is left in the bypass circuit; the placenta requires about 40% of fetal cardiac output, which results in a normal cardiac output of 400 ml/kg per minute. Previous attempts to perform fetal cardiac bypass failed to consistently achieve these high flow rates because of cannula size limitations. On the basis of previous work done in our laboratory with an isolated-placenta model, which demonstrated that at normothermia the placenta would tolerate at least 30 minutes of cessation of umbilical blood flow, we hypothesized that exclusion of the placenta from the fetal cardiac bypass circuit would reduce fetal cardiac output by one half and allow us to obtain better systemic perfusion without compromising placental function. Cardiac bypass was performed in 20 late-gestation fetal lambs. In 10 lambs, no drugs were given; 5 served as controls in which the placenta was perfused; in the last 5, the placenta was excluded by clamping the umbilical cord during bypass. The latter 10 lambs were treated with indomethacin, which is known to improve placental blood flow after fetal cardiac bypass. We measured blood gases and determined regional blood flow with radiolabeled microspheres to assess placental function after bypass. The 5 control fetuses experienced rapid hypercapnea and hypoxemia after bypass, in association with minimal placental blood flow; when the placenta was excluded, arterial carbon dioxide tension rose somewhat more slowly, and placental blood flow after bypass was significantly better. When indomethacin was given, arterial blood gases in both groups showed a mild increase in carbon dioxide tension and similar placental blood flows (about 30% of baseline) after bypass. Indomethacin is known to block the vasoconstrictive response of the placenta to fetal cardiac bypass, implicating the release of vasoactive cyclooxygenase products as the cause of the adverse effects. In this study, placental perfusion on bypass without indomethacin caused much more severe placental dysfunction than did bypass with the placenta excluded from the circuit. The use of indomethacin improved postbypass placental function in both groups, but this effect was much more dramatic in the placenta-perfused group.(ABSTRACT TRUNCATED AT 400 WORDS)

  View details for PubMedID 8445928

• EXCLUSION OF THE PLACENTA DURING FETAL CARDIAC BYPASS AUGMENTS SYSTEMATIC FLOW AND PROVIDES IMPORTANT INFORMATION ABOUT THE MECHANISM OF PLACENTAL INJURY JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY Fenton, K. N., Heinemann, M. K., Hanley, F. L., Jones, K. 1993; 105 (3): 502-512

  View details for Web of Science ID A1993KR71500015

• PREOPERATIVE MANAGEMENT OF NEONATAL TETRALOGY OF FALLOT WITH ABSENT PULMONARY VALVE SYNDROME ANNALS OF THORACIC SURGERY Heinemann, M. K., Hanley, F. L. 1993; 55 (1): 172-174

  Abstract

  In tetralogy of Fallot with absent pulmonary valve syndrome, aneurysmal enlargement of the central pulmonary arteries can be associated with severe bronchial stenosis and respiratory distress in the neonatal period. The ventilatory status of these patients must be stabilized before diagnostic and therapeutic interventions can be performed. This report describes the experience with prone positioning and emergency sternotomy in the preoperative care of a neonate.

  View details for Web of Science ID A1993KF24800035

  View details for PubMedID 8417672

• Surgical management of late right ventricular failure after Mustard or Senning repair. Circulation Chang, A. C., Wernovsky, G., Wessel, D. L., Freed, M. D., Parness, I. A., Perry, S. B., O'Brien, P., Van Praagh, R., Hanley, F. L., Jonas, R. A. 1992; 86 (5): II140-9

  Abstract

  Information on surgical management and outcome in patients who develop symptomatic right ventricular failure after prior Mustard or Senning operations is limited.From March 1987 to March 1991, 10 patients 3.6-23.5 years old (median, 7.0 years) with transposition of the great arteries and prior Mustard (six patients) or Senning (four patients) repairs (performed at ages 2 months to 5 years; median, 6 months) underwent surgical intervention for symptomatic right ventricular failure. In five of 10 patients, anatomic correction with either an arterial switch operation (three patients) or a pulmonary artery-to-aorta anastomosis and right ventricle-to-pulmonary artery conduit (two patients) was performed. Before anatomic correction in these five patients, four of five patients had a pulmonary artery band to prepare the left ventricle. The interval between preparation and correction ranged from 8 days to 12 months (median, 2 months). One patient died after an arterial switch operation. In the remaining five patients, coexisting left ventricular dysfunction precluded anatomic correction; all five patients survived cardiac transplantation. Survival for the entire group of 10 patients is 90%, and the median postoperative hospital stay was 17 days. During follow-up (12-62 months; median, 27 months), there were no deaths. Neoaortic insufficiency after anatomic correction was common (mild in one patient, moderate in two patients, and severe in one patient who required aortic valve replacement 4 months after surgery). In the transplantation group, one patient developed lymphoma 3 months after transplantation but is currently in remission after reduction of immunosuppression.In patients who develop late right ventricular failure after Mustard or Senning repair, surgical intervention with either anatomic correction or cardiac transplantation can be done with acceptable morbidity and low mortality. Neoaortic valve insufficiency demands close follow-up after anatomic correction.

  View details for PubMedID 1423991

• FETAL CARDIAC BYPASS ALTERS REGIONAL BLOOD FLOWS, ARTERIAL BLOOD-GASES, AND HEMODYNAMICS IN SHEEP AMERICAN JOURNAL OF PHYSIOLOGY Bradley, S. M., Hanley, F. L., Duncan, B. W., Jennings, R. W., JESTER, J. A., Harrison, M. R., Verrier, E. D. 1992; 263 (3): H919-H928

  Abstract

  Successful fetal cardiac bypass might allow prenatal correction of some congenital heart defects. However, previous studies have shown that fetal cardiac bypass may result in impaired fetal gas exchange after bypass. To investigate the etiology of this impairment, we determined whether fetal cardiac bypass causes a redistribution of fetal regional blood flows and, if so, whether a vasodilator (sodium nitroprusside) can prevent this redistribution. We also determined the effects of fetal cardiac bypass with and without nitroprusside on fetal arterial blood gases and hemodynamics. Eighteen fetal sheep were studied in utero under general anesthesia. Seven fetuses underwent bypass without nitroprusside, six underwent bypass with nitroprusside, and five were no-bypass controls. Blood flows were determined using radionuclide-labeled microspheres. After bypass without nitroprusside, placental blood flow decreased by 25-60%, whereas cardiac output increased by 15-25%. Flow to all other fetal organs increased or remained unchanged. Decreased placental blood flow after bypass was accompanied by a fall in PO2 and a rise in PCO2. Nitroprusside improved placental blood flow, cardiac output, and arterial blood gases after bypass. Thus fetal cardiac bypass causes a redistribution of regional blood flow away from the placenta and toward the other fetal organs. Nitroprusside partially prevents this redistribution. Methods of improving placental blood flow in the postbypass period may prove critical to the success of fetal cardiac bypass.

  View details for Web of Science ID A1992JP90200040

  View details for PubMedID 1415619

• EXTRACORPOREAL-CIRCULATION IN THE ISOLATED INSITU LAMB PLACENTA - HEMODYNAMIC CHARACTERISTICS JOURNAL OF APPLIED PHYSIOLOGY Assad, R. S., Lee, F. Y., BERGNER, K., Hanley, F. L. 1992; 72 (6): 2176-2180

  Abstract

  Decreased placental perfusion and respiratory gas exchange have been observed after experimental fetal cardiopulmonary bypass (CPB). To better characterize placental hemodynamics during CPB, seven isolated in situ lamb placentas were placed on a CPB circuit by use of umbilical arterial and venous cannulation. Measures were taken to simulate normal placental hemodynamics. Perfusion flow rates were varied from 15 to 300 ml.min-1.kg fetal wt-1 during normothermia and hypothermia. Placental vascular resistance (PVR) remained constant when perfusion pressure and flow were varied above 40 mmHg and 150 ml.min-1.kg-1, respectively. Below these values, PVR varied inversely. This increase in PVR was more marked when CPB was performed with hypothermia than with normothermia. The clinical implication is that decreased placental flow and pressure on CPB may lead to a vicious cycle, resulting in further impairment of placental perfusion and respiratory gas exchange. Hypothermia promotes this impairment.

  View details for Web of Science ID A1992HZ31700019

  View details for PubMedID 1629070

• LEFT HEART SUPPORT WITH A VENTRICULAR ASSIST DEVICE IN AN INFANT WITH ACUTE MYOCARDITIS CRITICAL CARE MEDICINE Chang, A. C., Hanley, F. L., Weindling, S. N., Wernovsky, G., Wessel, D. L. 1992; 20 (5): 712-715

  View details for Web of Science ID A1992HT86600028

  View details for PubMedID 1572200

• PROSTAGLANDIN SYNTHESIS INHIBITION PREVENTS PLACENTAL DYSFUNCTION AFTER FETAL CARDIAC BYPASS JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY Sabik, J. F., Assad, R. S., Hanley, F. L. 1992; 103 (4): 733-742

  Abstract

  Surgical therapy of certain congenital heart lesions in utero may have advantages over postnatal repair or palliation. For fetal heart operations to be done, it will be necessary to devise a method of fetal cardiac bypass. Previous studies in which standard cardiopulmonary bypass techniques were used have reported fetal death resulting from increased placental vascular resistance, which causes decreased placental blood flow and depressed respiratory gas exchange. The mechanism responsible for this increase in placental vascular resistance has remained unknown. In a series of 10 fetal cardiac bypass experiments we examined the role of prostaglandins as the mediators of this response. Observations were made during a 1-hour prebypass period, a 30-minute bypass period, and a 2-hour postbypass period. The cardiac bypass circuit consisted of a centrifugal pump, and bypass flows were adjusted to equal a normal fetal cardiac output of 400 ml/min/kg. In six of the experiments indomethacin (3 mg/100 ml) was added to the pump priming to block prostaglandin synthesis. By means of the microsphere technique, fetal cardiac output, placental blood flow, individual organ blood flow, and placental vascular resistance were determined at five times during the experiments: presternotomy, poststernotomy, during cardiac bypass, at 5 minutes after cessation of bypass, and 30 minutes after cessation of bypass. Fetal arterial blood gas measurements were made every 15 to 30 minutes. When indomethacin was used to inhibit prostaglandin synthesis, placental vascular resistance did not increase, placental blood flow did not decrease, and fetal blood gases remained at normal prebypass levels during and after fetal cardiac bypass. We propose that production of vasoactive prostaglandins is responsible for the increased placental vascular resistance and decreased placental blood flow observed after fetal cardiac bypass. An understanding of the mechanism responsible for the increased placental vascular resistance seen after fetal cardiac bypass will be an important first step before clinical application.

  View details for Web of Science ID A1992HM95900015

  View details for PubMedID 1548915

• FACTORS ASSOCIATED WITH MARKED REDUCTION IN MORTALITY FOR FONTAN OPERATIONS IN PATIENTS WITH SINGLE VENTRICLE 71ST ANNUAL MEETING OF THE AMERICAN ASSOC FOR THORACIC SURGERY Mayer, J. E., Bridges, N. D., Lock, J. E., Hanley, F. L., Jonas, R. A., Castaneda, A. R. MOSBY-ELSEVIER. 1992: 444–52

  Abstract

  The Fontan operation was originally employed for patients with tricuspid atresia, but its application has been extended to those with a variety of complex defects in which there is functionally only a single ventricular chamber. The outcome of 225 modified Fontan operations carried out between 1984 and 1990 at the Children's Hospital, Boston, for patients with defects other than tricuspid atresia was reviewed. Overall 30 patients (13.3%) had failure of this operation (death or takedown). Results improved significantly during the period of the study, with failure rates of 6.5% (2 of 31) and 3.4% (2 of 59) in the last 2 years of the review. Multivariate analysis showed that pulmonary artery distortion, pulmonary artery pressure, age less than 3 years, use of a nonoxygenated glucose K+ cardioplegic solution, and cardiopulmonary bypass time greater than 180 minutes were associated with worse outcome, while "favorable" atrioventricular valve anatomy (non-left atrioventricular valve stenosis/atresia or common atrioventricular valve) and age greater than 9 years were associated with improved outcome. Excluding cardiopulmonary bypass time from the multivariate analysis, the technique of atrial partitioning for patients with left atrioventricular valve atresia/stenosis became the most important variable, followed by the others noted in the initial multivariate analysis except for age greater than 9 years. In the last 2 years of the study 31.1% of patients were less than 3 years of age. During the period of the study there was no significant decline in preoperative risk factors. These results show that modified Fontan operations can be carried out with a high likelihood of success in properly selected patients with complex defects in whom there is functionally a single ventricle.

  View details for Web of Science ID A1992HJ64500007

  View details for PubMedID 1545543

• TOLERANCE OF PLACENTA TO NORMOTHERMIC UMBILICAL CIRCULATORY ARREST JOURNAL OF MATERNAL-FETAL INVESTIGATION Assad, R. S., Lee, F. Y., Sabik, J., Mackenzie, S., Hanley, F. L. 1992; 2 (3): 145-150

  View details for Web of Science ID A1992JV44100001

• FUNCTION OF THE ANATOMIC PULMONARY VALVE IN THE SYSTEMIC CIRCULATION 63RD SCIENTIFIC SESSIONS OF THE AMERICAN HEART ASSOC Jenkins, K. J., Hanley, F. L., Colan, S. D., Mayer, J. E., Castaneda, A. R., Wernovsky, G. AMER HEART ASSOC. 1991: 173–79

  View details for Web of Science ID A1991GP41600025

• Function of the anatomic pulmonary valve in the systemic circulation. Circulation Jenkins, K. J., Hanley, F. L., Colan, S. D., Mayer, J. E., Castañeda, A. R., Wernovsky, G. 1991; 84 (5): III173-9

  Abstract

  The anatomic pulmonary valve, which has thin leaflets with little elastic tissue in the normal heart, must function as the neoaortic valve after arterial switch operation (ASO) for transposition of the great arteries, palliative surgery for hypoplastic left heart syndrome (HLHS), and pulmonary artery-to-aortic (P-A) anastomosis for complex heart disease with subaortic obstruction. The long-term function of this valve under these circumstances is not known. To investigate the function of this valve in the systemic circulation, the follow-up echocardiograms, catheterization data, and angiograms were reviewed for 189 patients at our institution after an ASO (n = 112), palliative surgery for HLHS (n = 45), or P-A anastomosis (n = 32). In addition, the effect on valve function of preoperative anatomy, prior placement of a pulmonary artery band (PAB), and length of follow-up was examined. Neoaortic regurgitation was present in 41% of patients after an ASO (mean +/- SD) follow-up (20 +/- 20; range, 2.2-80.5 months), 60% of patients after an HLHS repair (21 +/- 15; range, 3.7-62.4 months) and 50% after a P-A anastomosis (27 +/- 21; range, 2.6-89.4 months). Only eight patients had more than trivial/mild regurgitation. No neoaortic stenosis was observed. Minor preoperative valve abnormalities did not influence postoperative valve function. Prior PAB placement significantly increased the likelihood of postoperative neoaortic regurgitation after a two-stage ASO but not after a P-A anastomosis. In the ASO group, patients with an intact ventricular septum had a significantly higher prevalence of neoaortic regurgitation than those with a ventricular septal defect.(ABSTRACT TRUNCATED AT 250 WORDS)

  View details for PubMedID 1718628

• A RANDOMIZED CLINICAL-TRIAL OF 10-PERCENT PENTASTARCH (LOW-MOLECULAR WEIGHT HYDROXYETHYL STARCH) VERSUS 5-PERCENT ALBUMIN FOR PLASMA-VOLUME EXPANSION AFTER CARDIAC OPERATIONS JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY London, M. J., Ho, J. S., Triedman, J. K., Verrier, E. D., Levin, J., Merrick, S. H., Hanley, F. L., Browner, W. S., Mangano, D. T. 1989; 97 (5): 785-797

  Abstract

  Pentastarch is a hydroxyethyl starch similar to hetastarch, but with a lower average molecular weight (264,000 versus 450,000) and fewer hydroxyethyl groups (molar substitution ratio = 0.45 versus 0.70). These characteristics result in enhanced enzymatic hydrolysis, faster renal elimination (initial intravascular half-life = 2.5 versus 25.5 hours), and less effect on coagulation. We report on a randomized clinical trial comparing the clinical efficacy and safety of 10% pentastarch (group P) for plasma volume expansion after cardiac operations with that of 5% serum albumin (group A). During the first 24 hours after arrival of the patient in the intensive care unit, colloid was infused to maintain a cardiac index of 2.0 L/m2 or more and a mean arterial pressure within 10% of the preinduction value. Group P (n = 50) received 1706 +/- 393 ml of colloid (mean +/- standard deviation) during this period, and group A (n = 44), 1794 +/- 341 ml (p = no significant difference). Hemodynamic responses to infusion were similar for both groups, although in group P a greater increase in both cardiac index (0.5 +/- 0.5 versus 0.3 +/- 0.5 L/min/m2 in group A, p less than 0.01) and left ventricular stroke work index (10.8 +/- 8.0 versus 5.8 +/- 6.0 gm-m/m2, p less than 0.01) was observed during infusion of the first 500 ml. There were no significant differences in any of the measured respiratory parameters (alveolar-arterial oxygen gradient, estimated shunt fraction, and effective pulmonary compliance). Hemodilution with colloid significantly reduced serum protein levels in group P by 24 hours postoperatively (4.0 +/- 0.6 versus 5.0 +/- 0.7 gm/dl in group A, p less than 0.05), although mean serum colloid osmotic pressure was similar (15.4 +/- 2.6 [P] versus 15.5 +/- 2.7 mmHg [A], p = no significant difference). There were no significant between-group differences in prothrombin time, activated partial thromboplastin time, platelet count, bleeding time, or coagulation factors (fibrinogen, V, VII, VIII, or IX) on postoperative days 1 and 7. Perioperative fluid balance, weight change, chest tube output, red blood, platelet, or fresh frozen plasma usage, reexploration for bleeding, and clinical outcome were also similar. These findings indicate that pentastarch is as safe and effective s 5% albumin for plasma volume expansion after cardiac operations with no apparent adverse effects on coagulation. If commercially available at a lower cost than albumin, it would appear to be a reasonable first choice for colloid therapy in this setting.

  View details for Web of Science ID A1989U452900017

  View details for PubMedID 2468978

• THE MUSTARD PROCEDURE IN INFANTS (LESS THAN 100 DAYS OF AGE) - 10-YEAR FOLLOW-UP JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY Turley, K., Hanley, F. L., Verrier, E. D., Merrick, S. H., Ebert, P. A. 1988; 96 (6): 849-853

  Abstract

  Surgical treatment of transposition of the great arteries and intact ventricular septum has changed markedly in the past 10 years. However, long-term follow-up on new approaches is not available. In a unique group of patients, operated on in the first 100 days of life with the Mustard procedure, such follow-up is available, and the results of these true infant repairs represent a benchmark against which subsequent techniques applied to infants should be compared. During the period of 1975 to 1980, 36 infants, less than 100 days of age, who had transposition of the great arteries and intact ventricular septum, underwent Mustard repair at the University of California, San Francisco. Ages ranged from 4 to 98 days (mean 46 days) and weights from 2.3 to 6.6 kg (mean 3.5 kg). There were no early deaths, and late follow-up was available from 8 to 13 years (mean 10 years). The late survival rate was 97% (mean 10 years). There was a 62% rhythm disturbance-free survival rate, 89% reoperation-free survival rate, and 91% pacemaker-free survival rate. Echocardiographic evaluation revealed obstruction of the superior vena cava in eight patients, tricuspid insufficiency in four, right ventricular dysfunction in two, and left ventricular outflow tract obstruction in three. The Mustard procedure, performed in the first 100 days of life, results in a high rate of survival (early 100% and late 97%, at a mean of 10 years) and a low incidence of late complications against which other techniques of infant repair should be compared.

  View details for Web of Science ID A1988R320700003

  View details for PubMedID 3193798

• ROLE OF ADENOSINE IN CORONARY AUTOREGULATION AMERICAN JOURNAL OF PHYSIOLOGY Hanley, F. L., GRATTAN, M. T., Stevens, M. B., Hoffman, J. I. 1986; 250 (4): H558-H566

  Abstract

  The role of cardiac interstitial adenosine as an important metabolite in coronary autoregulation has not been established. We therefore measured steady-state cardiac interstitial adenosine concentration at a high and a low coronary inflow pressure using an epicardial diffusion well in anesthetized dogs. Although coronary resistance for the high and low pressure points showed highly significant differences (P less than 0.001), adenosine averaged 302 +/- 98 and 286 +/- 91 (SD) pmol/ml for the high and low pressure points, respectively (P greater than 0.20). Cardiac interstitial adenosine concentration was then measured with and without an intracoronary infusion of adenosine deaminase catalytic subunit. Adenosine averaged 28 +/- 21 (SD) pmol/ml during the infusion compared with 281 +/- 68 during control conditions (P less than 0.001). Finally, pressure-flow relations were obtained with and without the adenosine deaminase infusion, and there was no loss of autoregulation in the pressure of adenosine deaminase. These findings indicate that intracoronary adenosine deaminase markedly reduces interstitial adenosine concentration, that cardiac interstitial adenosine concentration remains constant during autoregulation, and that the coronary bed autoregulates normally when interstitial adenosine is reduced to levels close to zero. We conclude that cardiac interstitial adenosine concentration is not an important component in coronary autoregulation.

  View details for Web of Science ID A1986A933300004

  View details for PubMedID 3963213

• TRANSMURAL CORONARY FLOW RESERVE PATTERNS IN DOGS AMERICAN JOURNAL OF PHYSIOLOGY GRATTAN, M. T., Hanley, F. L., Stevens, M. B., Hoffman, J. I. 1986; 250 (2): H276-H283

  Abstract

  To investigate transmural variations in coronary flow reserve, we studied 20 anesthetized dogs with a Gregg cannula in the left main coronary artery. In 11 dogs, radionuclide-labeled microspheres were injected over a range of perfusion pressures in the control state and during maximal coronary vasodilation produced with chromonar or adenosine. In another nine dogs, control, reactive hyperemic, and adenosine-vasodilated flows were compared at the same perfusion pressures. Adenosine dilated vessels more than did reactive hyperemia, which in turn vasodilated more than did hypoperfusion. Adenosine or chromonar vasodilated more than did hypoperfusion alone in all layers of the heart at perfusion pressures as low as 30 mmHg (P less than 0.05). This effect was greatest in the subepicardium and least in the subendocardium and varied with perfusion pressure (P less than 0.05). Subendocardial-to-subepicardial flow ratios declined with diminishing perfusion pressure despite the fact that flow reserve was present in all layers. We conclude that exhaustion of flow reserve is not the mechanism by which subendocardial ischemia occurs.

  View details for Web of Science ID A1986A168500018

  View details for PubMedID 3946628

• REGULATION OF TRANSMURAL MYOCARDIAL BLOOD-FLOW JOURNAL OF BIOMECHANICAL ENGINEERING-TRANSACTIONS OF THE ASME Hoffman, J. I., Baer, R. W., Hanley, F. L., Messina, L. M. 1985; 107 (1): 2-9

  Abstract

  A major problem in understanding how myocardial blood flow is regulated is the common occurrence of subendocardial ischemia in many diseases, with or without coronary arterial disease. Two commonly held explanatory hypotheses were that high systolic intramyocardial pressures prevented flow to deep but not superficial muscle, or that in diastole tissue pressures were highest subendocardially. Neither hypothesis is tenable today, and the likeliest hypothesis is that retrograde systolic flow from the deeper muscle produces a longer time constant for diastolic flow in deep than in superficial muscle.

  View details for Web of Science ID A1985ACW1300001

  View details for PubMedID 3884895

• EFFECTS OF PRESSURE-GRADIENTS BETWEEN BRANCHES OF THE LEFT CORONARY-ARTERY ON THE PRESSURE AXIS INTERCEPT AND THE SHAPE OF STEADY-STATE CIRCUMFLEX PRESSURE-FLOW RELATIONS IN DOGS CIRCULATION RESEARCH Messina, L. M., Hanley, F. L., Uhlig, P. N., Baer, R. W., GRATTAN, M. T., Hoffman, J. I. 1985; 56 (1): 11-19

  Abstract

  When steady state pressure-flow relations are studied in the circumflex coronary artery, pressure gradients develop between it and other branches of the left coronary artery. To assess the effects of these pressure gradients, we compared the pressure axis intercept and shape of steady state circumflex pressure-flow relations in the presence and absence of gradients after autoregulation was abolished, both in the beating heart and during long diastoles in dogs. We used peripheral coronary pressures and radionuclide-labeled microspheres to assess arterial collateral flow. In the beating heart, interarterial pressure gradients reduced the curvature at low circumflex pressures, and overestimated the mean pressure axis intercept by 7.8 mm Hg (P less than 0.05). The results were similar for the pressure-flow relations derived during long diastoles. This overestimation exaggerates the difference between the pressure axis intercept and coronary sinus pressure. The peripheral coronary pressure and microsphere results indicate that these effects are mediated largely by arterial collateral flow.

  View details for Web of Science ID A1985AAV3500002

  View details for PubMedID 3967342

• DISORDERED AUTO-REGULATION OF CORONARY BLOOD-FLOW AFTER CARDIOPULMONARY BYPASS SURGICAL FORUM GRATTAN, M. T., Hanley, F. L., Messina, L. M., Turley, K., Hoffman, J. I. 1984; 35: 271-272

  View details for Web of Science ID A1984TW53800115

• THE EFFECTS OF CARDIOPULMONARY BYPASS ON CORONARY BLOOD-FLOW IN THE DOG JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY GRATTAN, M. T., Baer, R. W., Hanley, F. L., Messina, L. M., Turley, K., Hoffman, J. I. 1984; 88 (6): 982-992

  Abstract

  The effects of cardiopulmonary bypass on autoregulation, maximum coronary flow, and regional blood flow in the heart were investigated in 25 dogs. A Gregg cannula was inserted into the left main coronary artery, and pressure-flow relations were then measured in the autoregulating state or with vasodilation produced by intracoronary adenosine infusion before, during, and after cardiopulmonary bypass. Seventeen of the dogs had radioactive microspheres injected to investigate regional blood flow changes at the same times. (1) Autoregulation was not present after bypass for at least 3 hours. (2) Blood flow was shifted toward the subendocardium on bypass (increased subendocardial/subepicardial ratio) and tended to return to prebypass distribution following bypass. (3) Blood flow after bypass was not significantly different to the subendocardium and subepicardium. (4) Response to a coronary vasodilator (maximum coronary flow) was significantly affected by cardiopulmonary bypass: blood flow to all layers of the heart could be increased with adenosine after bypass. (5) Global lactate and oxygen metabolism were not adversely affected by bypass. We conclude that cardiopulmonary bypass abolished the normal autoregulation of coronary flow; this may predispose the incompletely revascularized patient to a "coronary steal" syndrome. However, the heart with normal coronary arteries is not underperfused in any layer after bypass. Thus, the bypass technique is not the cause of the subendocardial ischemia that sometimes complicates cardiac operations.

  View details for Web of Science ID A1984TV42000010

  View details for PubMedID 6503324

• ARTERIAL AND VENOUS CORONARY PRESSURE-FLOW RELATIONS IN ANESTHETIZED DOGS - EVIDENCE FOR A VASCULAR WATERFALL IN EPICARDIAL CORONARY VEINS CIRCULATION RESEARCH Uhlig, P. N., Baer, R. W., Vlahakes, G. J., Hanley, F. L., Messina, L. M., Hoffman, J. I. 1984; 55 (2): 238-248

  Abstract

  The coronary circulation of anesthetized dogs was tested for the presence of vascular waterfalls by manipulating coronary arterial and coronary venous pressures. The left main coronary artery and the coronary sinus were cannulated, and relationships between coronary artery pressure, coronary sinus pressure, and coronary flow were studied. Experiments were conducted during diastolic arrests, under steady state conditions, in the absence of autoregulation. Relations of coronary flow to coronary sinus pressure at constant coronary artery pressure were consistent with the presence of a vascular waterfall in the coronary sinus. When the great cardiac vein was cannulated, relations of great vein flow to great vein pressure at constant coronary artery pressure were consistent with the presence of a vascular waterfall in the great vein, indicating that waterfall behavior can occur in epicardial veins other than the coronary sinus. In dogs on right heart bypass, with the coronary sinus and great vein uncannulated, the relationship between right atrial pressure and coronary sinus pressure showed a waterfall pattern, indicating that the waterfall is not an artifact of venous cannulation. In the right heart bypass experiments, venous waterfall behavior was seen in beating hearts as well as during diastolic arrests. We conclude that a vascular waterfall is present in epicardial coronary veins which can significantly influence coronary blood flow.

  View details for Web of Science ID A1984TE60200010

  View details for PubMedID 6611215

• THE EFFECT OF CORONARY INFLOW PRESSURE ON CORONARY VASCULAR-RESISTANCE IN THE ISOLATED DOG HEART CIRCULATION RESEARCH Hanley, F. L., Messina, L. M., GRATTAN, M. T., Hoffman, J. I. 1984; 54 (6): 760-772

  Abstract

  The shape of the coronary arterial pressure-flow relationship results from the interaction of a number of poorly understood physiological factors. Experiments in which coronary inflow and outflow pressures were coupled so that driving pressure was held constant showed that changes in inflow or outflow pressures altered coronary blood flow: coronary vascular resistance varied inversely with changes inflow pressure below 50 mm Hg and with changes in outflow pressure below 80 mm Hg. The magnitude of the influence of inflow pressure on resistance also depended on the fixed level of outflow pressure, the influence being large when the outflow pressure was low, and small when it was high. Inflow and outflow pressures, then, are two physiological factors which are determinants of the shape of the pressure-flow relationship, and their interaction contributes to the degree of curvature found in a particular relationship. These findings suggest that the use of linear regression in the interpretation of pressure-flow relationships results in poor estimation of resistance and zero-flow pressure. Other experiments measuring regional coronary blood flow using radionuclide-labeled microspheres resulted in the same inverse relationship between inflow pressure and resistance, regardless of mural depth, indicating that inflow pressure may influence resistance by distending vessels, rather than by causing sequential cessation of perfusion in successive transmural layers.

  View details for Web of Science ID A1984SZ64400014

  View details for PubMedID 6733869

• DIRECT MEASUREMENT OF LEFT-VENTRICULAR INTERSTITIAL ADENOSINE AMERICAN JOURNAL OF PHYSIOLOGY Hanley, F., Messina, L. M., Baer, R. W., Uhlig, P. N., Hoffman, J. I. 1983; 245 (2): H327-H335

  Abstract

  Characterization of adenosine's role as a regulator of coronary blood flow requires accurate measurement of endogenous adenosine concentration in the left ventricular (LV) interstitial compartment. Existing techniques for determining adenosine in this compartment are indirect, requiring the acceptance of major assumptions before conclusions can be drawn. We describe a new technique utilizing a LV epicardial diffusion well that allows us to make rapid, direct measurement of LV interstitial adenosine concentration, avoiding many problems inherent in existing techniques. Our results show adenosine concentrations of 555 pmol/ml in resting anesthetized dogs, indicating a resting adenosine level well within the vasoactive range. Further experiments using intramyocardial bolus injections of methylene blue dye and [8-14C]adenosine indicate that the epicardial well receives adenosine from a transmural distribution of LV interstitium and not from epicardial sources only. The transmural interstitial adenosine is transported via small lymphatics to the epicardial surface of the heart where diffusion occurs into the epicardial well. We also examined diffusion characteristics of the parietal pericardial membrane and found that the rate constant of adenosine diffusion for this and the visceral pericardium are of the same order of magnitude, indicating that the extensively used standard pericardial superperfusate method probably underestimates cardiac interstitial adenosine concentration by 50% or more. The influence of the parietal pericardium adequately explains why our resting adenosine concentrations using the epicardial well are higher than those recently reported using the standard pericardial superperfusate method.

  View details for Web of Science ID A1983RC41900058

  View details for PubMedID 6881365

• INTRAUTERINE CARDIOTHORACIC SURGERY - THE FETAL LAMB MODEL ANNALS OF THORACIC SURGERY Turley, K., Vlahakes, G. J., Harrison, M. R., Messina, L., Hanley, F., Uhlig, P. N., Ebert, P. A. 1982; 34 (4): 422-426

  Abstract

  We have developed fetal lamb models of congenital cardiothoracic lesions that have been allowed to progress through birth for physiological study. Simulated lesions, simulated repairs, actual lesions, and actual repairs have been performed in this model. Sixty-two fetal lambs comprised the study group, including 48 in which models were created and 14 controls. Models included pulmonary stenosis, aortic stenosis, and diaphragmatic hernia. Gestational age ranged from 90 to 120 days (0.6 of normal gestation). In each pregnant ewe, laparotomy and hysterotomy were performed under general anesthesia, with care taken to avoid placental vessels. The foreleg was exposed, the appropriate anterior chest wall was isolated, and a thoracotomy was done. Thoracic or cardiac procedures then were performed under controlled transplacental anesthesia and perfusion. Following completion of the procedure, the fetal thoracotomy was closed, sterile antibiotic solution was placed in the amniotic sac, and the hysterotomy and laparotomy were closed. Subsequently the fetus either was allowed to progress to birth and infant study or underwent subsequent intrauterine repair and then was allowed to progress to birth and neonatal study. This fetal lamb model provides reproducible anatomical and pathophysiological lesions to facilitate the development of techniques for repair of such lesions in early infancy. Further, it offers the potential for developing methods of intrauterine cardiothoracic surgical repair.

  View details for Web of Science ID A1982PL74300011

  View details for PubMedID 6890330