Clinical Focus


  • Cardiac Surgery
  • Cardiothoracic Surgery, Pediatric
  • Cardiothoracic Surgery

Academic Appointments


Administrative Appointments


  • Chief, Division of Pediatric Cardiac Surgery (2001 - Present)

Honors & Awards


  • Bristol-Meyer Travel Fellowship, American College of Cardiology (1994-95)
  • Finalist Samson Resident Award, Western Thoracic Surgical Assoc. (1995)

Professional Education


  • Residency:Delhi University (1989) India
  • Residency:Children's Hospital Boston (1991) MA
  • Fellowship:Miami Children's Hospital (1992) FL
  • Residency:Delhi University (1986) India
  • Internship:MGM Hospital (1983)
  • Medical Education:Kakatiya Medical College (1983) India
  • MB, BS, Kakatiya Medical College, Warangal, AP, India, Medicine, Surgery (1983)
  • MCh, CTVS, All India Institute of Medical Sciences, New Delhi, India, Cardiothoracic Surgery (1989)
  • Fellow, Children’s Hospital, Miami, FL, Pediatric Cardiothoracic Surgery (1992)
  • Fellow, UCSF Medical Center, San Francisco, CA, Pediatric Cardiothoracic Surgery (1996)

Current Research and Scholarly Interests


V. Mohan Reddy, MD, chief of pediatric cardiac surgery at Packard Children’s Hospital and associate professor of cardiothoracic surgery at Stanford’s School of Medicine, specializes in correcting heart defects in premature or extremely low birth weight babies. In a Stanford School of Medicine news release, Reddy states that “a lot of pediatric cardiologists and neonatologists think these kids are inoperable, but I believe that is a myth. We have the most experience in the world operating on these extremely low birth-weight babies.”

A pioneer in the field of pediatric cardiac surgery, Reddy successfully operated on what surgeons at Lucile Packard Children’s Hospital at Stanford believe is the smallest baby ever to survive a type of open-heart surgery called an arterial switch procedure. He also provided the surgical expertise required to fashion the stent/valve combination required to replace a heart valve in a 9-month-old girl, the youngest child ever to undergo the procedure, without opening her chest or putting her on a heart-lung machine.
Reddy earned his masters degree from the Institute of Medicine in New Delhi and his medical degree from Kakatiya Medical College in Warngal, India. He completed residencies in India as well as serving as senior resident in pediatric cardiac surgery at The Children's Hospital in Boston. In 1992, he completed his fellowship at UCSF where he later became associate professor of surgery and director of pediatric cardiac surgery. He was a finalist for the Western Thoracic Surgical Association’s Samson Resident Award in 1995 and joined Stanford in July of 2001.

His research and clinical work focuses on fetal heart surgery, surgery of premature and very low birth weight neonates, and cerebral protection during heart surgery. Reddy is a member of many professional societies, including the American Association for Thoracic Surgeons, the American Heart Association, and the Society of Thoracic Surgeons, and has published more than 100 peer-reviewed articles.

2013-14 Courses


Graduate and Fellowship Programs


Journal Articles


  • 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

  • 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

  • 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 Web of Science ID 000317150600036

    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

  • Surgical reconstruction of peripheral pulmonary artery stenosis in Williams and Alagille syndromes JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY Monge, M. C., Mainwaring, R. D., Sheikh, A. Y., Punn, R., Reddy, V. M., Hanley, F. L. 2013; 145 (2): 476-481

    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

  • 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. 2013

    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 PubMedID 23764088

  • Low birth weight and very low birth weight neonates with congenital heart disease: timing of surgery, reasons for delaying or not delaying surgery. Seminars in thoracic and cardiovascular surgery. Pediatric cardiac surgery annual Reddy, V. M. 2013; 16 (1): 13-20

    Abstract

    Conventional management of low birth weight and very low birth weight neonates was composed of deferring corrective surgery by aggressive medical management or palliative surgery which does not require cardiopulmonary bypass. However, while waiting for weight gain, these neonates are at risk for various comorbidities. In the current era, this "wait and let the baby grow" approach has not been shown to result in better clinical outcomes. Early primary repair hence has become the standard strategy for congenital heart disease requiring surgery in these neonates. However, there still exist some circumstances, which are considered to be unfavorable for corrective surgery due to medical, physiologic, surgeon's technical and institutional-systemic factors. We reviewed the recent literature and examined the reasons for delaying or not delaying surgery.

    View details for DOI 10.1053/j.pcsu.2013.01.004

    View details for PubMedID 23561813

  • 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., Mainwaring, R. D., Reddy, V. M., Hanley, F. L. 2013

    View details for PubMedID 23871887

  • Short-term Neurodevelopmental Outcomes in Neonates with Congenital Heart Disease: The Era of Newer Surgical Strategies CONGENITAL HEART DISEASE Chock, V. Y., Chang, I. J., Reddy, V. M. 2012; 7 (6): 544-550

    Abstract

    The objective of this study was to determine neurodevelopmental outcomes up to 30 months of age in a cohort of neonates requiring surgical intervention without circulatory arrest for congenital heart disease and to correlate these outcomes with characteristics detected prior to hospital discharge.An observational cohort of surviving neonates who underwent surgical intervention without circulatory arrest for congenital heart disease between 2002 and 2003 was studied at a single tertiary care institution.Thirty-five patients were followed from 4 to 6 months of age until 24-30 months of age.Neuromotor abnormalities, use of special services, and degree of developmental delay at set intervals between 4 and 30 months of age were retrospectively obtained from clinical reports. The relationship between these outcomes and clinical characteristics prior to hospital discharge was analyzed.Those with neuromotor abnormalities prior to discharge were likely to have persistent abnormalities in muscle strength, tone, and symmetry until 4-6 months of age, odds ratio 6 (1.3-29). By 24-30 months of age, motor abnormalities or developmental delay occurred in 10 of 20 infants (50%), but were no longer significantly associated with predischarge findings.Infants undergoing surgical intervention for congenital heart disease are at risk for neurodevelopmental abnormalities, which may not become apparent until months after hospital discharge. Early impairment may also resolve over time. Close developmental follow-up in this high-risk cohort of patients is warranted.

    View details for DOI 10.1111/j.1747-0803.2012.00678.x

    View details for Web of Science ID 000311611000011

    View details for PubMedID 22676547

  • Reconstruction of pulmonary artery with porcine small intestinal submucosa in a lamb surgical model: Viability and growth potential JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY Boni, L., Chalajour, F., Sasaki, T., Snyder, R. L., Boyd, W. D., Riemer, R. K., Reddy, V. M. 2012; 144 (4): 963-?

    Abstract

    This study investigated the time-dependent remodeling and growth potential of porcine small intestine submucosa as a biomaterial for the reconstruction of pulmonary arteries in a lamb model.Left pulmonary arteries were partially replaced with small intestine submucosal biomaterial in 6 lambs. Two animals each were humanely killed at 1, 3, and 6 months. Computed tomographic angiography, macroscopic examination of the implanted patch, and microscopic analysis of tissue explants were performed.All animals survived without complications. Patency and arborization of the pulmonary arteries were detected 6 months after implantation. There was no macroscopic narrowing or aneurysm formation in the patch area. The luminal appearance of the patch was similar to the intimal layer of the adjacent native pulmonary artery. Scanning electron microscopy showed that the luminal surface of the patch was covered by confluent cells. Immunohistochemical examination confirmed endothelialization of the luminal side of the patch in all of the explanted patches. The presence of smooth muscle cells in the medial layer was confirmed at all time points; however, expression of elastin, growth of the muscular layer, and complete degradation of patch material were detectable only after 6 months. The presence of c-Kit-positive cells suggests migration of multipotent cells into the patch, which may play a role in remodeling the small intestine submucosal biomaterial.Our data confirmed that remodeling and growth potential of the small intestine submucosal biomaterial are time dependent. Additional experiments are required to investigate the stability of the patch material over a longer period.

    View details for DOI 10.1016/j.jtcvs.2012.07.024

    View details for Web of Science ID 000309111600034

    View details for PubMedID 22917684

  • 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 Web of Science ID 000308138900031

    View details for PubMedID 22857982

  • 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

  • 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 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

  • 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

  • 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

  • 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

  • A randomized, controlled trial of aprotinin in neonates undergoing open-heart surgery PEDIATRIC ANESTHESIA Williams, G. D., Ramamoorthy, C., Pentcheva, K., Boltz, M. G., Kamra, K., Reddy, V. M. 2008; 18 (9): 812-819

    Abstract

    Neonates undergoing open-heart surgery are especially at risk for massive bleeding and pronounced inflammation. The efficacy of aprotinin, a serine protease inhibitor, at ameliorating these adverse effects of cardiopulmonary bypass has not been clearly demonstrated in neonates.Term neonates were enrolled and randomly assigned in a blinded fashion to receive saline (group P, placebo) or high-dose aprotinin (group A). Intraoperative management was standardized: surgeon, anesthesia, cardiopulmonary bypass and hemostasis therapy. Patients were admitted postoperatively to a pediatric cardiac intensive care unit. Primary outcome measure of efficacy was duration of the postoperative mechanical ventilation. Secondary outcome measures were total volume and units of blood products transfused intraoperatively and for 24 h after surgery, duration of chest tube in situ, and intensive care and hospital stays after surgery.Twenty-six neonates were enrolled; 13 received aprotinin and 13 received placebo. The study was halted prematurely because of US Food and Drug Administration's concerns about aprotinin's safety. Baseline patient, surgery and cardiopulmonary bypass characteristics were similar between groups. No outcome variables differed between groups (P > 0.05). Duration of postoperative ventilation was 115 +/- 139 h (group A); 126 +/- 82 h (group P); P = 0.29, and total blood product exposure was 8.2 +/- 2.6 U (group A); 8.8 +/- 1.4 U (group P); P = 0.1. Postoperative blood creatinine values did not differ between groups. In-hospital mortality rate was 4%.Aprotinin was not shown to be efficacious in neonates undergoing open-heart surgery. It is unclear whether adult aprotinin safety data are relevant to neonates undergoing open-heart surgery.

    View details for DOI 10.1111/j.1460-9592.2008.02678.x

    View details for Web of Science ID 000257990900002

    View details for PubMedID 18768040

  • Surgical management of coronary artery arising from the wrong coronary sinus, using standard and novel approaches JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY Gulati, R., Reddy, V. M., Culbertson, C., Helton, G., Suleman, S., Reinhartz, O., Silverman, N., Hanley, F. L. 2007; 134 (5): 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

  • Morphometry-based impedance boundary conditions for patient-specific modeling of blood flow in pulmonary arteries ANNALS OF BIOMEDICAL ENGINEERING Spilker, R. L., Feinstein, J. A., Parker, D. W., Reddy, V. M., Taylor, C. A. 2007; 35 (4): 546-559

    Abstract

    Patient-specific computational models could aid in planning interventions to relieve pulmonary arterial stenoses common in many forms of congenital heart disease. We describe a new approach to simulate blood flow in subject-specific models of the pulmonary arteries that consists of a numerical model of the proximal pulmonary arteries created from three-dimensional medical imaging data with terminal impedance boundary conditions derived from linear wave propagation theory applied to morphometric models of distal vessels. A tuning method, employing numerical solution methods for nonlinear systems of equations, was developed to modify the distal vasculature to match measured pressure and flow distribution data. One-dimensional blood flow equations were solved with a finite element method in image-based pulmonary arterial models using prescribed inlet flow and morphometry-based impedance at the outlets. Application of these methods in a pilot study of the effect of removal of unilateral pulmonary arterial stenosis induced in a pig showed good agreement with experimental measurements for flow redistribution and main pulmonary arterial pressure. Next, these methods were applied to a patient with repaired tetralogy of Fallot and predicted insignificant hemodynamic improvement with relief of the stenosis. This method of coupling image-based and morphometry-based models could enable increased fidelity in pulmonary hemodynamic simulation.

    View details for DOI 10.1007/s10439-006-9240-3

    View details for Web of Science ID 000245078300005

    View details for PubMedID 17294117

  • Visual light spectroscopy reflects flow-related changes in brain oxygenation during regional low-flow perfusion and deep hypothermic circulatory arrest JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY Amir, G., Ramamoorthy, C., Riemer, R. K., Davis, C. R., Hanley, F. L., Reddy, V. M. 2006; 132 (6): 1307-1313

    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

  • Modified and conventional ultrafiltration during pediatric cardiac surgery: Clinical outcomes compared JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY Williams, G. D., Ramamoorthy, C., Chu, L., Hammer, G. B., Kamra, K., Boltz, M. G., Pentcheva, K., McCarthy, J. P., Reddy, V. M. 2006; 132 (6): 1291-1298

    Abstract

    This prospective study compared clinical outcomes after heart surgery between three groups of infants with congenital heart disease. One group received dilutional conventional ultrafiltration (group D), another received modified ultrafiltration (group M), and a third group received both dilutional conventional and modified ultrafiltration (group B). We hypothesized that group B patients would have the best clinical outcome.Children younger than 1 year undergoing heart surgery for biventricular repair by the same surgeon were randomly allocated to one of the three study groups. Patient management was standardized, and intensive care staff were blinded to group allocation. Primary outcome measure was duration of postoperative mechanical ventilation. Other outcome measures recorded included total blood products transfused, duration of chest tube in situ, chest tube output, and stays in intensive care and in the hospital.Sixty infants completed study protocol. Mean age and weight were as follows: group D (n = 19), 61 days, 4.3 kg; group M (n = 20), 64 days, 4.5 kg; and group B (n = 21), 86 days, 4.4 kg. Preoperative and intraoperative characteristics were similar between groups. Ultrafiltrate volumes obtained were 196 +/- 93 mL/kg in group D, 105 +/- 33 mL/kg in group M, and 261 +/- 113 mL/kg in group B. There were no significant differences between groups for any outcome variable. Technical difficulties prevented completion of modified ultrafiltration in 2 of 41 infants.There was no clinical advantage in combining conventional and modified ultrafiltration. Because clinical outcomes were similar across groups, relative risks of the ultrafiltration strategies may influence choice.

    View details for DOI 10.1016/j.jtcvs.2006.05.059

    View details for Web of Science ID 000242626200010

    View details for PubMedID 17140945

  • The extracardiac conduit Fontan operation using minimal approach extracorporeal circulation: Early and midterm outcomes JOURNAL OF THORACIC AND CARDIOVASCULAR 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. 2006; 132 (5): 1054-1063

    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 Web of Science ID 000241497500009

    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

  • Unifocalization of major aortopulmonary collaterals in single-ventricle patients ANNALS OF THORACIC SURGERY Reinhartz, O., Reddy, V. M., Petrossian, E., Suleman, S., Mainwaring, R. D., Rosenthal, D. N., Feinstein, J. A., Gulati, R., Hanley, F. L. 2006; 82 (3): 934-939

    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 Web of Science ID 000239996300025

    View details for PubMedID 16928512

  • Homograft valved right ventricle to pulmonary artery conduit as a modification of the Norwood procedure CIRCULATION 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. 2006; 114: 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 DOI 10.1161/CIRCULATIONAHA.105.001438

    View details for Web of Science ID 000238688200097

    View details for PubMedID 16820644

  • Neurologic events in neonates treated surgically for congenital heart disease JOURNAL OF PERINATOLOGY Chock, V. Y., Reddy, V. M., Bernstein, D., Madan, A. 2006; 26 (4): 237-242

    Abstract

    The incidence of acute neurologic events prior to discharge in neonates with congenital heart disease (CHD) was determined and peri-operative characteristics predictive of a neurologic event were identified.A retrospective chart review over 1 year was conducted of infants <1 month of age with a diagnosis of CHD. Outcomes were measured by the occurrence of an acute neurologic event defined as electroencephalogram (EEG)-proven seizure activity, significant hypertonia or hypotonia, or choreoathetosis prior to hospital discharge. Stepwise logistic regression identified variables most likely to be associated with an acute neurologic event.Surgical intervention occurred in 95 infants who were admitted with a diagnosis of CHD. The survival rate was 92%. Of the survivors, 16 (17%) had an acute neurologic event, with 19% of events occurring preoperatively. Factors associated with neurologic events included an elevated nucleated red blood cell (NRBC) count, an abnormal preoperative brain imaging study, and a 5-min Apgar score <7 (P<0.05).Neonates with CHD have a significant risk of neurologic events. Preoperative brain imaging, the 5-min Apgar score, and initial serum NRBC counts may identify infants at highest risk for central nervous system injury.

    View details for DOI 10.1038/sj.jp.7211459

    View details for Web of Science ID 000241843200006

    View details for PubMedID 16496014

  • Percutaneous pulmonary valve placement in a 10-month-old patient using a hand crafted stent-mounte d porcine valve CATHETERIZATION AND CARDIOVASCULAR INTERVENTIONS Feinstein, J. A., Kim, N., Reddy, V. M., Perry, S. B. 2006; 67 (4): 644-649

    Abstract

    Percutaneous replacement of the pulmonary valve in a right ventricle to pulmonary artery (RV-PA) conduit using bovine jugular valves has recently been described as an alternative to surgical options in selected patients weighing >or=20 kg. We report the first case of transcatheter pulmonary valve implantation in an infant and the first use of "off the shelf" components. A 12-mm (Hancock) porcine valve was sutured into a predilated Genesis 2510b stent. The valved stent was manually crimped and mounted on a 12-mm balloon. Snare-assisted deployment in the middle of the RV-PA conduit was accomplished without the use of a long sheath. After the initial deployment, a 14-mm balloon was used to further approximate the stent to the walls of the conduit. Short-term (11 month) follow-up has demonstrated continued valve integrity, markedly decreased right ventricular size and improved function and symptomatic improvement. At 10 months of age, this case represents the youngest patient to undergo percutaneous valve placement and documents the technical feasibility of the procedure.

    View details for DOI 10.1002/ccd.20668

    View details for Web of Science ID 000236724200024

    View details for PubMedID 16532496

  • Antegrade cerebral perfusion reduces apoptotic neuronal injury in a neonatal piglet model of cardiopulmonary bypass JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY Chock, V. Y., Amir, G., DAVIS, C. R., Ramamoorthy, C., Riemer, R. K., Ray, D., Giffard, R. G., Reddy, V. M. 2006; 131 (3): 659-665

    Abstract

    Neonates with congenital heart disease might require surgical repair with deep hypothermic circulatory arrest, a technique associated with adverse neurodevelopmental outcomes. Antegrade cerebral perfusion is thought to minimize ischemic brain injury, although there are no supporting experimental data. We sought to evaluate and compare the extent of neurologic injury in a neonatal piglet model of deep hypothermic circulatory arrest and antegrade cerebral perfusion.Neonatal piglets undergoing cardiopulmonary bypass were randomized to deep hypothermic circulatory arrest or antegrade cerebral perfusion for 45 minutes. Animals were killed after 6 hours of recovery, and brain tissue was stained for evidence of cellular injury and for the apoptotic markers activated caspase 3 and cytochrome c translocation from mitochondria to cytosol.Piglets from the antegrade cerebral perfusion group exhibited less apoptotic or necrotic injury (4 +/- 3 vs 29 +/- 12 cells per field, P = .03). The piglets undergoing antegrade cerebral perfusion also had less evidence of apoptosis, with fewer cells staining for activated caspase 3 (57 +/- 8 vs 93 +/- 9 cells per field, P = .001) or showing cytochrome c translocation (6 +/- 2 vs 15 +/- 4 cells per field, P = .02).The use of antegrade cerebral perfusion in place of deep hypothermic circulatory arrest reduces evidence of apoptosis and histologic injury in neonatal piglets. Neonates with congenital heart disease might benefit from antegrade cerebral perfusion during complex cardiac surgery to improve their overall neurologic outcome.

    View details for DOI 10.1016/j.jtcvs.2005.09.005

    View details for Web of Science ID 000235940600024

    View details for PubMedID 16515920

  • 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

  • Myofibroblastic tumor of the heart: A rare intracardiac tumor JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY Gandy, K. L., Burtelow, M. A., Reddy, V. M., Silverman, N. H. 2005; 130 (3): 888-889

    View details for DOI 10.1016/j.jtcvs.2005.02.018

    View details for Web of Science ID 000231915400046

    View details for PubMedID 16153948

  • Preliminary results of fetal cardiac bypass in nonhuman primates JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY Ikai, A., Riemer, R. K., Ramamoorthy, C., Malhatra, S., Cassorla, L., Amir, G., Hanley, F. L., Reddy, V. M. 2005; 129 (1): 175-181

    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 expression of the hepatocyte growth factor receptor c-Met shifts from medial to intimal layer after cavopulmonary anastomosis JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY Ikai, A., Riemer, R. K., Ma, X. Y., Reinhartz, O., Hanley, F. L., Reddy, V. M. 2004; 127 (5): 1442-1449

    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

  • 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

  • Induced fibrillation is equally effective as crystalloid cardioplegia in the protection of fetal myocardial function JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY Malhotra, S. P., Thelitz, S., Riemer, R. K., Reddy, V. M., Suleman, S., Hanley, F. L. 2003; 125 (6): 1276-1282

    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

  • 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 Web of Science ID 000182807000014

    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
  • 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

  • Surgical creation of aortopulmonary window in selected patients with pulmonary atresia with poorly developed aortopulmonary collaterals and hypoplastic pulmonary arteries JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY Rodefeld, M. D., Reddy, V. M., Thompson, L. D., Suleman, S., Moore, P. C., Teitel, D. F., Hanley, F. L. 2002; 123 (6): 1147-1154

    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

  • 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

  • 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

  • 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

  • The Ross procedure in children and young adults: A word of caution JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY Laudito, A., Brook, M. M., Suleman, S., Bleiweis, M. S., Thompson, L. D., Hanley, F. L., Reddy, V. M. 2001; 122 (1): 147-153

    Abstract

    Disease of the aortic valve in children and young adults is a complex entity whose management is the subject of controversy. The Ross and the Ross-Konno procedures have become the primary choices for aortic valve replacement in children because of growth potential, optimal hemodynamic performance, and lack of the need for anticoagulation. However, concern persists regarding the longevity of the pulmonary autograft, especially in patients with aortic insufficiency.Between June 1993 and February 2000, 72 Ross and Ross-Konno procedures were performed at our institution: 81% of the patients were less than 15 years old. Preoperative, postoperative, and follow-up clinical, echocardiographic, and hemodynamic data were reviewed. Statistical analysis was performed to identify the risk factors for deteriorating autograft function.Aortic insufficiency was an indication for the Ross procedure in 17 patients and mixed lesions with predominant aortic insufficiency in 10. Of the 45 other patients, 32 had aortic stenosis and 13 had mixed lesions with predominant aortic stenosis. There were no deaths during a follow-up of 5 to 80 months. Autograft reoperation was necessary in the follow-up period in 7 patients for severe aortic insufficiency. Moderate insufficiency was identified in 5 additional patients. Aortic insufficiency or predominant aortic insufficiency, as a preoperative hemodynamic indication for the Ross procedure, reached statistical significance (P =.031) as a risk factor for autograft failure.The Ross and the Ross-Konno procedures have changed the prognosis of children and young adults with complex aortic valve disease. However, the Ross procedure should be performed with caution in older children in whom aortic insufficiency is a preoperative hemodynamic indication. Further follow-up to delineate the risk factors for autograft dysfunction in children and young adults is necessary to better define the indications for the Ross procedure.

    View details for Web of Science ID 000169837100020

    View details for PubMedID 11436048

  • 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

  • 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

  • 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

  • Cardiac surgery for premature and low birth weight neonates. Seminars in thoracic and cardiovascular surgery. Pediatric cardiac surgery annual Reddy, V. M. 2001; 4: 271-276

    Abstract

    Congenital heart defects in low birth weight infants are typically managed with supportive therapy or palliative surgery and definitive repair is delayed. However, the morbidity of this approach has been shown to be high. At the University of California San Francisco, our approach to these infants has been one of complete repair in the neonatal period or as early as possible. Since 1992, 116 neonates and infants under 2,500 g underwent complete repair of simple and complex cardiac defects using cardiopulmonary bypass and circulatory arrest was avoided in the majority. Actuarial survival was 80% at 1 year. Noncardiac abnormalities contribute significantly to perioperative and late morbidity and mortality. Reintervention rates are comparable to other neonates. Our data suggests that complete repair of simple and complex congenital heart defects can be performed safely and effectively in premature and low birth weight infants.

    View details for PubMedID 11460990

  • Techniques to avoid circulatory arrest in neonates undergoing repair of complex heart defects. Seminars in thoracic and cardiovascular surgery. Pediatric cardiac surgery annual Reddy, V. M., Hanley, F. L. 2001; 4: 277-280

    Abstract

    Advances in cardiopulmonary bypass and cardiac surgical techniques have tremendously improved the outlook for children born with congenital heart defects. However, cardiopulmonary bypass, hypothermia, and circulatory arrest are all unphysiologic states and are associated with some morbidity, and the most significant are neurologic sequelae, which can be subtle or devastating. Circulatory arrest, often used as an adjunct to deep hypothermia, is a major contributing factor to the neurologic complications after cardiopulmonary bypass. In the last decade, techniques to avoid circulatory arrest have been introduced with a substantial decrease in neurologic sequelae.

    View details for PubMedID 11460991

  • 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

  • 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

  • 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

  • Double-chambered right ventricle presenting in adulthood ANNALS OF THORACIC SURGERY McElhinney, D. B., Chatterjee, K. M., Reddy, V. M. 2000; 70 (1): 124-127

    Abstract

    Double-chambered right ventricle is a form of right ventricular outflow tract obstruction that develops over time, often in patients with an abnormally short distance between the moderator band and pulmonary valve. This lesion typically presents in childhood or adolescence and is often accompanied by a ventricular septal defect. Only a handful of previous cases have been described in which double-chambered right ventricle occurred in adulthood.Since 1992, three patients more than 30 years old (38, 43, and 66 years of age) have presented at our institution with unusual symptoms or a previous incorrect diagnosis. We reviewed the clinical data in these patients.Presenting symptoms included syncope, angina, and severe dyspnea resembling pulmonary hypertension. In 1 patient, disease was categorized as New York Heart Association class IV, and in the other 2 as class III. Coexisting anomalies included a patent foramen ovale or secundum atrial septal defect in 2 patients, a small ventricular septal defect in 1 (with a probable history of ventricular septal defect in another), and mild aortic regurgitation in 1. All patients required urgent or emergent operations, with peak pressures in the proximal right ventricular chamber of 135 to 180 mm Hg and severely depressed left ventricular function in 1 patient. Resection of the anomalous right ventricular muscle bundles was achieved through a right atrial approach in all patients. All patients were alive with improved functional status at follow-up, which was between 15 and 40 months.Right ventricular outflow tract obstruction resulting from a double-chambered right ventricle is rare in adults, but when it does occur it can present with unusual symptoms. When evaluating the patient with signs or symptoms of primary right heart failure, cardiologists should make an effort to image the entire right heart complex. Subcostal echocardiography can facilitate adequate visualization of the right ventricle when it is difficult to distinguish the subpulmonary outflow tract from the parasternal and apical windows.

    View details for Web of Science ID 000088318100030

    View details for PubMedID 10921695

  • Cardiac surgery in infants with very low birth weight. Seminars in pediatric surgery Reddy, V. M., Hanley, F. L. 2000; 9 (2): 91-95

    Abstract

    Congenital heart defects in low birth weight infants are typically managed with supportive therapy or palliative surgery, and definitive repair is delayed. However, the morbidity of such an approach has been shown to be high. Recent reports emphasize early surgical repair with good results in low birth weight and premature babies. However, there are no data on the outcomes in infants with very low birth weight (<1,500 g). Since 1990, the authors performed complete repair of congenital heart defects (other than isolated patent ductus arteriosus) in 20 infants weighing 1,500 g or less. Defects included aortic coarctation (n = 6), ventricular septal defect (n = 5), tetralogy of Fallot (n = 2), transposition of great arteries (n = 2), truncus arteriosus (n = 2), total anomalous pulmonary venous return (n = 1), double outlet right ventricle (n = 1), and pulmonary atresia with intact ventricular septum (n = 1). Preoperative morbidity was more common in patients who were referred late for corrective surgery. Modification of techniques of neonatal cardiopulmonary bypass were used. There were 2 early deaths (10%) caused by foot gangrene (n = 1), and pulmonary complications (n = 1). No patient had evidence of postbypass intracranial hemorrhage. At a median follow-up of 40 months, there was 1 late death, and 4 patients underwent a total of 5 surgical and catheter reinterventions. There was no evidence of neurological sequelae attributable to surgery. In most cases, delaying repair of congenital heart defects in low and very low birth weight infants does not confer any benefit and is associated with a higher incidence of preoperative morbidity. Complete repair of both simple and complex congenital heart lesions can be performed successfully in such patients with good early and medium-term results. Postoperative growth after repair approximates the normal growth curve for low birth weight infants without congenital heart disease. It is recommended that with few exceptions, such infants, especially when symptomatic, undergo early surgical correction rather than prolonged medical management or other forms of palliation.

    View details for PubMedID 10807231

  • 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

  • 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

  • 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

  • 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

  • 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

  • Endothelin receptor blockade prevents the rise in pulmonary vascular resistance after cardiopulmonary bypass in lambs with increased pulmonary blood flow JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY 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. 1999; 117 (2): 314-322

    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 JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY Reddy, V. M., McElhinney, D. B., Rajasinghe, H. A., Liddicoat, J. R., Hendricks-Munoz, K., Fineman, J. R., Hanley, F. L. 1999; 117 (2): 343-351

    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

  • Multiple ventricular septal defects: How and when should they be repaired? JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY Seddio, F., Reddy, V. M., McElhinney, D. B., Tworetzky, W., Silverman, N. H., Hanley, F. L. 1999; 117 (1): 134-139

    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

  • 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

  • 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

  • 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

  • Additional pulmonary blood flow with the bidirectional Glenn anastomosis: Does it make a difference? ANNALS OF THORACIC SURGERY McElhinney, D. B., Marianeschi, S. M., Reddy, V. M. 1998; 66 (2): 668-672

    Abstract

    The bidirectional cavopulmonary shunt has become a mainstay in the palliation of patients with a functional single-ventricle heart. However, there remain a number of unresolved issues regarding this procedure, many of which concern the response of the pulmonary vasculature to this unique circulatory physiology. Among the issues of debate are the role and effects of an additional source of pulmonary blood flow.Between January 1990 and April 1997, 160 patients underwent bidirectional cavopulmonary anastomosis. Median age at operation was 7.8 months, and age ranged from 24 days to 43 years. An additional source of pulmonary blood flow was included in 93 patients (58%). A retrospective review of our experience with this cohort was performed with a focus on the role of additional pulmonary blood flow.Eight patients (5%) died in the early postoperative period, and the overall early failure rate (death or take-down) was 7.5% (n=12). Eleven other patients underwent early reoperation to decrease (n=8) or increase (n=3) the amount of pulmonary blood flow. Early survivors were followed up for a median of 23 months, during which time 5 patients died and 30 patients underwent Fontan completion. Including early and late mortality, actuarial survival rates at 1 and 2 years were 91% and 88%, respectively.The bidirectional cavopulmonary shunt is a useful procedure in the early or intermediate-term management of patients with a functional univentricular heart. However, there is much still to be learned about this unique physiologic system. The role of accessory pulmonary blood flow remains unclear, as does the use of the bidirectional cavopulmonary shunt as long-term palliation. Pulmonary arteriovenous fistulas are a serious concern, especially in young patients with heterotaxy syndrome.

    View details for Web of Science ID 000075554900108

    View details for PubMedID 9725450

  • Pulmonary arteriovenous malformations in and out of the setting of congenital heart disease ANNALS OF THORACIC SURGERY Marianeschi, S. M., McElhinney, D. B., Reddy, V. M. 1998; 66 (2): 688-691

    Abstract

    Pulmonary arteriovenous malformations can occur in a variety of clinical situations, including liver disease, systemic disorders, or after palliation of congenital heart disease, with serious clinical consequences.We reviewed the potential mechanisms of this condition, diagnostic tools, and clinical management.Contrast echocardiography is an important diagnostic modality, which has been shown to be more sensitive than pulmonary arteriography, especially when rapid contrast injection is used. The finding that pulmonary capillary vasodilation is observed in hepatopulmonary syndrome, in cirrhotic patients, and after congenital heart repair is strongly suggestive that an unidentified hepatic factor is involved in inhibiting the development of pulmonary arteriovenous malformations.Prompt detection and treatment of pulmonary arteriovenous malformations is of utmost importance, to prevent serious clinical consequences. It may very well be the case that the etiology of arteriovenous malformations is multifactorial. We are now investigating the role of alterations of gene expression in the vascular remodeling that results in formation of pulmonary arteriovenous malformations.

    View details for Web of Science ID 000075554900113

    View details for PubMedID 9725455

  • Anomalous pulmonary venous return in the staged palliation of functional univentricular heart defects ANNALS OF THORACIC SURGERY McElhinney, D. B., Reddy, V. M. 1998; 66 (2): 683-687

    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, pulmonary, or systemic and 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 March 1997, 32 patients with functional single ventricle and anomalous pulmonary venous return underwent operation at our institution. Five of 25 patients who underwent neonatal palliation died in the early postoperative period, all of whom had obstructed anomalous pulmonary venous return. Twenty-one patients have undergone bidirectional cavopulmonary shunt, including 7 in whom this was the primary palliative procedure. There was one early and two late deaths after the bidirectional Glenn procedure, two in patients with asplenia syndrome and none in patients with previously obstructed pulmonary venous return. Seven patients have undergone Fontan completion, 5 with an extracardiac conduit. There was one early death and one take-down to a classic Glenn shunt, both in patients who did not undergo the extracardiac conduit Fontan operation.Anomalous pulmonary venous return can significantly complicate the management of the single-ventricle patient, with the major impact on survival coming in the neonatal period. Palliation with the aim of performing an extracardiac conduit Fontan procedure allows greater latitude and more streamlined management in this group of patients.

    View details for Web of Science ID 000075554900112

    View details for PubMedID 9725454

  • Partial biventricular repair for complex congenital heart defects: An intermediate option for complicated anatomy or functionally borderline right complex heart JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY Reddy, V. M., McElhinney, D. B., Silverman, N. H., Marianeschi, S. M., Hanley, F. L. 1998; 116 (1): 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

  • 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

  • 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

  • Geometric mismatch of pulmonary and aortic anuli in children undergoing the Ross procedure: Implications for surgical management and autograft valve function JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY Reddy, V. M., McElhinney, D. B., Phoon, C. K., Brook, M. M., Hanley, F. L. 1998; 115 (6): 1255-1262

    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? JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY Reddy, V. M., McElhinney, D. B., Brook, M. M., Parry, A. J., Hanley, F. L. 1998; 115 (5): 1032-1038

    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 ANNALS OF THORACIC SURGERY McElhinney, D. B., Reddy, V. M., Rajasinghe, H. A., Mora, B. N., Silverman, N. H., Hanley, F. L. 1998; 65 (2): 517-524

    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

  • Late results of repair of truncus arteriosus. Seminars in thoracic and cardiovascular surgery. Pediatric cardiac surgery annual Reddy, V. M., Hanley, F. 1998; 1: 139-146

    Abstract

    Over the last three decades, management of truncus arteriosus has evolved with improving outcomes. Surgical repair is currently performed primarily during the neonatal period. This has prevented the severe morbidity and mortality resulting from congestive heart failure and pulmonary vascular obstructive disease. Although it has been 30 years since successful surgical repair of this lesion, the long-term follow-up studies have been limited to children who underwent truncus repair beyond the infancy period. In this article, we review the literature and also summarize the long-term results of truncus arteriosus at the University of California, San Francisco, where a repair in early infancy has been routine since 1975. A retrospective review was performed to assess long-term outcomes among 165 patients (81% of patients were < 1 year of age) who survived the initial hospital stay following complete repair of truncus arteriosus since 1975. 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. The actuarial survival rate 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. Significant independent risk factors for poorer long-term survival were truncus with moderate to severe truncal valve insufficiency before repair. During the follow-up period, 107 patients underwent 133 conduit reoperations at a median of 5.5 years after the initial repair. In addition, 26 patients underwent 30 truncal valve replacements. Actuarial freedom from truncal valve replacement was 63% at 10 years among patients with truncal insufficiency before initial repair. Ten- to 20-year survival and functional status were excellent among infants undergoing complete repair of the truncus arteriosus. Copyright 1998 by W.B. Saunders Company

    View details for PubMedID 11486216

  • Cytokine Response to Fetal Cardiac Bypass Journal of maternal-fetal investigation : the official journal of French Society of Ultrasound in Medicine and Biology ... [et al.] Reddy, V. M., McElhinney, D. B., Rajasinghe, H. A., Rodriguez, J. L., Hanley, F. L. 1998; 8 (1): 46-9

    Abstract

    >Objective: Cytokines are associated with the systemic inflammatory response syndrome that occurs after cardiopulmonary bypass. We hypothesized that the placental dysfunction which has been found to complicate fetal cardiac bypass may be in part a function of a cytokine-mediated acute phase reaction. To test this hypothesis, we designed a study to investigate the effect of cardiac bypass on interleukin-1beta (IL-1beta), IL-6, and IL-8 in fetal sheep.Methods: Nine mixed-breed pregnant ewes at 118-122 days of gestation were assigned randomly to either the "fetal cardiac bypass group" (n = 5) or the "control group" (n = 4). After surgical exposure and instrumentation, cardiac bypass was performed for 30 min in study group fetuses, whereas control group fetuses were exposed and instrumented identically but did not undergo bypass. Placental and systemic hemodynamics were monitored in both groups. Pre- and post-bypass blood samples were analyzed for IL-1beta, IL-6, and IL-8 using enzyme-linked immunosorbent assays.Results: IL-6 levels were undetectable before bypass in all fetuses. IL-6 increased after bypass in all bypass group fetuses to 53.0 +/- 24.2 pg/ml, whereas IL-6 levels remained undetectable in all control animals. Fetal cardiac bypass produced no significant changes in IL-1beta and IL-8 in either group. Following bypass, placental blood flow decreased by 23% in the bypass group, which was significantly more (P = 0.0002) than the 6% decrease in the control group; placental vascular resistance increased significantly more in the bypass group (20%) than in control fetuses (1%; p = 0.004).Conclusions: Fetal cardiac bypass produces significant and consistent increases in fetal plasma IL-6, which correlate with increased placental vascular resistance and decreased placental blood flow. IL-6 may have an important role in placental dysfunction following fetal cardiac bypass, but further investigation will be necessary to elucidate its specific role in the impairment of placental function or as a marker of placental injury.

    View details for PubMedID 9524160

  • 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

  • Update on prospects for fetal cardiovascular surgery. Current opinion in pediatrics Reddy, V. M., McElhinney, D. B. 1997; 9 (5): 530-535

    Abstract

    With progress in the field of fetal echocardiography, it has become clear that certain obstructive lesions of the heart and great vessels may progress in utero, leading to more severe disease. Prenatal intervention may mitigate this process, ultimately leading to improved outcomes for patients with certain severe congenital cardiac defects. In this review, we provide an update on the prospects for fetal cardiac intervention, with a focus on the field of fetal cardiac surgery.

    View details for PubMedID 9360836

  • 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

  • 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

  • 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

  • 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

  • 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
  • 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

  • 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

  • Intraatrial baffle repair of isolated ventricular inversion with left atrial isomerism ANNALS OF THORACIC SURGERY McElhinney, D. B., Reddy, V. M., Silverman, N. H., Hanley, F. L. 1996; 62 (5): 1529-1532

    Abstract

    Isolated ventricular inversion with left atrial isomerism, partial anomalous pulmonary venous connection, and interruption of the inferior vena cava with azygos continuation to a right superior vena cava was diagnosed by echocardiography in a neonate. At 48 days of age, the patient underwent successful anatomic correction with redirection of flow from the superior vena cava and hepatic veins to the left-sided tricuspid valve, and flow from the pulmonary veins to the right-sided mitral valve. In the present report, the surgical techniques of this case are described, along with a survey of the surgical literature covering anatomic repair of isolated ventricular inversion.

    View details for Web of Science ID A1996VQ16700072

    View details for PubMedID 8893605

  • Key role of the alternate complement pathway in hyperacute rejection of rat hearts transplanted into fetal sheep TRANSPLANTATION Rajasinghe, H. A., Reddy, V. M., Hancock, W. W., Sayegh, M. H., Hanley, F. L. 1996; 62 (3): 407-411

    Abstract

    Hyperacute rejection (HAR) mediated by xenoreactive natural antibodies (XNA), which are thought to develop in early infancy, is a major impediment to transplantation between widely disparate species. The ability to diagnose certain forms of congenital heart defects early in prenatal life suggests the potential for these defects to be corrected by cardiac transplantation, prior to the development of XNA and host immunocompetence. This study investigated whether discordant cardiac xenotransplantation into fetal and neonatal recipients might obviate HAR due to the relative lack of XNA. Six neonatal lambs at 3 days (n=3) or 7 days (n=3) of life, and two fetal lambs at 125 and 142 days of gestation (term = 145 days) received cardiac grafts from adult Wistar-Furth (275-350 g) rats. All eight cardiac xenografts showed clinical evidence of HAR, with rapid swelling, loss of contractility, and ecchymosis and a mean survival time of 12.5 +/- 6.4 min. Sections of explanted grafts showed classical histologic features of HAR, including interstitial hemorrhages, platelet microthrombi, and edema, without leukocyte infiltration. Immunopathology of grafts harvested from fetal recipients showed a lack of significant intragraft deposition of sheep IgM, IgG, or C1q, but widespread endothelial labeling for C3, factor B, and properdin. In contrast, grafts in neonatal recipients showed IgM, IgG, and C1q deposition, as well as C3, factor B, properdin, and terminal complement (C) components. Fibrin deposition and platelet thrombi were seen in both groups of recipients. Injection of cobra venom factor resulted in prolongation of cardiac xenograft survival in neonatal lambs (n=3) to 12 hr. Analysis by immunohistology showed that normal sera from neonatal and adult, but not fetal, sheep contained IgM and IgG XNA reactive with rat cells. In conclusion, rodent grafts transplanted into fetal sheep undergo HAR, likely through direct activation of the alternate pathway of C, whereas neonatal lambs acquire XNA in the very early postnatal period and reject rat hearts through activation of both the classical and alternate pathways of C. Thus, at least in some species combinations, cardiac transplantation during the early postnatal period, or even in utero, may still be subject to development of HAR.

    View details for Web of Science ID A1996VC45000018

    View details for PubMedID 8779691

  • Fetal model of single ventricle physiology: Hemodynamic effects of oxygen, nitric oxide, carbon dioxide, and hypoxia in the early postnatal period JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY Reddy, V. M., Liddicoat, J. R., Fineman, J. R., McElhinney, D. B., Klein, J. R., Hanley, F. L. 1996; 112 (2): 437-449

    Abstract

    In patients with single ventricle physiology, the distribution of flow to the systemic and pulmonary circulations, which are in parallel, largely depends on the relative resistances in the respective vascular beds. Although neonatal palliation in patients with single ventricle physiology has become more common, medical management during the perinatal and perioperative periods is often based primarily on personal and institutional experience and is complicated by the transition to postnatal life and the effects of cardiopulmonary bypass. The lack of an animal model that suitably mimics single ventricle physiology has impeded progress in this area.The purpose of the current study was to investigate the effects of respiratory manipulations on pulmonary hemodynamics in the early neonatal period in a reproducible model of single ventricle physiology created in utero.A 10 mm Damus-Kaye-Stansel aortopulmonary anastomosis was created in fetal sheep (n = 14) at 140 +/- 1.2 days' gestation, with pulmonary blood flow provided through a 5 mm aortopulmonary shunt after ligation of the main pulmonary artery distally. Two to 3 days after delivery at term, lambs (n = 11) underwent an open sternotomy and 30 minutes of deep hypothermic circulatory arrest. Both before and after bypass, respiratory manipulations, including administration of nitric oxide (80 ppm), 100% oxygen, 10% oxygen, and 5% carbon dioxide, were performed and hemodynamic variables were measured.Nitric oxide and oxygen caused a decrease in pulmonary vascular resistance and an increase in the pulmonary/systemic blood flow ratio, both before and after bypass. Hypoxia and carbon dioxide produced a significant rise in pulmonary vascular resistance and a significant drop in the ratio of pulmonary to systemic blood flow.Oxygen, nitric oxide, and carbon dioxide all appear to be useful means of manipulating pulmonary vascular resistance and pulmonary/systemic blood flow ratio in neonatal lambs with single ventricle physiology, but further investigation is necessary to understand their dose responsive effects and the effects of prolonged administration.

    View details for Web of Science ID A1996VC10200032

    View details for PubMedID 8751513

  • Altered endothelium-dependent responses in lambs with pulmonary hypertension and increased pulmonary blood flow AMERICAN JOURNAL OF PHYSIOLOGY-HEART AND CIRCULATORY PHYSIOLOGY Reddy, V. M., Wong, J., Liddicoat, J. R., Johengen, M., Chang, R., Fineman, J. R. 1996; 271 (2): H562-H570

    Abstract

    To investigate early endothelial function associated with increased pulmonary blood flow, vascular shunts were placed between the ascending aorta and main pulmonary artery in 18 late-gestation fetal sheep. Four weeks after delivery, the lambs were instrumented to measure vascular pressures and blood flows, and blood was collected to measure plasma concentrations of guanosine 3',5'-cyclic monophosphate [cGMP, the second messenger to nitric oxide (NO)-mediated vasodilation] and L-arginine (the precursor for NO synthesis). The responses to the endothelium-dependent vasodilators acetylcholine (ACh, 1.0 microgram/kg) and ATP (0.1 mg.kg-1.min-1), the endothelium-independent vasodilators M & B-22948 (a cGMP-specific phosphodiesterase inhibitor, 2.5 mg/kg) and inhaled NO (40 ppm), and N omega-nitro-L-arginine (an inhibitor of NO synthase, 5 mg/kg) were then compared with responses in 12 age-matched controls. Vasodilator responses in control lambs were determined during pulmonary hypertension induced by U-46619 (a thromboxane A2 mimic). Shunted lambs displayed a selective impairment of endothelium-dependent pulmonary vasodilation, an augmented pulmonary vasoconstricting response to NO synthase inhibition, increased plasma cGMP concentrations, and decreased L-arginine concentrations. Taken together, these data suggest that lambs with pulmonary hypertension and increased pulmonary blood flow have early aberrations in endothelial function, as manifested by increased basal NO activity, that cannot be further increased by agonist-induced endothelium-dependent vasodilators.

    View details for Web of Science ID A1996VD63300024

    View details for PubMedID 8770097

  • Fetal cardiac bypass using an in-line axial flow pump to minimize extracorporeal surface and avoid priming volume ANNALS OF THORACIC SURGERY Reddy, V. M., Liddicoat, J. R., Klein, J. R., McElhinney, D. B., WAMPLER, R. K., Hanley, F. L. 1996; 62 (2): 393-400

    Abstract

    Progressive metabolic acidosis, hypoxia, and hypercarbia develop rapidly after fetal cardiac bypass mainly as a result of an increase in placental vascular resistance and a decrease in placental blood flow. A number of factors including fetal stress, priming substances, and extracorporeal surfaces have been identified as possible stimuli causing this placental dysfunction. The purpose of this study was to examine the effects of avoiding priming volume and minimizing extracorporeal surface area on placental hemodynamics and function.Fetal sheep (n = 16) at 118 to 122 days of gestation were subjected to cardiac bypass for 30 minutes using either an in-line axial-flow pump (Hemopump group: n = 8, no prime) or a roller pump with a venous reservoir (control group: n = 8, priming volume = 150 mL). After bypass, the fetuses were observed for 90 minutes. Placental blood flow and combined ventricular output were continuously measured with ultrasonic flow probes, and fetal blood gases were measured at specific intervals.Three fetuses in the control group died during the study, whereas all 8 fetuses in the Hemopump group remained in stable condition throughout the study period. During and after bypass, placental blood flow was significantly higher (p < 0.0001) and placental vascular resistance was significantly lower (p < 0.0001) in the Hemopump group than in the control group. Arterial pH and partial pressure of arterial oxygen declined significantly less (p < 0.0001), and partial pressure of arterial carbon dioxide increased significantly less (p = 0.0002) in the Hemopump group than in the control group.Reducing the extracorporeal surface area and avoiding external priming substances preserves placental hemodynamics after fetal cardiac bypass. An in-line axial-flow pump is useful in miniaturizing the bypass circuits for potential use in fetal cardiac surgery.

    View details for Web of Science ID A1996VA28700018

    View details for PubMedID 8694597

  • Long-term fetal outcome after fetal cardiac bypass: Fetal survival to full term and organ abnormalities JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY Reddy, V. M., Liddicoat, J. R., Klein, J. R., WAMPLER, R. K., Hanley, F. L. 1996; 111 (3): 536-543

    Abstract

    Earlier work suggests that fetal cardiac bypass is technically feasible but results in significant placental dysfunction. Many of the stimuli that initiate this placental dysfunction have been identified in the past several years and these involve fetal stress, extracorporeal surfaces, priming substances (maternal blood), and flow characteristics. Fetal survival with conventional methods of bypass has been far less than optimal. A novel fetal bypass circuit requiring no priming volume was designed incorporating an in-line axial flow pump (Hemopump, Johnson & Johnson Interventional Systems, Rancho Cordova, Calif.) and was demonstrated to have a marked beneficial effect on placental function.The purpose of this study was to investigate the effect of this newly developed customized fetal bypass circuit on fetal survival and developing fetal organs.Nine fetuses at 122 to 126 days of gestation were subjected to fetal cardiac bypass via a transsternal approach, with a 16F single right atrial venous cannula and a 12F arterial cannula. Normothermic cardiac bypass was continued for 30 minutes at flow rates of 320 +/- 32 ml/kg. Of the nine fetuses, one fetus was stillborn 4 days after bypass and eight (89%) were delivered alive after progressing to term gestation. One lamb died of blunt trauma 1 day after birth. All other lambs (n = 7) thrived normally, and at 1 week of age they were subjected to autopsy. No gross hemorrhagic or thromboembolic lesions were detected in the organs examined including the brain. Microscopic examination of representative sections from all organs revealed mild pleural reaction in two lambs, and in two other lambs the hepatocytes showed evidence of mild increase in glycogen content, the significance of which is unknown in relation to fetal bypass. In one fetus that was aborted there was evidence of mild to moderate neuronal loss in the cerebral cortex.This study demonstrates that with improvements in fetal extracorporeal circuitry and techniques very favorable fetal outcome can be achieved. Further studies are necessary to evaluate the effects of bypass on fetal brain in an appropriate animal model. Advances in extracorporeal circuitry to suit the unique fetal physiology increase the possibility of future clinical application.

    View details for Web of Science ID A1996UB98000008

    View details for PubMedID 8601967

  • Coarctation repair using end-to-side anastomosis of descending aorta to proximal aortic arch ANNALS OF THORACIC SURGERY Rajasinghe, H. A., Reddy, V. M., VANSON, J. A., Black, M. D., McElhinney, D. B., Brook, M. M., Hanley, F. L. 1996; 61 (3): 840-844

    Abstract

    Recurrent aortic coarctation after primary operative repair in the neonate and small infant is seen most commonly within the first year of life. Inadequate removal of ductal tissue, failure to address hypoplasia of the aortic arch, and suture line tension have been cited as important factors in early recurrence.To address these issues, we have used a technique of coarctation resection and extended anastomosis of the descending aorta to the undersurface of the aortic arch. THe salient features of this approach include extensive mobilization of the aortic arch and neck vessels, careful trimming of all ductal tissue, ligation of the isthmus just beyond the left subclavian artery, and end-to-side anastomosis of the descending aorta to a separate incision in the undersurface of the aortic arch proximal to all tubular hypoplasia. Between July 1992 and January 1995, 19 consecutive neonates (median age, 13 days) and 4 consecutive infants under 3 months of age (median age, 69 days) with a mean peak systolic upper to lower extremity resting gradient of 27.9 +/- 16.9 mm Hg underwent repair of aortic coarctation and tubular hypoplasia of the arch. Other procedures performed at the time of repair included ligation of a patent ductus arteriosus (n = 19), pulmonary artery banding (n = 3), and closure of ventricular septal or atrial septal defect (n = 3).There were no perioperative deaths. Early postoperative complication included a recurrent laryngeal nerve injury and a transient focal tonic clonic seizure. There was one late death, after a subsequent intracardiac surgical procedure, at a median follow-up of 16 months (range, 1 to 29 months). Twenty-one of 22 late survivors were free of recurrent aortic coarctation by echocardiography findings and clinical examination, with a median upper to lower extremity gradient of 0 mm Hg. Reintervention for recurrent aortic coarctation was not required in any survivor.The technique described herein completely removes all potentially abnormal tissue from the aorta, including ductal tissue and all tubular hypoplastic tissue proximal to the coarctation site.

    View details for Web of Science ID A1996TY99600017

    View details for PubMedID 8619703

  • Regression of tricuspid regurgitation after two-stage arterial switch operation for failing systemic ventricle after atrial inversion operation JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY VANSON, J. A., Reddy, V. M., Silverman, N. H., Hanley, F. L. 1996; 111 (2): 342-347

    Abstract

    The cases of five patients with previous Senning (n = 4) or Mustard (n = 1) operations and failing systemic ventricles in whom banding of the pulmonary artery was performed as an interim step toward an arterial switch procedure are reported. The rise in the ratio of left to right mean systolic ventricular pressure, from 0.35 before operation to 0.90 during operation and 0.80 on the first postoperative day, caused a shift of the ventricular septum from a leftward to a midline or nearly midline position. This shift was associated with a reduction of tricuspid regurgitation. At a median interval of 5.1 months after pulmonary artery banding, the mean left ventricular posterior wall thickness had increased to 8.2 mm, versus 5 mm before operation, and the mean left ventricular myocardial mass index had increased to 90 gm/m2, versus 55.6 gm/m2 before operation. After the arterial switch operation, which was performed in four patients, the tricuspid regurgitation decreased to a trivial amount (n = 1) or disappeared completely (n = 3).

    View details for Web of Science ID A1996TV53200012

    View details for PubMedID 8583807

  • Endothelin-1 vasoactive responses in lambs with pulmonary hypertension and increased pulmonary blood flow AMERICAN JOURNAL OF PHYSIOLOGY-HEART AND CIRCULATORY PHYSIOLOGY Wong, J., Reddy, V. M., HENDRICKSMUNOZ, K., Liddicoat, J. R., Gerrets, R., Fineman, J. R. 1995; 269 (6): H1965-H1972

    Abstract

    Increased concentrations of endothelin-1 (ET-1) are found in children with congenital heart diseases that produce increased pulmonary blood flow and pulmonary hypertension, but the role of ET-1 in the pathophysiology of pulmonary hypertension is unclear. Therefore, we investigated ET-1-induced vasoactive responses and ET-1 concentrations in an animal model of pulmonary hypertension and increased pulmonary blood flow. Vascular shunts were placed between the ascending aorta and main pulmonary artery in seven late-gestation fetal sheep. Four weeks after spontaneous delivery, ET-1 increased pulmonary vascular resistance by 29.7 +/- 34.4% (P < 0.05), the ETb-receptor agonist [Ala1,3,11,15]ET-1 (4AlaET-1) had no effect, and the ETa-receptor antagonist cyclo(D-Asp-L-Pro-D-Val-L-Leu-D-Trp) (BQ-123) decreased pulmonary vascular resistance by -16.0 +/- 5.6% (P < 0.05). In contrast, in six control lambs with a similar degree of pulmonary hypertension induced by U-46619, ET-1 and 4AlaET-1 decreased pulmonary vascular resistance by 24.8 +/- 17.6, and 20.0 +/- 13.8%, respectively (P < 0.05). In addition, systemic arterial concentrations of immunoreactive ET-1 were elevated in lambs with pulmonary hypertension (29.2 +/- 9.6 vs. 15.2 +/- 10.7 pg/ml, P < 0.05). Pulmonary hypertension and increased pulmonary blood flow alters the response of ET-1 from pulmonary vasodilation to vasoconstriction. These altered responses suggest a role for ET-1 and its receptors in the pathogenesis of pulmonary hypertension secondary to increased pulmonary blood flow.

    View details for Web of Science ID A1995TM58100016

    View details for PubMedID 8594905

  • MODIFIED SURGICAL TECHNIQUES FOR RELIEF OF AORTIC OBSTRUCTION IN (S,L,L) HEARTS WITH RUDIMENTARY RIGHT VENTRICLE AND RESTRICTIVE BULBOVENTRICULAR FORAMEN JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY VANSON, J. A., Reddy, V. M., Haas, G. S., Hanley, F. L. 1995; 110 (4): 909-915

    Abstract

    Modified techniques of aortopulmonary anastomosis were performed in six neonates with atrioventricular and ventriculoarterial discordance [S,L,L], double-inlet left ventricle, and restrictive bulboventricular foramen area (mean index 1.10 cm2/m2) with unobstructed aortic arch (n = 3) or with hypoplasia (n = 2) or interruption (n = 1) of the aortic arch. In cases of unobstructed aortic arch, a flap of autogenous aortic tissue was used to augment the posterior aspect of the anastomosis of the main pulmonary artery to the ascending aorta, thus creating the potential for anastomotic growth; this technique is applicable regardless of the position of the ascending aorta relative to the main pulmonary artery. In case of levo-transposition of the aorta with hypoplasia or interruption of the aortic arch, a modified Norwood procedure was performed, in that the proximal ascending aorta was divided at the same level as the main pulmonary artery with subsequent homograft patch augmentation from the main pulmonary artery-ascending aorta anastomosis to the level of the proximal descending aorta; this technique avoids a spiraling incision of the aorta and therefore reduces the risk of torsion of the aortic root with its inherent risks of obstruction of the coronary circulation and aortic or pulmonary valve regurgitation. There was no early or late mortality. At a mean follow-up of 16 months, in all patients, there was unobstructed aortic outflow, as evidenced by echocardiographic absence of a significant ventricular-aortic systolic gradient (mean 4.5 +/- 4 mm Hg) and absence of distal aortic arch obstruction. There was no evidence of aortic or pulmonary valve regurgitation. The reported modified techniques provide effective relief of restrictive bulboventricular foramen and aortic obstruction in [S,L,L] hearts.

    View details for Web of Science ID A1995TB91400004

    View details for PubMedID 7475156

  • IN-UTERO PLACEMENT OF AORTOPULMONARY SHUNTS - A MODEL OF POSTNATAL PULMONARY-HYPERTENSION WITH INCREASED PULMONARY BLOOD-FLOW IN LAMBS CIRCULATION Reddy, V. M., Meyrick, B., Wong, J., Khoor, A., Liddicoat, J. R., Hanley, F. L., Fineman, J. R. 1995; 92 (3): 606-613

    Abstract

    The development of pulmonary hypertension and its associated increased vascular reactivity is a common accompaniment of congenital heart disease with increased pulmonary blood flow. Although the morphology of the pulmonary vascular changes is well described, the mechanisms of vascular remodeling and increased reactivity remain incompletely understood.To elucidate these mechanisms, we established an accurate and reliable experimental model of pulmonary hypertension with increased pulmonary blood flow. An aortopulmonary shunt was created with an 8.0-mm expanded polytetrafluoroethylene vascular graft in 11 late-gestation fetal lambs. At 1 month of age, shunted lambs had a pulmonary-to-systemic blood flow ratio of 2.2 +/- 1.2. Compared with 11 age-matched control lambs, mean pulmonary arterial pressure (44.8 +/- 11.7 versus 16.2 +/- 2.9 mm Hg) and the ratio of pulmonary to systemic arterial pressure were significantly increased (P < .05). Pulmonary vascular resistance was not significantly increased. The pulmonary vasoconstricting response to the infusion of U46619 (a thromboxane A2 mimic) or acute alveolar hypoxia also was augmented in the shunted lambs. Morphometric analysis of the barium-filled pulmonary artery bed revealed medial hypertrophy, abnormal extension of muscle distally into the walls of the intra-acinar arteries, and increased numbers of barium-filled intra-acinar arteries.In utero placement of aortopulmonary shunts reproduces the aberrant hemodynamic state of children with cogenital heart disease with left-to-right shunts; postnatal pulmonary hypertension, increased pulmonary blood flow, and vascular remodeling. In addition, the lambs have a unique paradoxical increase in pulmonary vascular volume that attenuates an increase in pulmonary vascular resistance. This experimental preparation provides a useful and consistent model for the study of the pathogenesis of pulmonary hypertension.

    View details for Web of Science ID A1995RL56200046

    View details for PubMedID 7634475

  • 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

  • PRIMARY BIDIRECTIONAL SUPERIOR CAVOPULMONARY SHUNT IN INFANTS BETWEEN 1 AND 4 MONTHS OF AGE ANNALS OF THORACIC SURGERY Reddy, V. M., Liddicoat, J. R., Hanley, F. L. 1995; 59 (5): 1120-1126

    Abstract

    The performance of a primary bidirectional superior cavopulmonary shunt procedure in early infancy is attractive because it minimizes the number of operations needed before a Fontan procedure, avoids ventricular volume overload and its sequelae, and eliminates pulmonary artery distortion. However, concerns over elevated or labile pulmonary vascular resistance have limited its use in the first few months of life. Nine patients aged 1 to 4 months (5 patients, < 2 months) have undergone a primary bidirectional superior cavopulmonary shunt procedure between October 1992 and March 1994. Primary diagnoses were tricuspid atresia (n = 4), asplenia syndrome (n = 2), polysplenia syndrome (n = 1), double-outlet right ventricle (n = 1), and double-inlet left ventricle (n = 1). Associated lesions of immediate surgical importance were total anomalous pulmonary veins (n = 2), a restrictive atrial septum (n = 4), bilateral superior venae cavae (n = 5), and patent ductus arteriosus (n = 5). The surgical procedure consisted of unilateral (n = 4) or bilateral (n = 5) bidirectional superior cavopulmonary shunt and the repair of associated lesions. Of significance, in 4 of our first 5 patients a very limited additional source of pulmonary blood flow was provided because of a low arterial oxygen tension immediately after cardiopulmonary bypass. Pleural effusions developed in 2 of these 4 patients. In subsequent patients cardiopulmonary bypass was not used whenever possible or, if it was needed, use of an extra source of pulmonary blood flow was avoided.(ABSTRACT TRUNCATED AT 250 WORDS)

    View details for Web of Science ID A1995QW01400012

    View details for PubMedID 7733707

  • NEW APPROACH TO GREAT-VESSEL RECONSTRUCTION IN TRANSPOSITION COMPLEXES WITH INTERRUPTED AORTIC-ARCH ANNALS OF THORACIC SURGERY Liddicoat, J. R., Reddy, V. M., Hanley, F. L. 1994; 58 (4): 1146-1150

    Abstract

    Interrupted aortic arch can be associated with transposition of the great arteries with ventricular septal defect and with the Taussig-Bing anomaly. This usually results in a marked disparity between the hypoplastic ascending aorta and the dilated main pulmonary artery. Currently, standard arterial switch and arch repair is a widely applied approach to the surgical management of these lesions. Though the morphology of the great vessels in these lesions does not preclude this approach, the great-vessel mismatch can result in difficulties at and beyond the proximal neo-aortic to ascending aortic suture line that cause excessive bleeding, obstruction, tension, distortion, and the need of patch augmentation of the ascending aorta or arch with nonviable material. We present an alternative surgical approach used in 2 patients, 1 with Taussig-Bing anomaly and interrupted aortic arch and the other with transposition of the great arteries, ventricular septal defect, and interrupted aortic arch, that greatly simplifies the reconstruction of the neo-aorta and repair of the interrupted arch and avoids these potential hazards.

    View details for Web of Science ID A1994PL57200045

    View details for PubMedID 7944768

Conference Proceedings


  • Surgical outcomes for patients with pulmonary atresia/major aortopulmonary collaterals and Alagille syndrome Mainwaring, R. D., Sheikh, A. Y., Punn, R., Reddy, V. M., Hanley, F. L. OXFORD UNIV PRESS INC. 2012: 235-241

    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

  • Surgical Reconstruction of Tracheal Stenosis in Conjunction With Congenital Heart Defects Mainwaring, R. D., Shillingford, M., Davies, R., Koltai, P., Navaratnam, M., Reddy, V. M., Hanley, F. L. ELSEVIER SCIENCE INC. 2012: 1266-1273

    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 Web of Science ID 000302120200049

    View details for PubMedID 22381444

  • Selective Right Ventricular Unloading and Novel Technical Concepts in Ebstein's Anomaly 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: 1975-1981

    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

  • Cavopulmonary anastomosis induces pulmonary expression of the angiotensin II receptor family Malhotra, S. P., Reddy, V. M., Thelitz, S., He, Y. P., Hanley, F. L., Suleman, S., Riemer, R. K. MOSBY-ELSEVIER. 2002: 655-660

    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

  • Hemidiaphragmatic paralysis increases postoperative morbidity after a modified Fontan operation 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: 856-862

    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

  • A prospective randomized study comparing volume-standardized modified and conventional ultrafiltration in pediatric cardiac 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: 220-228

    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

  • Improved surgical outcome after fetal diagnosis of hypoplastic left heart syndrome Tworetzky, W., McElhinney, D. B., Reddy, V. M., Brook, M. M., Hanley, F. L., Silverman, N. H. LIPPINCOTT WILLIAMS & WILKINS. 2001: 1269-1273

    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

  • Coronary arterial size late after the atrial inversion procedure for transposition of the great arteries: Implications for the arterial switch operation Amin, Z., McElhinney, D. B., Moore, P., Reddy, V. M., Hanley, F. L. MOSBY-ELSEVIER. 2000: 1047-1052

    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

  • Revision of previous Fontan connections to total extracardiac cavopulmonary anastomosis: A multicenter experience 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: 340-344

    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

  • 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 McElhinney, D. B., Reddy, V. M., Silverman, N. H., Hanley, F. L. MOSBY-YEAR BOOK INC. 1997: 718-725

    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

  • Long-term follow-up of truncus arteriosus repaired in infancy: A twenty-year experience Rajasinghe, H. A., McElhinney, D. B., Reddy, V. M., Mora, B. N., Hanley, F. L. MOSBY-YEAR BOOK INC. 1997: 869-878

    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

  • Revision of previous fontan connections to extracardiac or intraatrial conduit cavopulmonary anastomosis McElhinney, D. B., Reddy, V. M., Moore, P., Hanley, F. L. ELSEVIER SCIENCE INC. 1996: 1276-1282

    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 McElhinney, D. B., Reddy, V. M., Hanley, F. L. ISRAEL JOURNAL MED SCIENCES. 1996: 880-885

    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

  • Morphologic determinants favoring surgical aortic valvuloplasty versus pulmonary autograft aortic valve replacement in children VANSON, J. A., Reddy, V. M., Black, M. D., Rajasinghe, H., Haas, G. S., Hanley, F. L. MOSBY-YEAR BOOK INC. 1996: 1149-1156

    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