Bio


I specialize in providing cardiac critical care to infants, children and adults with congenital heart disease and heart failure. I am the Associate Director for the Pulmonary Artery Reconstruction Program at Stanford, helping to coordinate comprehensive multidisciplinary care for children with severe pulmonary artery abnormalities and right ventricular dysfunction. I was a Faculty Fellow at the Stanford Center for Biodesign in 2019-2020 and continue to work on development of new technologies for the unmet needs of pediatric patients. I have an interest in physician wellness and completed the Wellness Director course through the WellMD Center at Stanford.

Clinical Focus


  • Pediatric Cardiology

Academic Appointments


Professional Education


  • Residency: University of California San Francisco (2005) CA
  • Medical Education: Michigan State University College of Human Medicine Office of the Registrar (2002) MI
  • Fellowship: Lucile Packard Children's Hospital (2009) CA
  • Fellowship: Columbia University (2008) NY
  • Board Certification: American Board of Pediatrics, Pediatric Cardiology (2010)

All Publications


  • Airway Characteristics of Patients With 22q11 Deletion Undergoing Pulmonary Artery Reconstruction Surgery: Retrospective Cohort Study. Pediatric critical care medicine : a journal of the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care Societies Sganga, D., Meister, K., Sidell, D. R., Wise-Faberowski, L., Shek, J., Ma, M., Martin, E., Hanley, F. L., McElhinney, D., Asija, R. 2022

    Abstract

    OBJECTIVES: We have previously shown that patients with a chromosome 22q11 microdeletion are at risk for prolonged respiratory failure after pulmonary artery reconstruction surgery compared with those with normal genotype. We sought to describe preexisting airway abnormalities in this patient population and examine relationships between airway abnormalities and outcomes.DESIGN: Single-center retrospective chart review from Society of Thoracic Surgery and Pediatric Cardiac Critical Care Consortium databases and the electronic medical record.SETTING: Lucile Packard Children's Hospital at Stanford from September 2017 to February 2019.PATIENTS: All patients undergoing pulmonary artery reconstruction surgery were considered for inclusion.INTERVENTIONS: We identified 127 patients meeting study inclusion criteria. Thirty-nine patients met specific criteria and underwent screening preoperative bronchoscopy including microdirect laryngoscopy and lower airway examination. Postoperative bronchoscopy was performed at the discretion of the intensive care team.MEASUREMENTS AND MAIN RESULTS: Airway abnormalities were detected in 25/26 of children (96%) with a chromosome 22q11 deletion who underwent preoperative bronchoscopy. Upper and lower airway pathologies were found in 19/25 (73%) and 21/25 (81%) patients, respectively, and it was common for patients to have more than one abnormality. Presence of 22q11 deletion was associated with longer duration of mechanical ventilation (9.1 vs 4.3 d; p = 0.001), use of noninvasive positive pressure support (13 vs 6 d; p = 0.001), and longer hospital stays (30 vs 14 d; p = 0.002). These outcomes were worse when compared with patients with known airway abnormalities who did not have 22q11 deletion.CONCLUSIONS: Preexisting upper and lower airway pathologies are common in patients with a chromosome 22q11 deletion who undergo pulmonary artery reconstruction surgery. Despite similar postoperative hemodynamics and outcomes as their counterparts without 22q11 deletion, 22q11 deletion is associated with more postoperative respiratory complications not entirely explained by preexisting airway abnormalities.

    View details for DOI 10.1097/PCC.0000000000002921

    View details for PubMedID 35213412

  • Standardized Training for Physicians Practicing Pediatric Cardiac Critical Care. Pediatric critical care medicine : a journal of the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care Societies Tabbutt, S., Krawczeski, C., McBride, M., Amirnovin, R., Owens, G., Smith, A., Wolf, M., Rhodes, L., Hehir, D., Asija, R., Teele, S. A., Ghanayem, N., Zyblewski, S., Thiagarajan, R., Yeh, J., Shin, A. Y., Schwartz, S. M., Schuette, J., Scahill, C., Roth, S. J., Hoffman, T. M., Cooper, D. S., Byrnes, J., Bergstrom, C., Vesel, T., Scott, J. P., Rossi, A., Kwiatkowski, D., DiPietro, L. M., Connor, C., Chen, J., Charpie, J., Bochkoris, M., Affolter, J., Bronicki, R. A. 2021

    Abstract

    OBJECTIVES: In the vast majority of Children's Hospitals, the critically ill patient can be found in one of three locations: the PICU, the neonatal ICU, and the cardiac ICU. Training, certification, and maintenance of certification for neonatology and critical care medicine are over seen by the Accreditation Council for Graduate Medical Education and American Board of Pediatrics. There is no standardization of training or oversight of certification and maintenance of certification for pediatric cardiac critical care.DATA SOURCES: The curricula from the twenty 4th year pediatric cardiac critical care training programs were collated, along with the learning objectives from the Pediatric Cardiac Intensive Care Society published "Curriculum for Pediatric Cardiac Critical Care Medicine."STUDY SELECTION: This initiative is endorsed by the Pediatric Cardiac Intensive Care Society as a first step toward Accreditation Council for Graduate Medical Education oversight of training and American Board of Pediatrics oversight of maintenance of certification.DATA EXTRACTION: A taskforce was established of cardiac intensivists, including the directors of all 4th year pediatric cardiac critical care training programs.DATA SYNTHESIS: Using modified Delphi methodology, learning objectives, rotational requirements, and institutional requirements for providing training were developed.CONCLUSIONS: In the current era of increasing specialized care in pediatric cardiac critical care, standardized training for pediatric cardiac critical care is paramount to optimizing outcomes.

    View details for DOI 10.1097/PCC.0000000000002815

    View details for PubMedID 34554132

  • Transfusion Outcomes in Patients Undergoing Unifocalization and Repair of Tetralogy of Fallot With Major Aortopulmonary Collaterals. World journal for pediatric & congenital heart surgery Wise-Faberowski, L. n., Irvin, M. n., Quinonez, Z. A., Long, J. n., Asija, R. n., Margetson, T. D., Hanley, F. L., McElhinney, D. B. 2020; 11 (2): 159–65

    Abstract

    Surgical repair of tetralogy of Fallot and major aortopulmonary collaterals (TOF/MAPCAs) involves unifocalization of MAPCAs and reconstruction of the pulmonary arterial circulation. Surgical and cardiopulmonary bypass (CPB) times are long and suture lines are extensive. Maintaining patency of the newly anastomosed vessels while achieving hemostasis is important, and assessment of transfusion practices is critical to successful outcomes.Clinical, surgical, and transfusion data in patients with TOF/MAPCAs repaired at our institution (2013-2018) were reviewed. Types and volumes of blood products used in the perioperative period, in addition to the use of antifibrinolytics and/or procoagulants (factor VIII inhibitor bypassing activity [FEIBA]; anti-inhibitor coagulant complex), were assessed. Outcome measures included days on mechanical ventilation (DOMV), postoperative intensive care unit and hospital length of stay (LoS), and incidence of thrombosis.Perioperative transfusion data from 279 patients were analyzed. Surgical (879 ± 175 minutes vs 684 ± 257 minutes) and CPB times (376 ± 124 minutes vs 234 ± 122 minutes) were longer in patients who received FEIBA than those who did not. Although the indexed volume of packed red blood cells (128.4 ± 82.2 mL/kg) and fresh frozen plasma (64.2 ± 41.1 mL/kg) was similar in patients who did and did not receive FEIBA, the amounts of cryoprecipitate (5.5 ± 5.2 mL/kg vs 5.8 ± 4.8 mL/kg) and platelets (19.5 ± 20.7 mL/kg vs 20.8 ± 13 mL/kg) transfused were more in those who did receive FEIBA.Perioperative transfusion is an important component in the overall surgical and anesthetic management of patients with TOF/MAPCAs. The intraoperative use of FEIBA was not associated with a decrease in the amount of blood products transfused, DOMV, or LoS or with an increase in thrombotic complications.

    View details for DOI 10.1177/2150135119892192

    View details for PubMedID 32093560

  • Unifocalization and pulmonary artery reconstruction in patients with tetralogy of Fallot and major aortopulmonary collateral arteries who underwent surgery before referral. The Journal of thoracic and cardiovascular surgery Ma, M. n., Zhang, Y. n., Wise-Faberowski, L. n., Lin, A. n., Asija, R. n., Hanley, F. L., McElhinney, D. B. 2020

    Abstract

    The study objective was to characterize and analyze outcomes in patients with tetralogy of Fallot and major aortopulmonary collateral arteries who had undergone surgery elsewhere before referral (prereferral surgery).Patients with tetralogy of Fallot and major aortopulmonary collateral arteries who underwent surgery between 2001 and 2019 at our center were reviewed. Prereferral surgery and unoperated patients were compared, as were subsets of prereferral surgery patients who had undergone different types of prior procedures. Primary outcomes included complete repair with survival to 6 months, death, and perioperative metrics.Of 576 patients studied, 200 (35%) had undergone a wide range and number of prior operations elsewhere, including 92 who had pulmonary blood supply through a shunt and 108 who had a right ventricle pulmonary artery connection. Patients who underwent prereferral surgery with an existing right ventricle pulmonary artery connection had undergone more prereferral surgery procedures than those with a shunt and were more likely to have a right ventricle outflow tract pseudoaneurysm or pulmonary artery stent (all P < .001) at the time of referral. The cumulative incidences of complete repair and death were similar regardless of prereferral surgery status, but the cumulative incidence of complete repair with 6-month survival was higher (P = .002) and of death lower (P = .18) in patients who had prior right ventricle pulmonary artery connection compared with those who had received a prior shunt only.Our comprehensive management strategy for tetralogy of Fallot and major aortopulmonary collateral arteries can be applied with excellent procedural results in both unoperated patients and those who have undergone multiple and varied procedures elsewhere.

    View details for DOI 10.1016/j.jtcvs.2020.03.062

    View details for PubMedID 32444187

  • Outcomes After Aortopulmonary Window for Hypoplastic Pulmonary Arteries and Dual-Supply Collaterals ANNALS OF THORACIC SURGERY Bauser-Heaton, H., Ma, M., McElhinney, D. B., Goodyer, W. R., Zhang, Y., Chan, F. P., Asija, R., Shek, J., Wise-Faberowski, L., Hanley, F. L. 2019; 108 (3): 820–27
  • Assessment of the Reconstructed Pulmonary Circulation With Lung Perfusion Scintigraphy After Unifocalization and Repair of Tetralogy of Fallot With Major Aortopulmonary Collaterals. World journal for pediatric & congenital heart surgery Wise-Faberowski, L., Irvin, M., Lennig, M., Long, J., Nadel, H. R., Bauser-Heaton, H., Asija, R., Hanley, F. L., McElhinney, D. B. 2019; 10 (3): 313–20

    Abstract

    BACKGROUND: Pulmonary vascular supply in tetralogy of Fallot (TOF) with major aortopulmonary collaterals (MAPCAs) is highly variable. Our approach to surgical management of this condition emphasizes early repair including unifocalization and reconstruction of the pulmonary circulation, incorporating all lung segments and addressing stenoses both proximal to and within the lung, in addition to ventricular septal defect closure. At our institution, we have over 15 years of experience using lung perfusion scintigraphy (LPS) to assess the distribution of pulmonary blood flow after complete unifocalization and repair.METHODS: We reviewed clinical and quantitative LPS data in 310 patients who underwent complete unifocalization and repair of TOF/MAPCAs from 2003 to 2018 at our institution. Postrepair relative lung perfusion distributions were determined from LPS initially obtained at our institution within 60 days after repair and thereafter.RESULTS: Total lung perfusion to the right and left lungs was 58.0% ± 14.2% and 42.0% ± 14.2%, respectively. Perfusion was balanced in 75% of patients and unbalanced in 25%, including 11% in whom it was extremely unbalanced. On multivariable analysis, older age at repair, surgery other than a single-stage complete unifocalization, and native anatomy consisting of unilateral pulmonary blood supply through a ductus arteriosus were associated with unbalanced perfusion.CONCLUSION: We present our experience using LPS as an outcome measure after surgical repair of TOF/MAPCAs. Balanced lung perfusion was present in the majority of patients who had complete repair of TOF/MAPCAs performed at our center.

    View details for PubMedID 31084304

  • Tetralogy of Fallot: Everything you wanted to know but were afraid to ask PEDIATRIC ANESTHESIA Wise-Faberowski, L., Asija, R., McElhinney, D. B. 2019; 29 (5): 475–82

    View details for DOI 10.1111/pan.13569

    View details for Web of Science ID 000468217100012

  • Assessment of airway abnormalities in patients with tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collaterals CARDIOLOGY IN THE YOUNG Wise-Faberowski, L., Irvin, M., Sidell, D. R., Rajashekara, S., Asija, R., Chan, F. P., Hanley, F. L., McElhinney, D. B., Pulm Artery Reconstruction Program 2019; 29 (5): 610–14
  • Assessment of the Reconstructed Pulmonary Circulation With Lung Perfusion Scintigraphy After Unifocalization and Repair of Tetralogy of Fallot With Major Aortopulmonary Collaterals WORLD JOURNAL FOR PEDIATRIC AND CONGENITAL HEART SURGERY Wise-Faberowski, L., Irvin, M., Lennig, M., Long, J., Nadel, H. R., Bauser-Heaton, H., Asija, R., Hanley, F. L., McElhinney, D. B. 2019; 10 (3): 313–20
  • Outcomes After Aortopulmonary Window for Hypoplastic Pulmonary Arteries and Dual-Supply Collaterals. The Annals of thoracic surgery Bauser-Heaton, H., Ma, M., McElhinney, D. B., Goodyer, W. R., Zhang, Y., Chan, F. P., Asija, R., Shek, J., Wise-Faberowski, L., Hanley, F. L. 2019

    Abstract

    BACKGROUND: Our institutional approach to tetralogy of Fallot (TOF) with major aortopulmonary collateral arteries (MAPCAs) emphasizes early unifocalization and complete repair (CR). In the small subset of patients with dual-supply MAPCAs and confluent but hypoplastic central pulmonary arteries (PAs), our surgical approach is early creation of an aortopulmonary window (APW) to promote PA growth. Factors associated with successful progression to CR, and mid-term outcomes have not been assessed.METHODS: Clinical data were reviewed. PA diameters were measured off-line from angiograms prior to APW and on follow-up catheterization >1 month after APW but prior to any additional surgical interventions.RESULTS: From 11/01-3/18, 352 patients with TOF/MAPCAs underwent initial surgery at our center, 40 of whom had a simple APW with or without ligation of MAPCAs as the first procedure (median age 1.4 months). All PA diameters increased significantly on follow-up angiography. Ultimately, 35 patients underwent CR after APW. Nine of these patients (26%) underwent intermediate palliative operation between 5 and 39 months (median 8 months). There were no early deaths. The cumulative incidence of CR was 65% 1 year post-APW and 87% at 3 years. Repaired patients were followed for a median of 4.2 years after repair; the median PA:aortic pressure ratio was 0.39 (0.22-0.74).CONCLUSIONS: Most patients with TOF/MAPCAs and hypoplastic but normally arborizing PAs and dual-supply MAPCAs are able to undergo CR with low right ventricular pressure after APW early in life. Long-term outcomes were good, with acceptable PA pressures in most patients.

    View details for PubMedID 30980823

  • Outcomes After Initial Unifocalization to a Shunt in Complex Tetralogy of Fallot with MAPCAs. The Annals of thoracic surgery Bauser-Heaton, H. n., Ma, M. n., Wise-Faberowski, L. n., Asija, R. n., Shek, J. n., Zhang, Y. n., Peng, L. F., Sidell, D. R., Hanley, F. L., McElhinney, D. B. 2019

    Abstract

    Our approach to tetralogy of Fallot (TOF) with pulmonary atresia and major aortopulmonary collateral arteries (MAPCAs) emphasizes early single-stage unifocalization and intracardiac repair. However, a subset of patients with small native pulmonary arteries (PAs) and MAPCAs undergo unifocalization to a shunt rather than simultaneous intracardiac repair.Patients with TOF/MAPCAs who underwent unifocalization to a systemic-to-PA shunt by a single surgeon were reviewed. The decision to perform simultaneous intracardiac repair was based on an intraoperative flow study or empirical assessment of PA and MAPCA size.From 11/01-12/17, 57 patients with TOF/MAPCAs underwent unifocalization to a shunt at a median age of 6.9 months. Genetic abnormalities were documented in 60% of patients, including a chromosome 22q11 deletion in 25 and Alagille syndrome in 8. Twenty patients (35%) had undergone prior surgery elsewhere (n=16) and/or at our center (n=7). During a median follow-up of 5.4 years, 9 patients had additional surgery to revise the PA reconstruction before complete repair, and 38 patients underwent complete repair. Survival was 74±6% 5 years after unifocalization. At follow-up, the median PA:aortic systolic pressure ratio was 0.36 and was >0.50 in 2 patients.In patients with the smallest MAPCAs and PAs, single-stage unifocalization to a shunt followed by intracardiac repair yields an excellent outcome in most cases.

    View details for DOI 10.1016/j.athoracsur.2019.01.030

    View details for PubMedID 30772338

  • Assessment of airway abnormalities in patients with tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collaterals. Cardiology in the young Wise-Faberowski, L. n., Irvin, M. n., Sidell, D. R., Rajashekara, S. n., Asija, R. n., Chan, F. P., Hanley, F. L., McElhinney, D. B. 2019: 1–5

    Abstract

    Children with tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collaterals (TOF/MAPCAs) are at risk for post-operative respiratory complications after undergoing unifocalisation surgery. Thus, we assessed and further defined the incidence of airway abnormalities in our series of over 500 children with TOF/MAPCAs as determined by direct laryngoscopy, chest computed tomography (CT), and/or bronchoscopy.The medical records of all patients with TOF/MAPCAs who underwent unifocalisation or pulmonary artery reconstruction surgery from March, 2002 to June, 2018 were reviewed. Anaesthesia records, peri-operative bronchoscopy, and/or chest CT reports were reviewed to assess for diagnoses of abnormal or difficult airway. Associations between chromosomal anomalies and airway abnormalities - difficult anaesthetic airway, bronchoscopy, and/or CT findings - were defined.Of the 564 patients with TOF/MAPCAs who underwent unifocalisation or pulmonary artery reconstruction surgery at our institution, 211 (37%) had a documented chromosome 22q11 microdeletion and 28 (5%) had a difficult airway/intubation reported at the time of surgery. Chest CT and/or peri-operative bronchoscopy were performed in 234 (41%) of these patients. Abnormalities related to malacia or compression were common. In total 35 patients had both CT and bronchoscopy within 3 months of each other, with concordant findings in 32 (91%) and partially concordant findings in the other 3.This is the largest series of detailed airway findings (direct laryngoscopy, CT, and bronchoscopy) in TOF/MAPCAS patients. Although these findings are specific to an at-risk population for airway abnormalities, they support the utility of CT and /or bronchoscopy in detecting airway abnormalities in patients with TOF/MAPCAs.

    View details for PubMedID 31044684

  • Deletion of 22q11 chromosome is associated with postoperative morbidity after unifocalisation surgery CARDIOLOGY IN THE YOUNG Koth, A., Sidell, D., Bauser-Heaton, H., Wise-Faberowski, L., Hanley, F. L., McElhinney, D. B., Asija, R. 2019; 29 (1): 19–22
  • Tetralogy of Fallot: Everything You Wanted to Know butWere Afraid to Ask. Paediatric anaesthesia Wise-Faberowski, L., Asija, R., McElhinney, D. B. 2018

    Abstract

    Tetralogy of Fallot (TOF) is the most common form of congenital cyanotic heart disease, occurring in approximately 4 to 5 per 10,0000 live births, and represents 7-10% of all congenital heart defects (1,2). Although TOF is often thought of in terms of the tetrad of anomalies-pulmonary stenosis, ventricular septal defect (VSD), aorta overriding the ventricular septum, and right ventricular hypertrophy-it has been proposed that all of these features are the result of anterior malalignment of the infundibular septum with the muscular septum (3,4). There are several variants of TOF as well, which manifest other abnormal features and are discussed in the following review. This article is protected by copyright. All rights reserved.

    View details for PubMedID 30592107

  • Repair of Untreated Older Patients with Tetralogy of Fallot with Major Aortopulmonary Collaterals. The Annals of thoracic surgery Vaikunth, S., Bauser-Heaton, H., Lui, G. K., Wise-Faberowski, L., Chan, F. P., Asija, R., Hanley, F. L., McElhinney, D. B. 2018

    Abstract

    BACKGROUND: Our programmatic approach to tetralogy of Fallot with major aortopulmonary collaterals emphasizes single-stage unifocalization with complete intracardiac repair during infancy. Little is known about suitability for complete repair in patients beyond infancy. We sought to analyze outcomes of our approach in older patients with previously untreated tetralogy of Fallot with major aortopulmonary collaterals.METHODS: Any patient with this lesion not treated prior to 2 years of age referred to our center from 2002-17 met inclusion criteria.RESULTS: Of 33 patients, 32 were out-of-state (64% international) referrals, and 33% (n=11) were >9 years old, had polycythemia, and/or at least 1 high pressure collateral (>25 mm Hg). Complete repair was achieved in 94% (n=31) of patients, 82% (n=27) in one stage and 12% (n=4) after unifocalization to a central shunt. The median right ventricular to aortic pressure ratio was 0.31 after surgery and 0.37 at follow-up. At a median of 4.8 years after repair, 9 patients (19%) underwent reintervention, including 5 conduit and 7 branch pulmonary artery interventions. Three patients also underwent aortic valve replacement.CONCLUSIONS: In this selected cohort of older patients with previously unoperated tetralogy of Fallot with major aortopulmonary collaterals, outcomes were comparable to infants undergoing treatment according to our approach. These findings support the notion that patients who are either born in low-resource settings or present to healthcare providers beyond infancy should be considered candidates and evaluated for complete repair.

    View details for PubMedID 30550802

  • Deletion of 22q11 chromosome is associated with postoperative morbidity after unifocalisation surgery - CORRIGENDUM. Cardiology in the young Koth, A., Sidell, D., Bauser-Heaton, H., Wise-Faberowski, L., Hanley, F. L., McElhinney, D. B., Asija, R. 2018: 1

    View details for DOI 10.1017/S1047951118001804

    View details for PubMedID 30370896

  • Deletion of 22q11 chromosome is associated with postoperative morbidity after unifocalisation surgery. Cardiology in the young Koth, A., Sidell, D., Bauser-Heaton, H., Wise-Faberowski, L., Hanley, F. L., McElhinney, D. B., Asija, R. 2018: 1–4

    Abstract

    BACKGROUND: A 22q11 chromosome deletion is common in patients with tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collaterals. We sought to determine whether 22q11 chromosome deletion is associated with increased postoperative morbidity after unifocalisation surgery.METHODS: We included all patients with this diagnosis undergoing primary or revision unifocalisation ± ventricular septal defect closure at our institution from 2008 to 2016, and we excluded patients with unknown 22q11 status. Demographic and surgical data were collected. We compared outcomes between those with 22q11 chromosome deletion and those without using non-parametric analysis.RESULTS: We included 180 patients, 41% of whom were documented to have a chromosome 22q11 deletion. Complete unifocalisation was performed in all patients, and intracardiac repair was performed with similar frequency regardless of 22q11 chromosome status. Duration of mechanical ventilation was longer in 22q11 deletion patients. This difference remained significant after adjustment for delayed sternal closure and/or intracardiac repair. Duration of ICU stay was longer in patients with 22q11 deletion, although no longer significant when adjusted for delayed sternal closure and intracardiac repair. Finally, length of hospital stay was longer in 22q11-deleted patients, but this difference was not significant on unadjusted or adjusted analysis.CONCLUSION: Children with tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collaterals and 22q11 deletion are at risk for greater prolonged mechanical ventilation after unifocalisation surgery. Careful attention should be given to the co-morbidities of this population in the perioperative period to mitigate risks that may complicate the postoperative course.

    View details for PubMedID 30160647

  • Postoperative feeding problems in patients with tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collaterals undergoing unifocalisation surgery. Cardiology in the young Koth, A. M., Sakarovitch, C., Sidell, D. R., Schultz, L. M., Freccero, A., Rizzuto, S., Hanley, F. L., Asija, R. 2018: 1–4

    Abstract

    BACKGROUND: Patients with tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collaterals are at risk for prolonged hospitalisation after unifocalisation. Feeding problems after congenital heart surgery are associated with longer hospital stay. We sought to determine the impact of baseline, intra-operative, and postoperative factors on the need for feeding tube use at the time of discharge.METHODS: We included patients with the aforementioned diagnosis undergoing unifocalisation from ages 3 months to 4 years from 2010 to 2016. We excluded patients with a pre-existing feeding tube. Patients discharged with an enteric tube were included in the feeding tube group. We compared the feeding tube group with the non-feeding-tube group by univariable and multi-variable logistic regression.RESULTS: Of the 56 patients studied, 41% used tube feeding. Median age and weight z-score were similar in the two groups. A chromosome 22q11 deletion was associated with the need for a feeding tube (22q11 deletion in 39% versus 15%, p=0.05). Median cardiopulmonary bypass time in the feeding tube group was longer (335 versus 244 minutes, p=0.04). Prolonged duration of mechanical ventilation was associated with feeding tube use (48 versus 3%, p=0.001). On multi-variable analysis, prolonged mechanical ventilation was associated with feeding tube use (odds ratio 10.2, 95% confidence intervals 1.6; 63.8).CONCLUSION: Among patients with tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collaterals who were feeding by mouth before surgery, prolonged mechanical ventilation after unifocalisation surgery was associated with feeding tube use at discharge. Anticipation of feeding problems in this population and earlier feeding tube placement may reduce hospital length of stay.

    View details for DOI 10.1017/S1047951118001270

    View details for PubMedID 30070195

  • Association of dead space ventilation and prolonged ventilation after repair of tetralogy of Fallot with pulmonary atresia. The Journal of thoracic and cardiovascular surgery Koth, A. M., Kwiatkowski, D. M., Lim, T. R., Bauser-Heaton, H., Asija, R., McElhinney, D. B., Hanley, F. L., Krawczeski, C. D. 2018

    Abstract

    BACKGROUND: We set out to determine whether patients with tetralogy of Fallot with pulmonary atresia and major aortopulmonary collateral arteries (TOF/PA/MAPCA) are at risk for elevated dead space ventilation fraction (VD/VT), and whether this is associated with prolonged mechanical ventilation. We hypothesized that elevated VD/VT (>20%) in the first 24hours after unifocalization surgery is associated with increased risk for prolonged mechanical ventilation (>7days).METHODS: All patients with TOF/PA/MAPCA undergoing unifocalization surgery between January 2003 and December 2015 were included in this study. Average VD/VT was calculated over the first 24hours after surgery. Demographic and surgical data were collected. Outcome data included duration of mechanical ventilation. Patients were separated into 2 groups: elevated VD/VT and normal DVSF. Groups were compared using the Student t test, Wilcoxon rank-sum test, and chi2 test. Univariable and multivariable regression analyses were performed with VD/VT as a continuous variable to test for association.RESULTS: Of the 265 included patients, 127 (48%) had an elevated VD/VT. The 2 groups did not differ significantly in any demographic characteristic. Patients with an elevated VD/VT had longer cardiopulmonary bypass times (P=.03), were more likely to have delayed sternal closure, and more likely to have prolonged respiratory failure (odds ratio, 2.2; 95% confidence interval, 1.2-4.0; P=.007). The percent VD/VT was associated with duration of mechanical ventilation in univariable (P<.001) and multivariable (P<.001) regression analyses when controlled for age, weight and bypass time.CONCLUSIONS: Elevated postoperative VD/VT is associated with prolonged mechanical ventilation in patients with TOF/PA/MAPCA following unifocalization. Elevated postoperative VD/VT may be an early indicator of patients who will require prolonged duration of mechanical ventilation, allowing optimization of medical management to promote better outcomes.

    View details for PubMedID 29884495

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

    Abstract

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

    View details for PubMedID 29352588

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

    Abstract

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

    View details for PubMedID 29544416

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

    Abstract

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

    View details for DOI 10.1002/ppul.23732

    View details for PubMedID 28504356

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

    Abstract

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

    View details for DOI 10.1161/CIRCINTERVENTIONS.116.004952

    View details for PubMedID 28356265

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

    Abstract

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

    View details for DOI 10.1016/j.athoracsur.2015.12.049

    View details for PubMedID 26947013

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

    Abstract

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

    View details for DOI 10.1016/j.jtcvs.2014.01.017

    View details for Web of Science ID 000342896200077

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

    Abstract

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

    View details for DOI 10.1016/j.jtcvs.2014.01.017

    View details for PubMedID 24534681

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

    Abstract

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

    View details for DOI 10.1097/PCC.0b013e318272062b

    View details for PubMedID 23439458

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

    Abstract

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

    View details for DOI 10.1097/PCC.0b013e3181e8b3e5

    View details for PubMedID 20693934