Mortality and morbidity after combined heart and liver transplantation in the failing Fontan: An updated dual center retrospective study.

INTRODUCTION: As the adult Fontan population with Fontan associated liver disease continues to increase, more patients are being referred for transplantation, including combined heart and liver transplantation. METHODS: We report updated mortality and morbidity outcomes after combined heart and liver transplant in a retrospective cohort series of 40 patients (age 14 to 49 years) with Fontan circulation across two centers from 2006-2022. RESULTS: The 30-day, 1-year, 5-year and 10-year survival rate was 90%, 80%, 73% and 73% respectively. Sixty percent of patients met a composite comorbidity of needing either post-transplant mechanical circulatory support, renal replacement therapy or tracheostomy. Cardiopulmonary bypass time > 283 min (4.7 h) and meeting the composite comorbidity were associated with mortality by Kaplan Meier analysis. CONCLUSION: Further study to mitigate early mortality and the above comorbidities as well as the high risk of bleeding and vasoplegia in this patient population is warranted.

An integrated program to expand donor utilization in pediatric heart transplantation: Case report of successful transplant with multiple donor risk factors.

BACKGROUND: Pediatric heart transplantation (HT) continues to be limited by the shortage of donor organs, distance constraints, and the number of potential donor offers that are declined due to the presence of multiple risk factors. METHODS: We report a case of successful pediatric HT in which multiple risk factors were mitigated through a combination of innovative donor utilization improvement strategies. RESULTS: An 11-year-old, 25-kilogram child with cardiomyopathy and pulmonary hypertension, on chronic milrinone therapy and anticoagulated with apixaban, was transplanted with a heart from a Hepatitis C virus positive donor and an increased donor-to-recipient weight ratio. Due to extended geographic distance, an extracorporeal heart preservation system (TransMedics™ OCS Heart) was used for procurement. No significant bleeding was observed post-operatively, and she was discharged by post-operative day 15 with normal biventricular systolic function. Post-transplant Hepatitis C virus seroconversion was successfully treated. CONCLUSIONS: Heart transplantation in donors with multiple risk factor can be achieved with an integrative team approach and should be taken into consideration when evaluating marginal donors in order to expand the current limited donor pool in pediatric patients.

Weight Matching in Infant Heart Transplantation: A National Registry Analysis.

BACKGROUND: Infants account for a significant proportion of pediatric heart transplantation but also suffer from a high waitlist mortality. Donor oversizing by weight-based criteria is common practice in transplantation and is prevalent in this group. We sought to analyze the impact of oversizing on outcomes in infants. METHODS: Infant heart transplantations reported to the United Network for Organ Sharing from January 1994 to September 2019 were retrospectively analyzed. 2384 heart transplantation recipients were divided into quintiles (Q1-Q5) on the basis of donor-to-recipient weight ratio (DRWR). Multivariate Cox regression was used to estimate the effect of DRWR. The primary end point was graft survival at 1 year. RESULTS: The median DRWR for each quintile was 0.90 (0.37-1.04), 1.17 (1.04-1.29), 1.43 (1.29-1.57), 1.74 (1.58-1.97), and 2.28 (1.97-5.00). Pairwise comparisons showed improved survival for Q3 and Q4 over each of the bottom 2 quintiles and the top quintile. Regression analyses found that Q3 and Q4 were protective against graft failure compared with the bottom 2 quintiles. There was no difference in hazard among the top 3 quintiles. Significant covariates included primary diagnosis, ischemia time, serum bilirubin level, transplantation year, mechanical ventilation at transplantation, and extracorporeal membrane oxygenation at transplantation. Sex, female-to-male transplantation, and mechanical circulatory support at transplantation were not significant in univariate analyses. CONCLUSIONS: Modest oversizing by DRWR (1.29-1.97) is associated with increased survival and lower risk in infant heart transplantation. Additional investigation is needed to establish best practices for size matching in this population.

Complete repair for Scimitar syndrome with transitional atrioventricular canal.

We describe an extremely rare case of a 15-year-old female, who underwent a complete repair for transitional atrioventricular canal with Scimitar syndrome by a two-patch technique including translocation of the Scimitar vein. This surgical technique offered superior patch shapes in order to better repair both anomalies.

Neonatal right mini thoracotomy for repair of critical pulmonary stenosis.

Over the last few decades, due to evolving surgical techniques and medical management, there has been a significant decrease in the rate of mortality and complications for neonates born with critical pulmonary valve stenosis. Median sternotomy is the standard approach; however, this longitudinal midline incision is invasive and leaves a significant scar. A right mini thoracotomy approach to this surgical repair decreases recovery time and the chance of possible future psychological distress from a visible median sternotomy scar. This is the first article to describe a right mini thoracotomy approach for critical pulmonary stenosis during the neonatal period.

Innominate artery patency after direct cannulation in neonates.

Objective: The study objective was to determine the short-term incidence of innominate artery stenosis for neonates who underwent direct innominate artery cannulation during the Norwood procedure. Methods: This is a retrospective, single-institution review of 92 patients who underwent the Norwood procedure with direct innominate artery cannulation from 2006 to 2017. The primary outcome was angiographic evidence of patency at pre-Glenn cardiac catheterization. Patient characteristics, intraoperative surgical and hemodynamic measurements, and postoperative neurologic findings were recorded. Results: At a median age of 5.0 days, 92 neonates underwent the Norwood procedure with direct innominate artery cannulation. These patients underwent cardiac catheterization at a median of 3.0 months after the index operation. In 5 of 92 patients with catheterization images available for review, there was angiographic evidence of mild innominate artery stenosis, and none had moderate or severe stenosis. Review of follow-up records did not reveal evidence of clinically significant stenosis or innominate artery reintervention. Conclusions: In neonates undergoing the Norwood procedure with direct innominate cannulation, innominate artery stenosis was uncommon and clinically significant stenosis did not occur.

Evaluating predicted heart mass in adolescent heart transplantation.

BACKGROUND: Predicted Heart Mass (PHM) has emerged as an attractive size matching metric in adult cardiac transplantation. However, since PHM was derived from a healthy adult cohort, its generalizability to the pediatric population is unclear. We hypothesize that PHM can be extended to older adolescents, and potentially broaden the donor pool available to this group. METHODS: The United Network for Organ Sharing database was retrospectively analyzed for patients aged 13 to 18 undergoing heart transplantation. Recipients were divided into quintiles (Q1-Q5) based on donor-to-recipient predicted heart mass ratios (PHMR). Primary end-point was graft survival at 5 years. RESULTS: Two thousand sixty-one adolescent heart transplant recipients between January 1994 and September 2019 were retrospectively analyzed. The median PHMR's for each quintile was 0.84 (0.59-0.92), 0.97 (0.92-1.02), 1.08 (1.02-1.14), 1.21 (1.14-1.30), and 1.44 (1.30-2.31). Kaplan-Meier survival curves demonstrated comparable survival across all quintiles of PHMR (p = 0.9). Multivariate Cox regression showed no significant difference in graft failure of the outer quintiles when compared to the middle quintile (Q1: 1.04 HR, p = 0.80; Q2: 1.02 HR, p = 0.89; Q4: 1.19 HR, p = 0.28; Q5: 1.02 HR, p = 0.89). Significant covariates included transplant year (HR: 0.95, p < 0.0001), serum bilirubin (HR: 1.04, p = 0.0004), ECMO at transplantation (HR: 2.85, p < 0.0001), and underlying diagnosis of dilated cardiomyopathy (vs congenital heart disease, HR: 0.66, p = 0.0004). CONCLUSIONS: Matching by PHM is not associated with survival or risk in adolescent heart transplant recipients. Our results underscore the ongoing need to develop an improved size-matching method in pediatric heart transplantation.

Single Ventricular Assist Device Support for the Failing Bidirectional Glenn Patient.

BACKGROUND: Given poor outcomes, strategies to improve ventricular assist devices (VADs) for single-ventricle patients with bidirectional Glenn (BDG) palliation are needed. METHODS: This retrospective review describes an institutional experience with VAD support for patients with BDG from April 2011 to January 2019. Surgical strategies, complications, and causes of death are described. Survival to heart transplantation for various strategies are compared. RESULTS: A total of 7 patients with BDG (weights, 5.6 to 28.8 kg; ages, 7 months to 11 years) underwent VAD implantation. Three patients underwent implantation of Berlin Heart EXCOR devices (Berlin Heart, Inc, Spring, TX), 2 had HeartWare HVADs (Medtronic, Minneapolis, MN) implanted, and 2 patients underwent implantation of paracorporeal continuous flow devices. Four patients underwent ventricular inflow cannulation, and 3 underwent atrial inflow cannulation. At the time of VAD implantation, the BDG was left intact in 3 patients, taken down in 3 patients, and created de novo in 1 patient. Over a total of 420 VAD support days, 2 patients survived to heart transplantation, 1 patient with HeartWare ventricular cannulation and intact BDG (after 174 days) and another with Berlin Heart atrial cannulation and BDG take-down (after 72 days). There were 3 deaths within 2 weeks of VAD implantation (2 from respiratory failure, 1 from infection) and 2 deaths after 30 days as a result of strokes. CONCLUSIONS: The surgical strategy and postoperative management of VAD with BDG are still evolving. Successful support can be achieved with (1) both pulsatile and continuous flow pumps, (2) atrial or ventricular cannulation, and (3) with or without BDG take-down. Surgical strategy should be determined by individual patient anatomy, physiology, and condition.

Aortic or Pulmonary Valved Homograft Right Ventricle to Pulmonary Artery Conduit in the Norwood Procedure.

BACKGROUND: Several modifications of the Norwood procedure utilizing valved right ventricle to pulmonary artery conduits have recently been reported. Our group has been using aortic or pulmonary valved homografts combined with PTFE tube grafts for now 16 years. METHODS: In this report, we review our technique in detail and describe any changes that have occurred over the years. We provide detailed illustrations of our preferred surgical technique, report outcome data, and compare it to the other conduit options available. RESULTS: Between 2006 and 2015, 130 stage I Norwood procedures were performed at our institution, 100 of them using valved conduits. Our technique is described and illustrated in detail. Early mortality was 15%. Postoperative percutaneous intervention on the conduit was required in 29% of cases. CONCLUSIONS: While a randomized trial comparing different valved conduits is lacking, we believe a composite conduit made from homograft aortic or pulmonary valves and PTFE tube grafts is an excellent choice in stage I Norwood procedure.