BACKGROUND: Challenges exist with heterotaxy due to the complexity of heart disease, abnormal venous connections, and infection risks. This study aims to understand heart transplant outcomes for children with heterotaxy. METHODS: All children with congenital heart disease listed for transplant from 1993 to 2018 were included. Those with and without heterotaxy were compared. Waitlist outcomes and survival post-listing and transplant were analyzed. Post-transplant risk factors were identified using multiphase parametric hazard modeling. RESULTS: There were 4814 children listed, of whom 196 (4%) had heterotaxy. Heterotaxy candidates were older (5.8 +/- 5.7 vs 4.2 +/- 5.5 years, p < 0.01), listed at a lower urgency status (29.8% vs 18.4%, p < 0.01), more commonly single ventricle physiology (71.3% vs 59.2%, p < 0.01), and less often supported by mechanical ventilation (22% vs 29.1%, p < 0.05) or extracorporeal membrane oxygenation (3.6% vs 7.5%, p < 0.05). There were no differences in waitlist outcomes of transplant, death, or removal. Overall, post-transplant survival was worse for children with heterotaxy: one-year survival 77.2% vs 85.1%, with and without heterotaxy, respectively. Heterotaxy was an independent predictor for early mortality in the earliest era (1993-2004), HR 2.09, CI 1.16-3.75, p = 0.014. When stratified by era, survival improved with time. Heterotaxy patients had a lower freedom from infection and from severe rejection, but no difference in vasculopathy or malignancy. CONCLUSIONS: Mortality risk associated with heterotaxy is mitigated in the recent transplant era. Early referral may improve waitlist outcomes for heterotaxy patients who otherwise have a lower status at listing. Lower freedom from both infection and severe rejection after transplant in heterotaxy highlights the challenges of balancing immune suppression. (C) 2021 International Society for Heart and Lung Transplantation. All rights reserved.

AIM: To describe the clinical and hemodynamic characteristics of Fontan failure in children listed for heart transplant. METHODS: In a nested study of the Pediatric Heart Transplant Society, 16 centers contributed information on Fontan patients listed for heart transplant between 2005and 2013. Patients were classified into four mutually exclusive phenotypes: Fontan with abnormal lymphatics (FAL), Fontan with reduced systolic function (FRF), Fontan with preserved systolic function (FPF), and Fontan with "normal" hearts (FNH). Primary outcome was waitlist and post-transplant mortality. RESULTS: 177 children listed for transplant were followed over a median 13 (IQR 4-31) months, 84 (47%) were FAL, 57 (32%) FRF, 22 (12%) FNH, and 14 (8%) FPF. Hemodynamic characteristics differed between the 4 groups: Fontan pressure (FP) was most elevated with FPF (median 22, IQR 18-23, mmHg) and lowest with FAL (16, 14-20, mmHg); cardiac index (CI) was lowest with FRF (2.8, 2.3-3.4, L/min/m(2)). In the entire cohort, 66% had FP >15 mmHg, 21% had FP >20 mmHg, and 10% had CI <2.2 L/min/m(2). FRF had the highest risk of waitlist mortality (21%) and FNH had the highest risk of post-transplant mortality (36%). CONCLUSIONS: Elevated Fontan pressure is more common than low cardiac output in pediatric failing Fontan patients listed for transplant. Subtle hemodynamic differences exist between the various pheno types of pediatric Fontan failure. Waitlist and post-transplant mortality risks differ by phenotype. (C) 2021 International Society for Heart and Lung Transplantation. All rights reserved.

With an increasing number of pediatric heart transplant patients, it is inevitable that they will seek medical care at an emergency department for cardiac or noncardiac complaints. Given the special issues associated with transplant (some of which are similar to other organ transplants and some of which are unique to cardiac transplantation), the emergency care provider should have an understanding of what care can be independently provided, concerns which may require additional testing or expertise, and when to call the cardiac consultant. The goal of this review is to address these issues.

Introduction: Heart-lung transplantation (HLT) may be indicated in adult congenital heart disease patients (ACHD) with or without Eisenmenger syndrome (ES) with end-stage heart and lung disease. Pr...

Pediatric heart transplantations are limited by the supply of donor allografts. We sought to determine the cardiac allograft utilization rate for pediatric donors and identify donor factors that predict graft use for transplantation. The United Network for Organ Sharing deceased donor database was queried from April 30, 2006, to March 31, 2014. Donor risk factors that might affect graft use for cardiac transplantation were evaluated. The pediatric cardiac graft utilization rate was calculated, and logistic regression modeling was performed to determine the relationship of risk factors with graft use for transplantation. During the study period, 6682 eligible cardiac donors <18 years of age were identified, and 3758 (56.2%) grafts were utilized for transplantation. Grafts from male donors (OR 1.181) were significantly associated with graft utilization. Graft donor age >1 year (OR 0.363), non-O blood type (OR 0.586), CDC 'high-risk' donor status (OR 0.676), use of inotropes (OR 0.718), use of >2 inotropes (OR 0.328), and donor left ventricular ejection fraction <50% (OR 0.045) were significantly associated with graft nonutilization. The pediatric cardiac allograft utilization rate and risk factors for graft use for transplantation have been identified. Additional studies will be needed to assess the donor-recipient relationship on pediatric transplant outcomes.