210 - Outcomes Following Transplant in Failed Fontan Patients: A Single Center Study

For children born with single ventricle physiology, the Fontan Procedure is a palliative, although not curative treatment option. While transplant-free survival has improved over time, 10% of Fontan patients require a heart transplant by early adulthood. The various reasons for Fontan failure are well documented, however, it is difficult to predict the ideal timing to initiate transplant listing. Defining ideal transplant candidates through specific parameters would improve the management of this patient population across the lifespan.

Objective:

The main objective of this study was to describe a cohort of patients that underwent the Fontan procedure but required a transplant in the pediatric period and to identify pre-transplant characteristics that correlate to better post-transplant outcomes. We hypothesize that older patients with fewer complications will have a higher survival rate at 5 and 10 years.

Design/Methods:

A retrospective review was conducting using records of 93 patients enrolled in the Pediatric Heart Transplant Study and listed for transplant for single ventricle physiology at Columbia University Medical Center from 1993 to 2020. Pre-transplant characteristics, complications, and outcome data were collected. Statistical analysis was performed using R.

Results:

There were 56 patients who received a transplant after the Fontan procedure (62.5% male). Forty seven percent of Fontan patients had developed protein losing enteropathy (PLE) at time of transplant. Patients with PLE were older on average (12.7 ± 4.35 years vs. 9.81 ± 4.85 years, p = 0.024). Kaplan-Meier analysis of survival over 5 and 10 years showed that patients without PLE prior to transplantation had greater survival (5 years HR=2.217, p = 0.04; 10 years HR=2.603, p = 0.014). There were no differences between the Fontan and the Glenn groups  in any other secondary outcomes including hospital length of stay, time on ventilator, cardiopulmonary bypass time or cold ischemic time.

Conclusion(s): In this single center retrospective study, Fontan patients with PLE generally were transplanted later than those without and had greater mortality at 5 and 10 years. This finding was not appreciated in prior retrospective studies and makes an argument that Fontan patients should be transplanted before PLE develops or as soon as it is diagnosed.