# Outcomes of Liver Transplantation in Patients With Congenital Heart Disease and Biliary Atresia. A Multicenter Analysis

## TL;DR

Liver transplants in children with heart defects and biliary atresia are safe, though they require more hospital resources.

## Contribution

This study is the first to analyze liver transplant outcomes in patients with congenital heart disease and biliary atresia using a large multicenter database.

## Key findings

- Patients with congenital heart disease had longer hospital stays before and after liver transplantation.
- No increased risk of in-hospital mortality or long-term retransplantation was found in patients with congenital heart disease.
- Despite higher resource use, long-term outcomes were similar between patients with and without congenital heart disease.

## Abstract

Congenital heart disease (CHD) frequently coexists with noncardiac malformations. Among which, biliary atresia (BA) occurs in approximately 5%–15% of patients Despite this, outcomes of liver transplantation (LT) in patients with CHD and BA remain unknown.

A retrospective review of the Pediatric Health Information System (PHIS) database from January 1, 2004 to October 30, 2023. All patients who underwent LT and had a diagnosis of BA were included. Multiorgan transplants were excluded. International Classification of Diseases 9th and 10th editions were utilized to identify patients with a diagnosis consistent with CHD.

A total of 1677 patients were identified with 983 (59%) female, 811 (5%) white non‐Hispanic, and a median age at transplant of 11.6 (interquartile range [IQR]: 7.7–26.6) months. A CHD diagnosis was present in 83 (5%). Overall, the majority of transplants were performed in the CHD population in the modern era (2016–2023) (42/83.51%). CHD had a longer median preoperative length of stay (LOS) compared to non‐CHD (1 [1.0–15.0] vs. 1 [0–6.0], p = 0.031). No increased risk of in‐hospital mortality was evident (OR: 1.61, 95% CI: 0.37–6.95, p = 0.519). On multivariable analysis, CHD was associated with a 29% (95% CI: 11.04–50.34) increase in LOS (p < 0.001), 25% (95% CI: 6.98–46.47, p = 0.005) increase in postoperative LOS, and was not associated with increased risk for 30‐day readmission (OR: 1.14; CI: 0.53–2.45, p = 0.736). Over a median follow‐up of 3.5 years (IQR: 0.86–7.78) years, no difference in retransplantation rate was evident.

LT in patients with CHD and BA is safe. Although patients with CHD display heightened utilization of in‐hospital resources, no discernible variance in long‐term outcomes was observed. However, additional research is imperative to comprehensively elucidate the influence of CHD on management decisions and outcomes throughout the liver transplant process.

Liver transplantation in patients with CHD and BA is safe. Although patients with CHD display heightened utilization of in‐hospital resources, no discernible variance in long‐term outcomes was observed.