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

INTRODUCTION

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.

METHODS

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.

RESULTS

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.

CONCLUSIONS

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.