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Outcomes of Liver Transplantation in Patients With Congenital Heart Disease and Biliary Atresia. A Multicenter
Mario O'Connor1,2,3, Joel T Adler4, Neil M Venardos1
1Department of Surgery and Perioperative Care, Dell Medical School at the University of Texas, Austin, Austin, TX, USA.
Insights
Liver transplantation (LT) for biliary atresia (BA) in patients with congenital heart disease (CHD) is safe. While these patients use more hospital resources, long-term outcomes are similar to those without CHD.
Area of Science:
- Pediatric Hepatology
- Congenital Cardiology
- Transplant Surgery
Background:
- Congenital heart disease (CHD) often co-occurs with noncardiac malformations like biliary atresia (BA).
- Liver transplantation (LT) outcomes in pediatric patients with both CHD and BA are not well-established.
Purpose of the Study:
- To evaluate the safety and outcomes of liver transplantation (LT) in pediatric patients diagnosed with biliary atresia (BA) and coexisting congenital heart disease (CHD).
Main Methods:
- Retrospective review of the Pediatric Health Information System (PHIS) database (2004-2023).
- Included patients undergoing LT for BA, excluding multi-organ transplants.
- Identified patients with CHD using ICD-9/10 codes.
Main Results:
- 83 (5%) of 1677 LT recipients with BA had CHD.
- Patients with CHD had significantly longer pre-operative and post-operative lengths of stay (LOS).
- No increased risk of in-hospital mortality, 30-day readmission, or retransplantation was observed in the CHD group.
Conclusions:
- Liver transplantation (LT) for biliary atresia (BA) is a safe procedure for patients with congenital heart disease (CHD).
- While CHD patients utilize more hospital resources, long-term outcomes are comparable to non-CHD patients.
- Further research is needed to fully understand CHD's impact on LT management and outcomes.
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.
