Title: Outcomes of Kasai portoenterostomy in patients with congenital heart disease: a silent comorbidity
Source: Journal of Pediatric Surgery 2025, Mar 18. [E–publication]
Date of publication: March 2025
Publication type: Retrospective review
Abstract: Introduction: Biliary atresia (BA) is the most common cause of end-stage liver disease in children. Around 5%-15% of patients with BA have a congenital heart disease (CHD) diagnosis. Despite high prevalence of a CHD diagnosis in patients with BA, outcomes of Kasai portoenterostomy (KPE) in the CHD population remain unexplored METHODS: This is a retrospective review of the American College of Surgeons National Surgical Quality Improvement Program-Pediatric(NSQIP-P) database from 2012-2022. All patients undergoing KPE were included. Patients with CHD were identified using previously validated ICD9/10 codes.
Results: A total of 854 patients underwent a KPE during the study period. Of this cohort, 502(59%) were female, 342(40%) White Non-Hispanic, median age of 57.0[IQR:40.0-72.0] days, and a median weight of 9.4[IQR:8.1-10.7] kilograms. A total of 89(10%) patients had a diagnosis of CHD. Of those, 63(7%) had a simple-CHD diagnosis and 26(3%) a complex-CHD diagnosis. A single-ventricle(SV-CHD) diagnosis was present in 7(1%) patients. Notably, no in-hospital mortalities were found. When compared to non-CHD, simple-CHD(OR:2.51;95%CI:1.45-4.36,p=0.001) and complex-CHD(OR:3.20;95%CI:1.30-7.87,p=0.011) had increased risk for any complication. Furthermore, when compared to non-CHD complex-CHD patients had higher odds of undergoing a Kasai related reintervention(OR:5.01;95%CI:1.76-14.22,p=0.002. After propensity score matching, when compared to non-CHD a CHD diagnosis was only associated with an increased risk for any complication(OR:2.50;95%CI:1.30-4.81,p=0.005).
Conclusion: A CHD diagnosis is present in 10% of children undergoing KPE. KPE appears to be safe in patients with CHD, but associated with increased in-hospital resource utilization. Further studies are needed to understand the impact of a CHD diagnosis on the long-term outcomes after KPE.
