StayCurrentMD · Letter to the editor - Congenital cardiac disease and biliary atresia - Not a benign relationship
Article1 min read·Published Sep 2025

Letter to the editor - Congenital cardiac disease and biliary atresia - Not a benign relationship

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Article · Sep 2025 · 1 min read

In brief

In brief

This letter critiques a database review examining outcomes in infants with biliary atresia and concurrent congenital heart disease undergoing Kasai portoenterostomy. While the study found increased complications in CHD patients with no 30-day mortality, the author highlights a critical limitation: meaningful outcomes in this population typically emerge beyond the 30-day data capture window.

  • 10% of infants undergoing Kasai portoenterostomy have congenital heart disease (CHD), per NSQIP-P data from 2012-2022.
  • Simple CHD doubles complication risk post-Kasai; complex CHD triples it compared to non-CHD patients.
  • No deaths occurred in the CHD cohort within 30 days, but NSQIP-P data cutoff limits long-term outcome assessment.
  • Critical outcomes in biliary atresia with CHD likely occur beyond the 30-day NSQIP-P surveillance window.
  • Clinicians should anticipate higher perioperative morbidity when managing Kasai patients with concurrent cardiac anomalies.

Written by the GCMD Library team from the article.

Connor et al. review the National Surgical Quality Improvement Progam-Pediatric (NSQIP-P) database (2012–22) identifying 854 infants from around the USA undergoing a Kasai portoenterostomy (KPE) with the aim of exploring the effect of any associated congenital heart disease (CHD) [1]. They identified 89(10 %). Compared to non-CHD these infants had a two-fold increase in complications if the CHD was “simple” and a three-fold increase if “complex” and importantly no deaths. The problem is that the data-stream stops conclusively at 30 days and all the real action tends to happen after that.

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