Objectives To describe the range of concurrent cardiac malformations in biliary atresia (BA) while providing a functional framework of risk.
Methods Demographic and variables were collected from a prospectively maintained single-centre database. Infants were grouped according to a cardiac functional framework (A=acyanotic, B=cyanotic and C=insignificant shunt). Primary outcome was set as clearance of jaundice (bilirubin ≤20 μmol/L) following Kasai portoenterostomy (KPE). Native liver survival and overall actuarial survival were compared with a date-matched control infant with BA (n=77). P value <0.05 was regarded as significant.
Results 524 infants with histologically confirmed BA were treated between January 1999 and December 2018, 37 (7%) had a concurrent cardiac anomaly (A: n=23 (62%), B: n=10 (27%), C: n=4 (11%)). Infants with biliary atresia splenic malformation (BASM) or cat-eye syndrome (CES) contributed over half of the cases (21/37; 57%).
Overall, 20 (54%) infants cleared jaundice (vs 50/77 (65%) controls; p=0.2), but with higher mortality compared with the non-cardiac controls (15/37 (40%) vs 3/77 (4%); HR 15.5 (95% CI 5.5 to 43.4); p<0.00001). Infants requiring cardiac intervention in the first year of life (n=15) were more likely to clear jaundice (6/7 vs 2/8; p=0.04) and had a trend towards higher survival (6/7 vs 3/8; p=0.1) when KPE followed cardiac surgery. Yet, the type of cardiac pathology did not impact clearance of jaundice or mortality.
Conclusion We propose the term cardiac-associated biliary atresia (CABA) as a high-risk group. We believe that restorative cardiac surgery should precede KPE wherever possible to improve outcome.
- cardiac surgery
- congenital abnorm
- paediatric surgery
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Contributors BA: collected data and provided the initial analysis, drafted and revised the manuscript. VG: collected data and reviewed and revised the manuscript. KP and OM: reviewed initial cardiac investigations and revised the manuscript. AB: assisted in data collection, reviewed and revised the manuscript. MD: conceptualised the study, performed data analysis, reviewed and revised the manuscript. All authors approved the final manuscript as submitted and agree to be accountable for all aspects of the work.
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Competing interests None declared.
Patient consent for publication Not required.
Provenance and peer review Not commissioned; externally peer reviewed.
Data availability statement All data relevant to the study are included in the article or uploaded as online supplementary information. All data relevant to this manuscript would be available on request from the corresponding author.