Cardiac-associated biliary atresia (CABA): a prognostic subgroup. Issue 1 (20th July 2020)

• Record Type: Journal Article
• Authors: Aldeiri, Bashar; Giamouris, Vangelis; Pushparajah, Kuberan; Miller, Owen; Baker, Alastair; Davenport, Mark
• Is Part Of: Archives of disease in childhood. Volume 106:Issue 1(2021)
• Page Start: 68
• Page End: 72
• Publication Date: 2020-07-20
• Subjects: cardiac surgery -- congenital abnorm -- paediatric surgery -- hepatology; Children -- Diseases -- Periodicals; Infants -- Diseases -- Periodicals; 618.920005
• DOI: 10.1136/archdischild-2020-319122
• Languages: English
• ISSNs: 0003-9888

Abstract

Abstract : 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.