Aldieri et al reported a subgroup of biliary atresia with a cardiac malformation. This identifies a high-risk patient population. The authors are from King's College London.
Patient selection: biliary atresia (BA)
Features of cardiac-associated biliary atresia (CABA):
(1) presence of biliary atresia
(2) concurrent cardiac anomaly (designated A if acyanotic; B if cyanotic; or C for insignificant shunt)
Variable features:
(1) biliary atresia splenic malformation (BASM)
(2) cat-eye syndrome
(3) other malformations
Outcomes:
(1) clearance of jaundice (serum total bilirubin <= 20 µmol/L) after Kasai portoenterostomy
(2) native liver survival
(3) overall actuarial survival
The combination of biliary atresia and congenital cardiac defects is associated with high mortality.
The recommendation was to perform early restorative cardiac surgery prior to Kasai portoenterostomy. This was associated with better clearance of jaundice and lower mortality.
