Sequential therapy in a case of extrahepatic biliary atresia associated with ventricular septal defect: liver transplantation followed by surgical closure of the septal defect.

Patients with extrahepatic biliary atresia (EHBA), the commonest indication for liver transplantation in pediatric cases, may have associated congenital anomalies, e.g. interventricular septal defect (IVSD). Even if there is a well established therapeutical algorithm for a ventricular septal defect as an isolated disease, the surgical approach raises special problems in a liver transplanted patient. We present the case of a little girl with EHBA and IVSD. The patient underwent a living related liver transplantation when she was 1 year old. Persistence of the symptomatic IVSD required open heart surgery, performed 9 months posttransplantation. The decision to perform the closure of the IVSD was difficult, yet it proved to be beneficial; the child had an uneventful postoperative course, with remission of the symptoms and a good late outcome. To our knowledge, this is the first case of a living-related liver transplantation patient undergoing open heart surgery for IVSD closure.