title: Sequential therapy in a case of extrahepatic biliary atresia associated with ventricular septal defect: liver transplantation followed by surgical closure of the septal defect.
abstract: 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.
authors.affiliation: Department of General Surgery and Liver Transplantation, Fundeni Clinical Institute, Sos. Fundeni no. 258, 72437 Bucharest, Romania. irinel.popescu@icfundeni.ro
authors.forename: Irinel
authors.initials: I
authors.lastname: Popescu
authors.forename: Massimo
authors.initials: M
authors.lastname: Malago
authors.forename: Giuliano
authors.initials: G
authors.lastname: Testa
authors.forename: Silviu
authors.initials: S
authors.lastname: Ciurea
authors.forename: Codruţ
authors.initials: C
authors.lastname: Stănescu
authors.forename: Ion
authors.initials: I
authors.lastname: Socoteanu
mesh_terms: D001656:Biliary Atresia / Q000601:surgery*; D005260:Female; D006345:Heart Septal Defects, Ventricular / Q000601:surgery*; D006801:Humans; D007223:Infant; D016031:Liver Transplantation