Combined open-heart surgery and living donor liver transplantation in a 4-year-old child with Alagille syndrome - A case report

Abstract

Alagille syndrome (ALGS) is a complex multisystem genetic disorder. Patients with ALGS usually require liver transplantation (LT). Congenital heart diseases are also common in ALGS patients. ALGS patients may become ineligible for LT procedure due to cardiac abnormalities whereas any corrective cardiac surgeries are precluded due to the Child C cirrhosis of the liver in these patients. We report a unique case of a 4-year- and 10-month-old girl who underwent open-heart surgery and living donor liver transplantation (LDLT). She had significant hepatic and cardiopulmonary involvement, and Child C cirrhosis with portal hypertension and complex cardiac defects, and was ineligible for up-front LT. An open-cardiac surgery was performed and after 12 h she received LDLT using a left lateral hepatic graft. This is the first report of a case of successful combined open-heart surgery and LDLT in a child with ALGS with cardiac defects and Child C liver cirrhosis.