抄録
A six year old boy presented jaundice and cardiac murmur at the age of three. Laboratory tests showed moderate cholestasis and the liver biopsy disclosed intrahepatic bile duct hy-poplasia. Cardiac catheterization showed peripheral pulmonary stenosis and atrial septal defect. His face and vertebral deformity was characteristic with arteriohepatic dysplasia, Alagilles' syndrome. He underwent direct closure of atrial septal defect, right ventricular outflow and pulmonary arterial patch plasty. Postoperative course was uneventful and no hepatic derrangement was observed. This is the first reported case of open heart surgery carried out on a patient with this syndrome.
