We report a successful open heart reoperation of a 14-year-old girl with Alagille syndrome. The patient underwent a living related donor liver transplantation at the age of 9 years in another hospital because of liver failure due to a paucity of interlobular bile ducts. Two years later, because of progression of her aortic valve stenosis, Ross operation and CABG were performed in the same hospital. Afterwards, her neoaortic valve regurgitation developed due to aortic root dilatation and myocardial ischemia developed by anastomosis site stenosis. She started to experience frequent angina attacks. She underwent AVR and redo CABG in our institution in April 2002. Her pre- and postoperative liver function was normal and no special procedure for the liver was needed, and she was discharged on the 18th postoperative day with no complications. In this country, few open heart surgeries for liver transplant recipient have been performed, and no case of reoperation has yet been reported. If pre- and postoperative liver function are normal, pre- and postoperative management of open heart surgery for a transplant may be perfomed conventionally.

The patient was a 15-month-old girl with Down's syndrome. She had a heart murmur on the first day after birth. The echocardiogram revealed that she had the tetralogy of Fallot (TOF) and mitral insufficiency (MI). She was observed because she had no heart failure or cyanosis. However, she developed heart failure with progressive MI. Then, she was admitted to our medical center for surgical treatment. During the operation, it was confirmed that the primary septum was intact and a large ventricular septal defect was located at the inlet to outlet portion with anterior malalignment. Each leaflet of the atrioventricular valve were attached to the same level and the ventricular septum was scooped out. TOF with endocardial cushion defect (ECD) without primary septal defect was diagnosed based on the operative findings. Surgical repair was performed through the right atrium and pulmonary artery. She was discharged 17 days after operation without any complications. This was a very rare combination of TOF with ECD without a primary septal defect. We discussed this rare condition with a review of the literature.