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.

An 82-year-old man was referred to our hospital because of fever and disequilibrium. Brain magnetic resonance imaging showed acute multiple cerebral infarctions with multiple small intracerebral hemorrhages. The laboratory tests revealed an elevated inflammatory response, and two separate sets of blood cultures were positive for Streptococcus oralis. Transesophageal echocardiography revealed a single site of vegetation (13×11 mm) of the mitral anterior annulus. The vegetation apparently did not involve the intervalvular fibrous body. Moderate mitral regurgitation and mild to moderate aortic regurgitation were detected. Early surgical intervention was considered, but there was a high risk of operative mortality. We thus initially performed only medical treatment. Transesophageal echocardiography was again performed 12 days after his admission and revealed vegetation of the mitral anterior annulus progressing to the aortic annulus via the intervalvular fibrous body. It seemed to be difficult to control this progressive infective endocarditis with medical treatment. We therefore performed a semi-urgent operation. With an incision into the right-side left atrium, we identified the vegetation of the center of the mitral anterior leaflet progressing to the mitral anterior annulus. Subsequently, we added an aortotomy with Manouguian’s incision. We were able to remove all vegetation that was present in the aortic annulus, intervalvular fibrous body, and mitral annulus with a Commando operation. Finally, we performed double valve replacement with reconstruction of the intervalvular fibrous body and other lost cardiac structures using one boat-shaped bovine pericardial patch. He was discharged to home 34 days after surgery with no neurological complications and no recurrence of infective endocarditis. He also had no recurrence of infective endocarditis and no paravalvular leakage on either prosthetic valve at one year after the surgery.