[A case report of the radical correction of a truncus arteriosus and peripheral pulmonary stenosis in association with Holt-Oram syndrome].

A one year and 6 months old female infant was admitted to our department for a radical correction of a truncus arteriosus (Collett-Edwards type II). She also had a peripheral pulmonary stenosis and Holt-Oram syndrome. Under a cardiopulmonary bypass with deep hypothermia, PFO was closed directly. First of all, the pulmonary arteries were dissected from the aorta, the defect of the aorta was closed directly and VSD was closed with Dacron fabric patch. The reconstruction of the pulmonary arteries were performed with valved conduit which consisted of Dacron graft and 14 mm Björk-Shily valve. The post-operative course was uneventful. Cardiac catheterization study, done 77 days after surgery, revealed 44 mmHg of pressure gradient between RV outflow tract and right PA. The patient was doing well and two years have passed since the surgery. She should be observed carefully at the out-patient clinic due to the mechanical valve and the presence of pressure gradient at RV outflow tract.