ABSTRACT
A 4-year-old boy was born with cyanosis and was given a diagnosis of tetralogy of Fallot and pulmonary atresia. Echocardiography showed membranous atresia of the pulmonary trunk that was connected to the left side of the ascending aorta via an aortopulmonary window 3 mm in diameter. Four major aortopulmonary collateral arteries (MAPCAs) were detected by cardiac catheterization and computed tomography angiography prior to undergoing surgery at 4 years of age. We performed one-stage complete unifocalization and definitive repair via a median sternotomy. The MAPCA supplying the left lower lobe was anastomosed to the true left pulmonary artery and the pulmonary artery trunk was augmented with an autologous pericardium patch. We then reconstructed the right ventricular outflow tract using a transannular patch and simultaneously patch-closed the VSD. The right/left ventricle pressure ratio after weaning from cardiopulmonary bypass was 0.8. The postoperative course was uneventful and the patient was discharged 26 days later. Seven months after the procedure, the right/left ventricle pressure ratio was decreased to 0.56 on cardiac catheterization.
ABSTRACT
A baby girl delivered at 41 weeks of gestation with persistent truncus arteriosus (PTA) and interrupted aortic arch (IAA) type A was referred to our institute for surgical intervention. Bilateral pulmonary artery banding (BPAB) proceeded through a median sternotomy at the age of 11 days to control excessive pulmonary blood flow. Thereafter, she gained weight under continuous prostaglandin E1 (PGE 1) infusion. Definitive repair proceeded at the age of 2 months. Cardiopulmonary bypass was established through a redo-median sternotomy, with two arterial cannulae (brachiocephalic artery and descending aorta). The aortic arch was reconstructed with direct anastomosis. The orifice of the pulmonary artery was removed from the arterial trunk and the defect in the aortic wall was directly closed. A ventricular septal defect was closed under cardioplegic arrest via a right ventriculotomy. The continuity from the right ventricle to the pulmonary artery was made using a hand-made, extended polytetrafluoroethylene (ePTFE) conduit with a bicusp. The sternum was left open at the end of the procedure and the chest was closed on post-operative day (POD) 3. She was weaned from mechanical ventilation on POD 4 and the postoperative course was uneventful. She was discharged on POD 49.