Transatrial-transpulmonary repair of atrioventricular septal defect with right ventricular outflow tract obstruction.

Twenty patients had a repair of an atrioventricular septal defect with tetralogy of Fallot (n = 13) or double outlet right ventricle (n = 7). Mean age was 3.5 years. Surgical technique included transatrial-transpulmonary resection of right ventricular outflow tract obstruction and transatrial two patch repair of the atrioventricular septal defect. Ten patients required a transannular patch and one patient had a right ventricle-pulmonary artery conduit placed. There was no hospital mortality, and mean hospital stay was 15 days. One patient had late sudden death of unknown cause. Six patients have required reoperation because of residual ventricular septal defect (VSD), mitral incompetence, residual right ventricular outflow tract obstruction, and/or conduit stenosis. No patient was reoperated on because of left ventricular outflow tract obstruction. Fifteen patients are asymptomatic, one has exertional dyspnea, and two have intermittent occasional bronchospasm. The transatrial-transpulmonary two patch repair and extensive relief of right ventricular outflow tract obstruction have given good immediate results. Reoperation rate has been high mainly due to residual VSD and mitral incompetence.