Aortic regurgitation in tetrad of Fallot and pulmonary atresia

Data on 18 patients, aged 12 to 42 years, with documented aortic regurgitation and tetrad of Fallot or pulmonary atresia with ventricular septal defect were reviewed. In two patients, aortic regurgitation was caused by surgical repair but in the others it was present before operation. There was increased volume overload from a long-standing surgical shunt (8 to 30 years' duration) or congenital systemic collateral vessels in 14. Six patients with a history of infective endocarditis had aortic cusp perforations. Failure to detect the presence of aortic regurgitation before radical repair in six patients contributed to operative problems and postoperative morbidity and mortality. Aortic valve surgery was performed in 13 patients. Aortic regurgitation is an acquired complication that should be specifically excluded by routine retrograde ascending aortography in all adolescents or adults with tetrad of Fallot or pulmonary atresia with ventricular septal defect. Earlier radical repair in the first decade of life may prevent the complication.