RESUMO
Two infants with aortico-left ventricular tunnel and associated aortic valvular disease were operated upon. The first patient, age 28 months, had successful surgical closure of the anomalous communication. Four years postoperatively he is asymptomatic, but mild aortic regurgitation persists. The second infant, aged 3 months (the youngest reported surgical case), also had severe aortic stenosis (dysplastic valve) and mitral regurgitation. She died, twelve hours after the operation, from intractable left ventricular failure. On the basis of the previously reported cases (22), the clinical profile of the malformation is outlined with special reference to associated aortic valve lesions. Early operation is recommended because of the poor natural history of the condition.