Tricuspid and pulmonary valve atresia with aortopulmonary fistula: survival of a patient to 21 years of age.

We report the case of a patient with tricuspid and pulmonic valve atresia, with survival to the age of 21 years. Postmortem examination revealed an anomalous vessel connecting the ascending aorta with the main pulmonary artery. This fistula measured 20 mm by 4 mm, arose independently of the coronary arteries and had the histologic features of an elastic artery. The remarkable longevity was ascribed in part to the maintenance of pulmonary arterial flow by the left-to-right shunt through the anomalous arterial connection.