Pulmonary atresia or severe stenosis and coronary artery-to-pulmonary artery fistula.

Five patients have had the common features of pulmonary atresia or severe stenosis associated with a septal defect and a coronary artery-to-pulmonary artery (CA-PA) fistula. Four had pulmonary valvular atresia, and one had severe pulmonary stenosis. In all five, the CA-PA fistula contributed the principal component of the pulmonary blood flow. Cyanosis, continuous murmur, right ventricular hypertrophy, and decreased or normal pulmonary vascularity were frequent clinical manifestations, but angiocardiography was required to establish the diagnosis. The fistula was a side-to-side communication between the left coronary artery and the main pulmonary artery in each of the five patients. The dilated proximal coronary artery connecting the aortic root with the pulmonary artery gave a unique angiocardiographic appearance distinguishing it from truncus arteriosus or aorticopulmonary window. It is suggested that this unique angiocardiographic appearance be called an `aorticopulmonary tunnel." The surgical repair of this anomaly is closely related to that for severe tetralogy of Fallot and should involve a comparable risk and achieve a comparable result.