Anomalous origin of one pulmonary artery from the ascending aorta. Diagnostic, physiological and surgical considerations.

Abstract

Six patients with origin of one pulmonary artery from the ascending aorta are presented. Two are alive: a 21-year-old female, unoperated, with bilateral pulmonary vascular obstructive disease (PVO) and a 4-year-old girl who underwent successful correction at age 5 months. In addition, the data are reviewed from previous reports of 44 patients with this anomaly. Among the total of 50 patients, 42 had congestive heart failure in infancy. The right pulmonary artery arose anomalously in 44. A patent ductus arteriosus was present in 38 cases. At cardiac catheterization, pressure in both pulmonary arteries was generally at systemic level and the diagnosis was confirmed by aortography. Histologically, no significant PVO was observed in the majority of 18 infants who died, while significant changes, mainly in the "systemic" lung, were observed in 6 of 10 older children. Twenty-two patients have been operated on with 13 survivors. Anastomosis of the aberrant pulmonary artery to the main pulmonary artery directly or using a graft has been successful in 12 of 18 children. Corrective tive surgery should be performed as early as possible to prevent PVO.