Anomalous origin of the right pulmonary artery from the ascending aorta: a surgical case study in an adult patient with "irreversible" pulmonary vascular disease.

An adult patient presented with an anomalous right pulmonary artery arising from the ascending aorta with severe unilateral pulmonary vascular disease of the left lung due to a large left-sided patent ductus arteriosus. A stenosis in the right pulmonary artery protected the right pulmonary vasculature. Right ventricular pressure was suprasystemic. After ductal ligation and surgical repair, pulmonary artery pressures fell dramatically. An immediate postoperative angiography confirmed extremely poor perfusion of the left lung. At four-and-a-half years of follow-up, the patient's quality of life had improved dramatically, main pulmonary artery pressure was one-sixth of systemic pressures, and there was vastly improved perfusion of the left lung at this lower perfusion pressure. The calculated pulmonary vascular resistance of the left lung was within normal limits. These findings suggested a significant degree of reversal of pulmonary hypertensive disease in the left lung sustained to 54 months postoperatively.