Surgical treatment for a 16-year-old girl with anomalous origin of the right pulmonary artery from ascending aorta

Abstract

Anomalous origin of the right pulmonary artery is a rare congenital malformation and is usually fatal without early surgical correction. A 16-year-old patient, who had received no previous treatment, demonstrated abnormal findings on ECG. On cardiac catheterization, the pressure in the normally arising left pulmonary artery was found to be greater than the systemic level. Bidirectional shunting across a patent ductus was identified and aortography revealed that the right pulmonary artery arose from the posterior wall of the proximal ascending aorta. The presence of severe pulmonary vascular obstructive disease was anticipated and right lung biopsy was performed. The index of pulmonary vascular disease rating was 2.8 and the Heath-Edwards classification was grade 3. Consequently, we considered that corrective right pulmonary circulation could be maintained after correction, and total correction was performed. The postoperative course was uneventful and the pulmonary artery pressure reduced significantly. Discussion focuses on the surgical indications for such infrequent older cases and we conclude that preoperative lung biopsy is useful in identifying severe pulmonary vascular disease and evaluating indications for surgery.