A case report of unilateral absence of left pulmonary artery.

Abstract

The Unilateral absence of a pulmonary artery (UAPA) is an uncommon congenital anomaly. Approximately 160 cases have been reported in the literature since Frantzel's first report in 1968. Most of the patients with UAPA are asymptomatic but some patients may suffer from recurrent respiratory infections, hemoptysis, or pulmonary hypertension. The diagnosis could be suspected from the chest roentgenogram and lung scan, and definitely confirmed by pulmonary angiography. We experienced a case of UAPA in a 39-year-old male with the recurrent hemoptysis. Chest X-ray revealed that the left lung volume was moderately decreased and the heart and mediastinum were displaced to the left side. Lung perfusion scan showed that the left lung was not perfused. Pulmonary angiography revealed the absence of the left main pulmonary artery. Aortic arch and descending aorta on aortogram were right sided. Blood supply to the left lung was originated from numerous systemic collaterals from intercostal and brachiocephalic origin. No other intrinsic or internal abnormalities of the cardiac chambers were noted.