Congenital absence of the left pulmonary artery. Association with multiple branch stenoses of the right pulmonary artery leading to pulmonary hypertension.

Abstract

CONGENITAL absence of a pulmonary artery is a rare anomaly.1-3When therightpulmonary artery is absent, most such patients have no associated heart disease. Absence of theleftpulmonary artery has usually been associated with heart disease, specifically tetrad of Fallot. We wish to report the first case of absence of theleftpulmonary artery with pulmonary hypertension from multiple branch stenoses of therightpulmonary artery in the absence of any associated heart disease. The basic malformation of absence of theleftpulmonary artery, though proven definitively only by cardiac catheterization and angiocardiography, has suggestive plain-film and bronchographic findings. Lung scanning adds information. Previously not stressed is the virtually diagnostic combination of arightaortic arch on barium swallow in a patient with a heart shifted to the left, a smaller left hemithorax, and diminished vascularity on chest x-ray films of the involved left lung. Report of