CONGENITAL absence of a pulmonary artery is a rare anomaly.1-3 When the right pulmonary artery is absent, most such patients have no associated heart disease. Absence of the left pulmonary artery has usually been associated with heart disease, specifically tetrad of Fallot. We wish to report the first case of absence of the left pulmonary artery with pulmonary hypertension from multiple branch stenoses of the right pulmonary artery in the absence of any associated heart disease.
The basic malformation of absence of the left pulmonary 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 a right aortic 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.
Dimich I, Berdon WE. Congenital Absence of the Left Pulmonary Artery: Association With Multiple Branch Stenoses of the Right Pulmonary Artery Leading to Pulmonary Hypertension. Am J Dis Child. 1967;113(3):378–384. doi:10.1001/archpedi.1967.02090180138016
Customize your JAMA Network experience by selecting one or more topics from the list below.
Create a personal account or sign in to: