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Pulmonary Artery Agenesis Presenting as Massive Hemoptysis — A Case Report

Department of Pulmonary Disease, The Cleveland Clinic Foundation, Cleveland, Ohio

Douglas R. Livingston, D.O.

Department of Pulmonary Disease, The Cleveland Clinic Foundation, Cleveland, Ohio

Wayne Kawalek, M.D.

Department of Internal Medicine, The Cleveland Clinic Foundation, Cleveland, Ohio

Joseph A. Golish, M.D., F.A.C.A.

Department of Pulmonary Disease, The Cleveland Clinic Foundation, Cleveland, Ohio

James K. O'Donnell, M.D., F.A.C.A.

Department of Radiology, The Cleveland Clinic Foundation, Cleveland, Ohio

Massive hemoptysis is the expectoration of approximately 600 ml of blood in twenty-four hours. Major causes of massive hemoptysis are tuberculosis, bronchiectasis, pulmonary neoplasm, fungus ball, bronchial adenomas, lung abscess, intrabronchial rupture of an aortic aneurysm, cystic fibrosis, pulmonary infarction, and pulmonary trauma. Other, less common causes include Good-pasture's syndrome, broncholiths, pulmonary varix, A-V malformation, and bleeding disorders. Agenesis of the pulmonary artery usually occurs in association with congenital cardiac anomalies, and isolated unilateral absence of the pulmonary artery is uncommon. About 10% of the patients with pulmonary artery agenesis develop inconsequential hemoptysis, but massive hemoptysis is a very rare complication of this anomaly. The following is a case report of a twenty-nine-year-old man with agenesis of the left pulmonary artery, who presented with massive hemoptysis requiring embolization and, eventually, pneumonectomy.

Angiology, Vol. 38, No. 1, 67-71 (1987)
DOI: 10.1177/000331978703800110


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