Pulmonary artery agenesis presenting as massive hemoptysis--a case report

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 Goodpasture'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.