/absence of the pulmonary artery

absence of the pulmonary artery

Rare Disease

MONDO:0020007

Also Known As

UAPAagenesis of pulmonary arteryaplasia of pulmonary arterypulmonary artery absentpulmonary artery agenesisunilateral pulmonary artery absenceunilateral pulmonary artery agenesiscongenital absence of pulmonary artery

Definition

A rare vascular anomaly characterized by congenital absence of the right or left pulmonary artery, usually ending within 2 cm of its expected origin from the pulmonary trunk. Patients often also have other cardiovascular abnormalities and respective symptoms and are then typically diagnosed in infancy or childhood, while isolated cases generally present with a mild clinical course and may go undiagnosed until adulthood. Presenting clinical features in isolated cases include hemoptysis, exertional dyspnea, and recurrent respiratory infections. The condition is typically accompanied by marked changes of lung tissue and may, if unrecognized, result in massive hemoptysis and pulmonary hypertension.