absence of the pulmonary artery
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.
MONDO:0000473
arterial disorder
is_a
MONDO:0020292
congenital anomaly of the great arteries
is_a
UAPA
exact
agenesis of pulmonary artery
exact
aplasia of pulmonary artery
exact
congenital absence of pulmonary artery
related
pulmonary artery absent
exact
pulmonary artery agenesis
exact
unilateral pulmonary artery absence
exact
unilateral pulmonary artery agenesis
exact