Synonyms: Aplasia of pulmonary artery | UAPA | Unilateral Pulmonary Artery Absence | Unilateral pulmonary artery agenesis
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.
Data from Orphanet are used to provide information on a disease's name, synonym(s), and overview. Reference: Access aggregated data from Orphanet at Orphadata. Orphadata: Free access data from Orphanet. © INSERM 1999. Available on http://www.orphadata.org. Data version September 2024
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Absence of the pulmonary artery?
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