Pneumonectomy for congenital isolated unilateral pulmonary artery agenesis.
Unilateral pulmonary artery agenesis (UPAA) is a rare congenital anomaly usually diagnosed in infancy due to associated cardiovascular malformations. We report a rare case of isolated right UPAA that presented atypically in adulthood with massive haemoptysis requiring a pneumonectomy. This case highlights the importance of maintaining a high clinical suspicion, the role of CT angiography and a multi disciplinary approach. Optimal management is often surgical however bronchial artery embolization (BAE) remains a useful adjunct.
Department of Cardiothoracic Surgery, University Hospital North Staffordshire NHS Trust, Stoke-on-Trent, ST4 7LN, United Kingdom.
J
J
Britton
A
A
Sachithanandan
L
L
Srinivasan
S
S
Ghosh
D000328:Adult; D001981:Bronchial Arteries; D004621:Embolization, Therapeutic; D005260:Female; D006801:Humans; D011013:Pneumonectomy / Q000379:methods*; D011651:Pulmonary Artery / Q000002:abnormalities*