Isolated unilateral pulmonary artery atresia in an adult presenting with cor pulmonale

BMJ Case Rep. 2023 Dec 30;16(12):e256663. doi: 10.1136/bcr-2023-256663.


Unilateral pulmonary artery atresia (UPAA) is a rare embryonic vascular malformation, leading to general presentations of exertional dyspnoea, pneumonia and haemoptysis. Our patient, a man in his early 30s, presented with a history of progressive breathlessness over a period of 2 years. History showed multiple admissions for pneumonia over his childhood and adolescence. Physical examination revealed a loud P2, an ejection systolic murmur and coarse crepitations in multiple lung fields. Severe pulmonary artery hypertension was noted on two-dimensional echocardiography. CT studies confirmed right pulmonary artery atresia with aspergilloma and bronchiectasis. With this diagnosis of UPAA, this case report intends to raise awareness among clinicians to consider this as a rare cause of cor pulmonale in an adult.

Keywords: Adult intensive care; Cardiovascular medicine; Pulmonary hypertension; Radiology; Respiratory medicine.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Chronic Disease
  • Heart Defects, Congenital* / complications
  • Humans
  • Hypertension, Pulmonary* / etiology
  • Male
  • Pneumonia* / complications
  • Pulmonary Artery / abnormalities
  • Pulmonary Artery / diagnostic imaging
  • Pulmonary Heart Disease* / diagnostic imaging
  • Pulmonary Heart Disease* / etiology
  • Vascular Malformations* / complications