Unilateral pulmonary vein atresia

Unilateral pulmonary vein atresia is a type of pulmonary vein atresia.

Clinical presentation

The condition usually present in infancy or childhood with recurrent episodes of pneumonia and/or hemoptysis. Presentation in adulthood does occur but is uncommon.

Pathology

It results from failure of incorporation of the common pulmonary vein into the left atrium. There is no recognized right or left predilection.

Radiographic features

Plain radiograph

Chest radiographic findings are not specific but may be seen as a small ipsilateral hemithorax and unilateral reticular opacities with septal lines.

CT

Allows direct visualization of absence of affected pulmonary vein. CT may additionally show:

• small ipsilateral hemithorax
• ipsilateral mediastinal shift
• small ipsilateral pulmonary artery

Other parenchymal findings (although each on its own is non-specific) include:

• interlobular septal thickening
• peribronchovascular thickening
• ground-glass opacities

Nuclear medicine

Shows decreased perfusion to affected lung.

Treatment and prognosis

Pneumonectomy is sometimes indicated once symptoms or complications are present.

Complications

• development of pulmonary hypertension