Isolated unilateral pulmonary vein atresia in adult patients: a case report and literature review.

ABSTRACT

Unilateral pulmonary vein atresia is a rare abnormality that usually presents in infants with recurrent hemoptysis and pneumonia. Presentation in adulthood without additional congenital heart disease is rare but does occur. Anatomic variations in the pulmonary vessels that supply and drain the affected lung can explain the mildly symptomatic process. The diagnosis of isolated unilateral pulmonary vein atresia is usually made angiographically. Pneumonectomy is indicated once symptoms or complications are present so that irreversible pulmonary hypertension can be avoided.