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Isolated unilateral absence of right proximal pulmonary artery: Surgical repair and follow-up ANNALS OF THORACIC SURGERY Welch, K., Hanley, F., Johnston, T., Cailes, C., Shah, M. J. 2005; 79 (4): 1399-1402

Abstract

The isolated unilateral absence of a proximal pulmonary artery is a rare congenital lesion with a diverse clinical presentation. If the connecting ductus arteriosus closes after birth, the ipsilateral pulmonary artery will lose its source of blood supply, resulting in hypoplasia or obliteration of intrapulmonary vessels. Despite a seemingly benign early clinical course, a significant number of untreated patients will develop pulmonary hypertension, hemoptysis, and recurrent respiratory infections. Early detection and surgical repair provides restoration of physiologic pulmonary circulation, regression of pulmonary hypertension, and the potential for normal distal pulmonary vascular development.

View details for DOI 10.1016/j.athoracsur.2003.10.037

View details for Web of Science ID 000228091100052

View details for PubMedID 15797092