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Ann Thorac Surg 2005;79:1399-1402
© 2005 The Society of Thoracic Surgeons

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Case report

Isolated Unilateral Absence of Right Proximal Pulmonary Artery: Surgical Repair and Follow-Up

^a Department of Pediatrics, University of Washington School of Medicine, Seattle, Washington, USA
^b Department of Cardiothoracic Surgery, Stanford University School of Medicine, Palo Alto, California, USA

Accepted for publication October 16, 2003.

^* Address reprint requests to Dr Shah, University of Pennsylvania, Childrens Hospital of Philadelphia, 34th and Civic Ctr Blvd, Philadelphia, PA 19104, USA
shahm{at}email.chop.edu

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.

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