title: Isolated unilateral absence of right proximal pulmonary artery: surgical repair and follow-up.
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.
authors.affiliation: Department of Pediatrics, University of Washington School of Medicine, Seattle, Washington, USA.
authors.forename: Kristin
authors.initials: K
authors.lastname: Welch
authors.forename: Frank
authors.initials: F
authors.lastname: Hanley
authors.forename: Troy
authors.initials: T
authors.lastname: Johnston
authors.forename: Colleen
authors.initials: C
authors.lastname: Cailes
authors.forename: Maully J
authors.initials: MJ
authors.lastname: Shah
mesh_terms: D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D011651:Pulmonary Artery / Q000002:abnormalities* / Q000000981:diagnostic imaging / Q000601:surgery; D011859:Radiography