Unilateral pulmonary vein atresia: clinical and radiographic spectrum

Lee B. Beerman, Kook Sang Oh, Sang C. Park, Michael D. Freed, Henry M. Sondheimer, Frederick J. Fricker, Robert A. Mathews, Donald R. Fischer
Children’s Hospital of Pittsburgh and University of Pittsburgh School of Medicine.
United States

Pediatric Cardiology
Pediatr Cardiol 1983; 4: 105-112
DOI: 10.1007/BF02076334

Abstract
Three cases of unilateral pulmonary vein atresia are presented to illustrate part of the clinical and radiographic spectrum of this anomaly. One patient had major associated cardiovascular abnormalities and pulmonary hypertension, one had recurrent hemoptysis, and the other patient was asymptomatic with normal pulmonary artery pressure and no associated abnormalities. In one case, serial catheterizations indicated that the pulmonary vein atresia was acquired. This report stresses the radiographic, hemodynamic, and angiographic findings in patients with unilateral severe pulmonary venous obstruction. The embryology, pathology, and surgical approach are also discussed. The diagnosis should be strongly suspected before cardiac catheterization when typical features are present on the chest radiograph and isotopic ventilation perfusion scan.

Category
Class II. Pulmonary Hypertension Associated with Pulmonary Vein Stenosis
Diagnostic Testing for Pulmonary Vascular Disease. Non-invasive Testing
Diagnostic Testing for Pulmonary Vascular Disease. Invasive Testing

Age Focus: Pediatric Pulmonary Vascular Disease

Fresh or Filed Publication: Filed (PHiled). Greater than 1-2 years since publication

Article Access
Free PDF File or Full Text Article Available Through PubMed or DOI: No

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