Leonard E. Swischuk, Philippe L’Heureux
Universityb of Texas Medical Branch. University of Minnesota Medical School.
United States
American Journal of Roentgenology
AJR Am J Roentgenol 1980; 135: 667-672
DOI: 10.2214/ajr.135.4.667
Abstract
Three cases of unilateral pulmonary vein atresia in children are described. The findings in all three cases were similar and consisted of hemoptysis, recurrent pulmonary infections, and a normal size or small lung with reticular markings. In combination, these findings should allow one to strongly suspect the diagnosis which then can be confirmed with radionuclide perfusion studies and pulmonary angiography. Both procedures demonstrate decreased blood flow to the involved lung, but the pulmonary arteriogram is the definitive study in that it shows a small pulmonary artery, pruned peripheral branches, stasis of contrast material, and nonvisualization of draining pulmonary veins. The condition generally is believed to be congenital and related to the somewhat more common, but still rare, focal congenital pulmonary vein stenosis. Treatment consists of surgical removal of the involved lung or lobe.
Category
Class II. Pulmonary Hypertension Associated with Pulmonary Vein Stenosis
Symptoms and Findings Associated with Pulmonary Vascular Disease
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: Yes