Hypoplastic right lung associated with right pulmonary vein stenosis and systemic collateral

Abstract

We present a case of a 6-month-old girl diagnosed as unilateral pulmonary vein stenosis (right inferior pulmonary vein) with dextroposition of the heart, hypoplastic right lung, hypoplastic right pulmonary artery, collateral from the descending aorta supplied right lower lung, connection between collateral and right pulmonary artery with retrograde flow from right pulmonary artery into main pulmonary artery, and right superior and middle pulmonary vein agenesis. A combined modality of imaging including chest film, echocardiography, multidector computerized tomography, and the cardiac catheterization nicely demonstrated all of the unique and rare congenital abnormalities. © 2010 Copyright the Authors. Journal Compilation © 2010 Wiley Periodicals, Inc.