All issues > Volume 38(3); 1995

Case Report

J Korean Pediatr Soc. 1995;38(3):409-416. Published online March 15, 1995.

Isolated Unilateral Pulmonary Vein Atresia

Yun Ae YA Jeon¹, Chung Il CI Noh¹, Ho Sung HS Kim¹, Jung Yun JY Choi¹, Yong Soo YS Yun¹, Woo Sun WS Kim², Je Geun JG Chi³

¹Department of Pediatrics, College of Medicine, Seoul National University, Seoul, Korea
²Department of Radiology, College of Medicine, Seoul National University, Seoul, Korea
³Department of Pathology, College of Medicine, Seoul National University, Seoul, Korea

Abstract

We report four cases of unilateral pulmonary vein atresia without associated congenital intracardiac anomalies to illustrate a part of the clinical and radiological characteristics and its diagnosis. Pulmonary vein atresia was in right side in all cases. Narrowing of contralateral pulmonary vein and pure red cell anemia were combined in one cases. In four cases, initial presentations were hemoptysis and lower respiratory tract infection. The simple chest X-ray revealed loss of volume, increased reticular interstitial markings of the involved lung and the shift of mediastinal structures to the involved side, and hilar prominency in the contralateral side. Lung perfusion scan revealed no visualization of involved lung. Cardiac Catheterization revealed pulmonary artery hypertension 2/4 and pulmonary artery wedge pressure 1/2. On pulmonary angiogram, nonvisualiaztion of pulmonary vein in the involved side in 3: partial visualization of pulmonary vein not connected to atrium in 1: dysmorphic pulmonary artery on the contralateral side in 1. Right pneumonectomy was done without specific problems in two cases. In conclusion, Unilateral pulmonary vein atresia should be suggested when the clinical and radiological characteristics are present.

Keywords :Pulmonary vein atresia, Pulmonary hypertension, Pulmonary vein obstruction