Journal of the Korean Pediatric Society 1995;38(3):409-416.
Published online March 15, 1995.
Isolated Unilateral Pulmonary Vein Atresia
Yun Ae Jeon1, Chung Il Noh1, Ho Sung Kim1, Jung Yun Choi1, Yong Soo Yun1, Woo Sun Kim2, Je Geun 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
심기형이 동반되지 않은 일측성 폐정맥 폐쇄증
전윤애1, 노정일1, 김호성1, 최정연1, 윤용수1, 김우선2, 지제근3
1서울대학교 의과대학 소아과학교실
2서울대학교 의과대학 진단방사선과학교실
3서울대학교 의과대학 병리학교실
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.
Key Words: Pulmonary vein atresia, Pulmonary hypertension, Pulmonary vein obstruction

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