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Clinical Studies on Total Anomalous Pulmonary Venous Connection.

Journal of the Korean Pediatric Society 1982;25(11):1120-1130.
Published online November 30, 1982.
Clinical Studies on Total Anomalous Pulmonary Venous Connection.
Young Jin Hong, Kee Hong Kwon, Hae Il Cheong, Jung Yeun Choi, Yong Soo Yoon, Chang Yee Hong
Department of Pediatrics,College of Medicine, Seoul National University
전폐정맥연결이상에 대한 임상적 관찰
홍영진, 권기홍, 정해일, 최정연, 윤용수, 홍창의
서울대학교 의과대학 소아과학교실
Total anomalous pulmonary venous connection (TAPVC) is a rare congenital defect in which all the pulmonary veins are connected to the right atrium or its tributary veins. Without surgical intervention, most patients with TAP VC have grave prognosis, with cardiac failure and death in infancy. But owing to the advances of surgical technique and postoperative care, complete correction has been accomplished with good results. Sixteen cases of TAP VC have been observed at the Department of Pediatrics, SNUH during past 16 years of period, from January, 1966 to May, 1982. Thirteen cases were male and three were female. Age distribution was from 2 day to 10 5/12 year. The anomalous site of connection was to the left innominate vein in 11 cases, to the right SVC in 2 cases, to the right atrium in 1 case, and mixed type in 2 cases. There was no subdiaphragmatic type. Eight cases were complicated with other cardiac anomalies and eight were not. Radiologically, characteristic superior mediastinal shadow, socalled “snowman” appearance was seen in 5 cases with TAP VC to the left innominate vein. The EKG showed right axis deviation in 4/5 and right ventricular hypertrophy in all cases. M-mode echocardiograms showed increased dimension of the right ventricle in all cases and the common pulmonary vein was visualized posterior to the left atrium in 5 cases. One case of pulmonary venous obstruction was observed by angiogram. Three of 5 cases submitted to surgery survived after corrective procedures.
Key Words: Pulmonary vein, total anomalous connection. Pulmonary hypertension. Pulmonary venous obstruction. Cardiac catheterization. Electrocardiographic findings. Echocardiographic findings. Radiographic findings. Corrective surgery.

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