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Follow up of Patients with Total Anomalous Pulmonary Venous Return in Right Atrial Isomerism

Journal of the Korean Pediatric Society 2000;43(11):1451-1457.
Published online November 15, 2000.
Follow up of Patients with Total Anomalous Pulmonary Venous Return in Right Atrial Isomerism
Soo Jin Kim1, Jae Young Lee1, Mi Young Han1, Do Jun Jo1, In Seung Park1, Mee-Hye Oh2, Eun Jung Bae3, Seong Ho Kim4
1Department of Pediatrics, Sejong Heart Institute
2Department of Pathology, Sejong Heart Institute
3Department of Pediatrics, Seoul National University College of Medicine, Seoul, Korea
4Department of Pediatrics, Gachon Medical School
총 폐정맥 환류 이상을 동반한 우측 심방 이성체 환자의 추적 관찰
김수진1, 이재영1, 한미영1, 조도준1, 박인승1, 오미혜2, 배은정3, 김성호4
1부천세종병원 소아과
2부천세종병원 해부병리과
3서울대학교 의과대학 소아과학교실
4가천의과대학 소아과학교실
: Total anomalous venous return(TAPVR) is associated in more than 60% of patients with right isomerism and can significantly complicate the management of single ventricle patients at any stage of management. We studied the results of management and sought to determine factors that may influence survival in patients with TAPVR in right atrial isomerism.
: Between February 1991 and July 1999, 14 patients with TAPVR in right atrial isomerism underwent operations; we reviewed our experience after performing single ventricle palliation
: Seven patients were of the obstructive type TAPVR and seven patients were of the non-obstructive type TAPVR. The mean age at operation was 17 months and mean body weight at operation was 7.3kg. Direct surgical repair for the pulmonary vein was performed in seven patients and in the others, TAPVR could be satisfactorily managed by the use of bilateral cavopulmonary anastomosis(BCPS) to exclude the distal superior vena cava(SVC). At a mean follow-up of 27 months, there were five deaths, and pulmonary vein restenosis developed in four patients in the direct surgical repair group. In the other group, there was neither mortality nor morbidity. Also, the presence of pulmonary venous obstruction was associated with high mortality.
: According to our study, TAPVR can be satisfactorily managed by the use of BCPS to exclude the distal SVC in the non-obstructive type. But further evaluation of surgical methods about other types of TAPVR are warranted, because TAPVR not requiring intervention includes any low supracardiac and some mixed types.
Key Words: Right atrial isomerism, Total anomalous pulmonary venous return

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