All issues > Volume 43(11); 2000

Original Article

J Korean Pediatr Soc. 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 SJ Kim¹, Jae Young JY Lee¹, Mi Young MY Han¹, Do Jun DJ Jo¹, In Seung IS Park¹, Mee-Hye MH Oh², Eun Jung EJ Bae³, Seong Ho SH 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

Abstract

Purpose
: 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.
Methods
: 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
Results
: 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.
Conclusion
: 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.

Keywords :Right atrial isomerism, Total anomalous pulmonary venous return