J Korean Pediatr Soc.  2000 Nov;43(11):1451-1457.

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

• Kim SJ
• Lee JY
• Han MY
• Jo DJ
• Park IS
• Oh MH
• Bae EJ
• Kim SH

Abstract

PURPOSE: Total anomalous venous return(TAPVR) is associated in more than 60Yo 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 cavopulrnonary anastomosis(BCPS) to exclude the distal superior vena cava(SVC). At a mean follow-up of 27 months, there were five deaths, arid pulrnonary 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.

Keyword

Right atrial isomerism; Total anornalous pulmonary venous return

MeSH Terms

Body Weight
Follow-Up Studies*
Heterotaxy Syndrome*
Humans
Isomerism
Mortality
Pulmonary Veins
Scimitar Syndrome*
Veins