J Korean Pediatr Soc.  2000 Feb;43(2):229-235.

Long-term Clinical Study of Total Anomalous Pulmonary Venous Return

• Song JY
• Yoon YS

Abstract

PURPOSE: Total anomalous pulmonary venous return is a rare congenital heart disease, which is fatal if untreated, especially, if the obstruction in pulmonary venous return is obstructed. With the technical development of echocardiogram, we are now able to do an early diagnosis if TAPVR and perform surgery. Accordingly, this report was aimed to evaluate patients with TAPVR as well as evaluate the problematic factors in order to contribute to future treatment.
METHODS
From Sept. 1st, 1985 to June 30, 1999, we retrospectively evaluated 107 patients who were diagnosed TAPVR by echocardiogram and catheterization and angiogram. TAPVR in complex heart diseases were excluded.
RESULTS
The patients were composed of 64 boys and 43 girls with the mean age of 206 days. The subtype of TAPVR is as follows : supracardiac type 45%, cardiac type 33%, infracardiac type 9% and mixed type was 13%. In 39 patients(36%), pulmonary venous obstruction was detected by echocardiogram and all the patients with infracardiac type TAPVR, except one patient, revealed obstructive pulmonary venous return. Surgery was performed in 96 patients and the mean age at operation was 270 days old. The operative mortality was not different statistically among subtypes(P=0.212) and also was not different between obstructive type and nonobstructive type(P=0.370). The mean age at operation from 1994 to 1999 was younger than that of up to 1994. Pulmonary vein stenoses after operation were detected in 14 patients(14.6%) and only in 3 patients developed, pulmonary vein stenoses at the same site of previous ones. Thus, pulmonary vein stenoses after operation could be mainly considered as secondary due to operation.
CONCLUSION
The results from our study reveal that operative results for TAPVR were much improved with early diagnosis and operation and those results were not dependent on the subtypes of TAPVR. Postoperative pulmonary vein stenosis was a poor prognostic factor and for early detection, catheterization and angiogram could be recommended if there is suspicion.

Keyword

Total anomalous pulmonary venous return; Perioperative mortality; Pulmonary venous obstruction; Pulmonary vein stenosis

MeSH Terms

Catheterization
Catheters
Constriction, Pathologic
Early Diagnosis
Female
Heart Defects, Congenital
Heart Diseases
Humans
Mortality
Pulmonary Veins
Retrospective Studies
Scimitar Syndrome*