Total anomalous pulmonary venous return: a clinical observation of 36 cases.

BACKGROUND

More and more infants with total anomalous pulmonary venous return (TAPVR) has been detected in this hospital (Taichung Veterans General Hospital) in recent years as new diagnostic methods have developed. Several of these methods have been compared to assess their influence on accuracy of diagnosis and the perceived incidence of TAPVR:

METHODS

A retrospective study was made of 36 patients, hospitalized with TAPVR between January, 1983 and June, 1993. All 36 had received 2D echo with color Doppler. Fifteen patients were diagnosed by cardiac catheterization. For the 21 patients who did not receive cardiac catheterization, diagnosis was proved in 16 by operation; in 3, by autopsy; another 2, by 2D echo with color Doppler and magnetic resonance imaging (MRI).

RESULTS

Twenty-five patients were boys and 11, girls. Their ages ranged from 1 day to 6 years with a mean of 3.6 months. Twelve patients were supracardiac type (33%), 18 cardiac type (50%), 5 infracardiac type (14%); and only 1 was mixed (3%). Nine of the 11 girls (82%) were cardiac type, while only 9 (36%) of the 25 boys were cardiac type (P < 0.05). In 4 (80%) of 5 infracardiac-type patients the pulmonary venous returns were obstructed, while in only 8 (26%) of 31 non-infracardiac type patients was there evidence of obstruction (P = 0.06). The 11 patients who did not receive operations died. Operation mortality for isolated TAPVR was 0% (0/9) for the nonobstructed-nonrestrictive type patients, and was 50% (5/10) in the other two types (P < 0.05). Operation mortality in those younger than 30 days was 66.6% (2/3), and in those older than 30 days was 18.8% (3/16) (P = 0.15). Of 10 patients associated with complex heart diseases, 9 (90%) were common atrioventricular canal. Of the 18 patients diagnosed before July 1990, 15 patients received cardiac catheterization; of the 18 diagnosed after August 1990 (13 of whom were simple TAPVR), none had catheterizations. Eight patients received MRI which revealed good images of the pulmonary veins.

CONCLUSIONS

Both echo with color Doppler and MRI are non-invasive tools which can make definite diagnoses of TAPVR: These new methods of diagnosis reveal that this disease is not so rare as was once thought. Cardiac catheterization adds little except risks and costs to diagnosis of simple TAPVR: