Clinical Study on Total Anomalous Pulmonary Venous Return

Han, Dong Kyun; Chung, Hae Yul; Ma, Jae Sook

Abstract

Total anomalous pulmonary venous return (TAPVR) defines the anomaly in which the pulmonary veins drain abnormally into the right atrium or one of the venous tributaries leading to the right atrium. The clinical patterns and prognosis in TAPVR are variable, depending on whether there are obstruction to the pulmonary venous return, pulmonary hypertension, and associated cardiac anomalies. If untreated, death usually occurs within 1 year of age. We retrospectively evaluated 30 patients who were diagnosed as TAPVR by echocardiogram from October 1990 to April 2005 at the Department of Pediatrics, Chonnam National University Hospital. The results were as follows: 1) The ratio for male to female was 1.7 : 1 (19 boys, 11 girls). 2) Age at initial diagnosis ranged from 2 days to 264 days. The signs and symtoms were variable with cyanosis, feeding difficulty, irritability, failure to thrive, and so on. 3) The anatomic types of TAPVR were supracardiac (type 1), in 16 of 30 cases (53.3%); cardiac (type 2), in 5 cases (16.7%); infracardiac (type 3), in 5 cases (16.7%); mixed (type 4), in 4 cases (13.3%). The most common type was the supracardiac. 4) Obstructed TAPVR were found in 10 of 30 cases (33.3%): supracardiac type, in 4 of 16 cases (25.0%); cardiac type, in 1 of 5 cases (20.0%); infracardiac type, in 4 of 5 cases (80.0%); mixed type, in 1 of 4 cases (25.0%). In our series, pulmonary venous obstruction seemed more common in the infracardiac types, although not significant. 5) There were 8 deaths (8 of 30 patients; 26%): 4 died before operation. The overall postoperative mortality was 13.3% (4 of 15 patiens); three died unsuspectedly, one died of the failed relieving of the recurrent pulmonary vein stenosis after reoperation. In conclusion, the diagnosis of TAPVR tends to be delayed depending on the varying clinical manifestations, which may lead to an early death, frequently combined with pulmonary venous obstruction. Therefore, early diagnosis and surgical intervention, combined with close attention to pre- and postoperative managements and to the occurrence of progressive postoperative pulmonary venous obstruction cannot be over-emphasized for reducing the mortality rate.