The results in the management of 36 cases of isolated total anomalous pulmonary venous connection are presented. This patients are the 1.55% of the 2,322 children diagnosed of congenital heart disease by catheterization and angiography in our hospital at the 1971-1988 period. Nineteen had a type I, five a type II, eight a type III and four a mixed type lesion. The total mortality rate was 63.8%. Twenty six infants underwent surgical correction at a mean age of 2.5 months with an operative mortality rate of the 57.7%. Eleven infants survived operation. There have been no late deaths but one children developed stenosis at the anastomosis. The final actuarial survival rate was the 34.4% in the total group and the 42.3% in the operated group. The strongest determinants of survival were the type of lesion and pulmonary venous obstruction. No improving in the total and the surgical results has been observed in the last 10 years.
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