[Total anomalous pulmonary venous drainage: 29 years of experience in a single institution]

Total anomalous pulmonary venous drainage (TAPVD) is a rare disease, with an approximate incidence of 0.058 to 0.083:1,000 live births. It has a mortality rate of around 80% to 90% in the first year, if not treated early.

Using the 29-year experience of a single center, possible variables related to hospital death were tested.

Retrospective analysis of 123 consecutive patients with a diagnosis of TAPVD that underwent surgical treatment between January 1979 and March 2008. Only patients with isolated TAPVD were included and the complex associations were excluded. The variables were tested for the identification of their influence on death and of the interference of temporal evolution.

The medians of weight and age were 4.1 kg and 120 days, respectively. The supracardiac anatomic subtype was the most prevalent throughout the analyzed period. Obstruction to drainage was present in 30% of the cases and the infracardiac subtype was the most frequently associated form. The general mortality rate was 27%, being equal to zero in the last five years. At both the univariate and multivariate analysis, the presence of obstruction to pulmonary venous flow was the only variable positively associated to the deaths (p = 0,005), regardless of the temporal evolution. The mortality among these cases was 44%.

After three decades of evolution, an improvement in the early mortality rate of TAPVD can be observed in our service, which currently presents levels consistent with the literature. The obstruction to pulmonary drainage is still a bad prognostic factor in our country.