[Total anomalous pulmonary venous connection. Experience with 70 cases].

Purpose: To evaluate the clinical and surgical experience with consecutive patients with isolated total anomalous pulmonary venous connection admitted to the Heart Institute, São Paulo, Brasil.

Methods: Hospital records of 70 consecutive patients (January 1985 to December 1993) were retrospectively reviewed. There were 28 girls and 42 boys whose ages ranged from two days to four years (mean 20.4 +/- 29 weeks). The following variables were selected for analysis: anatomic type, age at admission, clinical status, the presence of infection, restrictive atrial septal defect, and the presence of pulmonary venous obstruction. Univariate and multivariate analysis were used to identify possible risk factors for death.

Results: The abnormal anatomical connection was supracardiac in 63%, cardiac in 17%, infracardiac in 8.5% and mixed in 11.5% of the cases. Eighteen (25.7%) patients were admitted in poor clinical condition. Infection, mainly pulmonar, was present in 31 (44%) patients. Pulmonary venous obstruction was present in 21% of the patients. Atrial septostomy was performed in 38 patients, 13 of them admitted in a poor clinical status. The preoperative mortality was 15.7% and the operative, 17%. Statistical analysis demonstrated that infection, poor clinical status and age were risk factors for death.

Conclusion: Surgical correction of total anomalous pulmonary venous drainage in infancy can be performed at low risk with good results. However early referral to a cardiac care center, agressive preoperative stabilization and postoperative care are important.