TOTAL ANOMALOUS PULMONARY VENOUS CONNECTION - SURGERY - OUTCOME - CHILDREN
Background - Total anomalous pulmonary venous connection (TAPVC) is a rare life threatening congenital heart disease. Without surgical repair, the majority of patients die within the first year of life. We have performed a study on our institutional experience with TAPVC and its surgical correction.
Patients and Methods - A retrospective analysis was carried out in 60 patients, 56 of whom had undergone surgical correction over a 10-year period (1990- 2000) at our department. The group included 35 boys and 21 girls aged from 45 days to 20 years (mean age 4.8 years). TAPVC type was supra-cardiac in 29 patients (52%), cardiac in 18 (32%), infra-cardiac in 5 (9%) and mixed type in 4 (7%). The most common associated cardiac anomaly was atrial septal defect (ASD).
Results - Four of the patients died before surgery, early mortality after surgery was 30% (17 patients) with one late non-cardiac death. Sixty eight percent of the patients are currently well at a mean follow-up of 4.4 years (range 2.4 months to 10 years).
Conclusion - TAPVC is a rare life threatening congenital heart disease. A prompt accurate diagnosis and early surgical repair is life–saving