Early results and long-term follow-up after corrective surgery for total anomalous pulmonary venous return

An ever increasing number of patients with an operated congenital cardiac anomaly is reaching adulthood. Insight in the long-term characteristics of these patients is steadily growing for the more common anomalies, but is still scarce for the less frequent entities. In this regard we report our results and long-term follow-up of surgically treated total anomalous pulmonary venous return (TAPVR).

Since 1973 44 patients with TAPVR were treated with surgery. There were 23 girls and 21 boys. Hospital mortality concerned six patients and additionally one patient died 2.5 months after surgery. Follow-up data could be collected from all the surviving patients at last outpatient visit or by phone contact. The mean duration of the follow-up was 12 years (range 1 month to 24 years).

The Kaplan-Meier estimate of survival at 15 years was 84% (95% CI 73-95%). Reoperations were performed for five patients at 7 days to 4.5 years after surgery. In one patient this involved obstruction of pulmonary venous return, in four complications of the primary repair. The reoperation-free survival at 15 years was 74% (95% CI 61-87%). At the end of follow-up all survivors were in NYHA functional class I and, at appropriate age, normally attended school or were employed. None of the patients was using cardiac medication.

We conclude that, despite a possible hectic initial postoperative course of surgery for TAPVR, only a limited number of reoperations is necessary relatively short after initial surgery. The long-term perspective for those surviving surgical treatment is good.