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

Objective: An ever increasing number of patients with an operated congenita l cardiac anomaly is reaching adulthood. Insight in the longterm characteri stics of these patients is steadily growing for the more common anomalies, but is still scarce for the less frequent entities. In this regard we repor t our results and long-term follow-up of surgically treated total anomalous pulmonary venous return (TAPVR). Methods: Since 1973 44 patients with TAPV R were treated with surgery. There were 23 girls and 21 boys. Hospital mort ality concerned six patients and additionally one patient died 2.5 months a fter surgery. Follow-up data could be collected from all the surviving pati ents at last outpatient visit or by phone contact. The mean duration of the follow-up was 12 years (range 1 month to 24 years). Results: The Kaplan-Me ier 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 o ne patient this involved obstruction of pulmonary venous return, in four co mplications of the primary repair. The reoperation-free survival at 15 year s was 74% (95% CI 61-87%). At the end of follow-up all survivors were in NY HA functional class I and, at appropriate age, normally attended school or were employed. None of the patients was using cardiac medication. Conclusio ns: We conclude that, despite a possible hectic initial postoperative cours e 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. (C) 1999 Elsevier Science B.V. All r ights reserved.