OBJECTIVES: The Risk Adjusted Classification for Congenital Heart Surgery can predict early mortality. However, the relation to long-term outcome in terms of mortality and morbidity is unknown.

DESIGN: We did a population-based follow-up study of 801 children undergoing congenital heart surgery between 1996 and 2002. All patients were followed from surgery until death or January 1, 2008. Operations were classified according to the Risk Adjusted Classification for Congenital Heart Surgery. Each patient was matched by age and sex with 10 population controls. Cox regression analysis, area under the receiver operator curve and competing risk analysis were used for the analyses.

RESULTS: Overall follow-up was 99.6%. The distribution of the Risk Adjusted Classification for Congenital Heart Surgery was: Category one 20%, category two 37%, category three 27%, category four 8%, category five 0% and category six 2%. Overall survival after a median follow-up of 8.2 years was 86% (95% confidence interval: 83-88%), with 54 early deaths occurring within 30 days after surgery and 57 late deaths. Long-term survival in those who were alive 30 days after surgery was 92% (90-94%); ranging from 98% (93-100%) in risk category one to 33% (5-68%) in category six. Survival overall and beyond 30 days was lower in each risk category than in controls (P < .001). During follow-up, 124 (15%) patients had new operations and 106 (13%) catheter-based interventions. These events were more frequent in category three, four, and six compared with category one, with no difference between category one and two. The area under the receiver operator curve for long-term mortality was 0.81 (95% confidence interval 0.75-0.87).

CONCLUSIONS: Children operated for congenital heart disease have impaired survival and often undergo new operations or catheter-based interventions. The risk of these events is related to the surgical complexity according to the Risk Adjusted Classification for Congenital Heart Surgery.