Survival trends of adults with congenital heart disease after heart surgery in Sweden.

OBJECTIVE

Congenital heart disease is the most common congenital anomaly. This study evaluated long-term mortality in patients who underwent childhood heart surgery and survived to adulthood.

METHODS

Using Swedish national registries, we identified 24,774 adults with congenital heart disease born between 1970 and 1999 who were alive at 18 years. Of these, a total of 7585 underwent childhood heart surgery and were matched with 54,540 controls by birth year and sex. Follow-up began at age 18 years and extended until death or study end (2017). Survival was assessed using Kaplan-Meier estimates and Cox proportional hazards models.

RESULTS

Over a mean follow-up of 12.7 years, 227 patients (3.0%) died, with a hazard ratio of 5.02 (95% CI, 4.23-5.95, P < .001). Complex lesions had a higher hazard ratio of 7.03 (95% CI, 5.45-9.06, P < .001), peaking at 8.27 (95% CI, 6.13-11.16, P < .001) for conotruncal defects. In noncomplex lesions, ventricular septal defects had a hazard ratio of 5.03 (95% CI, 3.20-7.92, P < .001). Mortality risk was highest for those born in the 1970s, standing at 5.95 (95% CI, 4.67-7.58, P < .001), with improving survival in subsequent decades.

CONCLUSIONS

Patients with congenital heart disease surviving childhood heart surgery had a 5-fold higher mortality risk compared with controls, particularly the complex lesion groups. Even though being noncomplex, ventricular septal defects showed a 5-fold higher mortality risk than controls. These patients were repaired but not cured and in need of lifelong follow-up. However, survival has significantly improved for those born in the 1990s, now comparable to matched controls up to 10 years.