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.
Keywords: adults with congenital heart disease; congenital heart disease; congenital heart surgery; long-term outcome.
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