Background Patients with congenital heart disease (CHD) are at increased risk of developing ischemic stroke (IS) compared with controls without CHD. However, the long-term outcomes after IS, including IS recurrence and mortality risk, remain unclear. Methods and Results We identified all patients with CHD in Sweden who were born between 1930 and 2017 using the Swedish National Patient Register and the Cause of Death Register. Ten controls without CHD were randomly selected from the general population and matched for birth year and sex for each patient with CHD. The follow-up of the study population was performed between January 1970 and December 2017. In total, 88 700 patients with CHD (50.6% men) and 890 450 matched controls (51.0%) were included in this study. During a mean follow-up of 25.1±22.0 years, patients with CHD had a 5-fold higher risk of developing an index IS (hazard ratio [HR], 5.01; 95% CI, 4.81-5.22) compared with controls. However, the risk of developing a recurrent IS was lower in patients with CHD compared with controls (HR, 0.66; 95% CI, 0.56-0.78), an observation that persisted after adjustment for cardiovascular risk factors and comorbidities. Patients with CHD were also at a significantly lower risk of all-cause mortality after index IS than controls (HR, 0.53; 95% CI, 0.49-0.58). Conclusions Patients with CHD had a 5-fold higher risk of developing index IS compared with matched controls. However, the risk of recurrent IS stroke and all-cause mortality were 34% and 47% lower, respectively, in patients with CHD compared with controls.
Keywords: congenital heart disease; ischemic stroke; mortality; recurrent stroke.