Long-Term Risk of Hemorrhagic Stroke in Young Patients With Congenital Heart Disease

Background and Purpose-The risk of ischemic stroke is increased in patients with congenital heart disease (CHD); however, data on the risk of hemorrhagic stroke, including intracerebral hemorrhage (ICH) and subarachnoid hemorrhage (SAH), are lacking. Methods-The Swedish Patient Register was used to identify all patients who were born with a diagnosis of CHD between 1970 and 1993. Each patient was compared with 10 randomly selected controls from the general population, matched for age, sex, and county. Follow-up data were collected until December 2011 for both cases and controls. Results-Of 21 982 patients with CHD, 70 developed ICH and 57 developed SAH up to the age of 42 years. CHD patients had more than an 8x higher risk (incidence rate ratio, 8.23; 95% confidence interval, 6-11.2) of developing ICH and almost an 8x higher risk of developing SAH (incidence rate ratio, 7.64; 95% confidence interval, 5.41-10.7) compared with controls. The absolute risk of ICH and SAH was low, with incidence rates of 1.18 and 0.96 cases per 10 000 person-years, respectively. Patients with severe nonconotruncal defects (incidence rate ratio, 16.5; 95% confidence interval, 5.63-51.2) or coarctation of the aorta (incidence rate ratio, 17.3; 95% confidence interval, 6.63-51.8) had the highest relative risk of developing hemorrhagic stroke, with incidence rates of 3.22 and 2.79 cases per 10 000 person-years, respectively. Conclusions-The relative risk of hemorrhagic stroke among children and young adults with CHD was almost 8x higher than that of matched controls from the general population, although the absolute risk was low. The highest risk of ICH and SAH occurred in patients with severe nonconotruncal defects and coarctation of the aorta.