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Review
. 2019 Apr 4:10:329.
doi: 10.3389/fneur.2019.00329. eCollection 2019.

Adults With Complex Congenital Heart Disease: Cerebrovascular Considerations for the Neurologist

Jonathan M C Smith  1 , Jason G Andrade  2 , Derek Human  3 , Thalia S Field  1   2   3
Affiliations
Review

Adults With Complex Congenital Heart Disease: Cerebrovascular Considerations for the Neurologist

Jonathan M C Smith et al. Front Neurol. .

Abstract

As infant and childhood mortality has decreased in congenital heart disease, this population is increasingly reaching adulthood. Adults with congenital heart disease (ACHD) represent a group with increased risk of stroke, silent brain infarcts, and vascular cognitive impairment. Cyanotic and other complex cardiac lesions confer the greatest risk of these cerebrovascular insults. ACHD patients, in addition to having an increased risk of stroke from structural cardiac issues and associated physiological changes, may have an accelerated burden of conventional vascular risk factors, including hypertension and impaired glucose metabolism. Adult neurologists should be aware of the risks of clinically evident and subclinical cerebrovascular disease in this population. We review the existing evidence on primary and secondary stroke prevention in individuals with complex congenital heart disease, and identify knowledge gaps in need of further research, including treatment of acute stroke in this population. Multisystemic genetic syndromes are outside the scope of this review.

Keywords: Adults with Congenital Heart Disease (ACHD); Cardiovascular Disease; Cerebrovascular disease; Congenital Heart Disease (CHD); cognition; cyanotic; fontan; stroke.

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Figures

Figure 1
Figure 1
Improved survival of people affected by CHD [data from Knowles et al. (23)].
Figure 2
Figure 2
Relative prevalence of congenital heart lesions [data from Hoffman and Kaplan (13)].
Figure 3
Figure 3
Mechanisms of cerebrovascular disease in ACHD.
See this image and copyright information in PMC

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