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Intracranial aneurysms and cervicocephalic arterial dissections associated with congenital heart disease
W I Schievink1, B Mokri, D G Piepgras
1Department of Neurologic Surgery, Mayo Clinic, Rochester, Minnesota, USA.
Insights
Patients with congenital heart disease (CHD) face increased risks of intracranial aneurysms and cervicocephalic arterial dissections, especially during adolescence. A potential neural crest abnormality may link these conditions.
Area of Science:
- Neurology
- Cardiology
- Genetics
Background:
- The increasing survival of patients with congenital heart disease (CHD) into adulthood necessitates understanding associated long-term health risks.
- Cerebrovascular abnormalities, including intracranial aneurysms and cervicocephalic arterial dissections, can occur in patients with CHD.
Purpose of the Study:
- To review the experience with noninfectious intracranial aneurysms and cervicocephalic arterial dissections in patients with CHD.
- To expand the understanding of cerebrovascular abnormalities in this growing patient population.
Main Methods:
- A retrospective review of patients with CHD and intracranial aneurysms or cervicocephalic arterial dissections evaluated at the Mayo Clinic from 1969 to 1992.
- Identification and analysis of patient demographics, cardiac diagnoses, and cerebrovascular conditions.
Main Results:
- Congenital heart disease was identified in 8% of children and 0.3% of adults with intracranial aneurysms, and in 4% of children and 2% of adults with cervicocephalic arterial dissections.
- The 14 study patients had a mean age of 32 years and various complex congenital heart disorders, including coarctation of the aorta and bicuspid aortic valve.
- Only one patient presented with both an intracranial aneurysm and coarctation of the aorta.
Conclusions:
- A variety of congenital heart disorders are associated with an increased risk of intracranial aneurysm and cervicocephalic arterial dissection, particularly in adolescents.
- A potential shared pathogenetic factor, possibly an abnormality of neural crest cells, may underlie the association between CHD and these cerebrovascular conditions.
Objective:
The number of patients with congenital heart disease who survive to adolescence and adulthood continues to increase. We review our experience with noninfectious intracranial aneurysms and cervicocephalic arterial dissections in patients with congenital heart disease, expanding the clinical spectrum of the cerebrovascular abnormalities that may be encountered in this group of patients.
Methods:
All patients with congenital heart disease and intracranial aneurysms of cervicocephalic arterial dissections, who were evaluated at the Mayo Clinic between 1969 and 1992, were identified.
Results:
Congenital heart disease was diagnosed in 3 (8%) of 36 children with intracranial aneurysms, in 5 (0.3%) of 1994 adults with intracranial aneurysms, in 1 (4%) of 25 children with cervicocephalic arterial dissections, and in 5 (2%) of 250 adults with cervicocephalic arterial dissections. The mean age of the 14 patients was 32 years (range, 13-58 yr). The congenital heart disorders consisted of complex cardiac anomalies in three patients (truncus arteriosus, transposition of the great arteries, and tricuspid atresia in one patient each), pulmonic valve or arterial stenosis in two patients, aortic coarctation in four patients, and bicuspid aortic valve in five patients. Only one patient had an intracranial aneurysm and coarctation of the aorta.
Conclusion:
Individuals with a variety of congenital heart disorders may be at an increased risk of intracranial aneurysm development and cervicocephalic arterial dissection, particularly in adolescence. The muscular arteries of the head and neck are derived from neural crest cells and the neural crest is also of major importance in early cardiac development, suggesting that an abnormality of the neural crest may be the common pathogenetic factor explaining this association.