Intracranial aneurysms and cervicocephalic arterial dissections associated with congenital heart disease

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 or 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.