Objective: Bicuspid aortic valve (BAV) is a common congenital heart defect affecting half to 2% of the population. A generalized connective tissue disorder also involving the intracranial arteries has been suspected in this patient population. We therefore screened a group of patients with BAV for the presence of intracranial aneurysms.
Methods: Magnetic resonance angiography or CT angiography of the brain was used in 61 patients with BAV (age, 29-70 years [mean 48 years]) and in 291 controls (28-78 years [mean 56 years]).
Results: Intracranial aneurysms were detected in 6 of 61 patients with BAV (9.8%; 95% confidence interval [CI] 2.4%-17.3%). This was significantly higher than in the control population (3/291 [1.1%; 95% CI 0%-2.2%]) (p = 0.0012). Female sex (p = 0.02) and advanced age (p = 0.003), risk factors for intracranial aneurysm development, were more common in the control population than among the patients with a BAV. No significant differences were detected in age, sex, smoking, arterial hypertension, alcohol use, aortic diameter, or frequency of aortic coarctation between BAV patients with and without intracranial aneurysms.
Conclusion: In this case-control study, the frequency of intracranial aneurysms among our bicuspid aortic valve patient population was significantly higher than in the control population.