Prevalence and predictors of intracranial aneurysms in patients with bicuspid aortic valve

Objective To determine the prevalence and outcomes of intracranial aneurysm (IA) in patients with bicuspid aortic valve (BAV). Methods Retrospective review of patients with BAV who underwent brain MR angiography at the Mayo Clinic from 1994 to 2013. Results There were 678 patients included in this study-mean age 57 +/- 13 years, men 480 (71%), mean follow-up 10 +/- 3 years (5913 patient-years). Coarctation of aorta (COA) was present in 154 (23%) patients. There were 59 IAs identified in 52 of 678 patients (7.7%). IA was present in 20/154 patients (12.9%) with COA and 32/524 patients (5.7%) without COA (p<0.001). For the patients without COA, female gender and right-left cusp fusion were risks factors for IA in women after adjustment for all potential variables (HR 1.76, CI 1.31 to 2.68, p=0.03). There was no significant trend in the risk for IA across age tertiles: age <= 40 years versus 41-60 years (HR 1.19, p=0.34), and age 41-60 years versus 61-80 years (HR 1.06, p=0.56). Among the 52 patients with IA, enlargement occurred in three patients (6%), rupture in two patients (4%) and four patients (8%) underwent coil embolisation. For the 626 patients without IA at baseline, no patient developed IA over 7 +/- 2 years of imaging follow-up. Conclusions BAV is associated with a higher prevalence of IA compared to the general population, and this risk is higher in patients with COA, right-left cusp fusion and female gender.