Bicuspid aortic valve: a synergistic factor for aortic dilation and dissection in Marfan syndrome?

Patients with either Marfan syndrome or bicuspid aortic valve are at increased risk for aortic dilation and dissection, but occurrence of both conditions has barely been reported. Whether bicuspid aortic valve adversely impacts the cardiovascular outcome in Marfan syndrome patients is unknown. The objective was to investigate the prevalence of bicuspid aortic valve and to define whether its combined presence would adversely impact cardiovascular outcome in patients with Marfan syndrome. We performed a retrospective review on a Marfan syndrome database from a single center. Comparisons between patients with or without bicuspid aortic valve were performed by chi2 or Student t test as appropriate. Bicuspid aortic valve was found in 4 of 89 Marfan syndrome patients (two males; mean age 21.5 +/- 13 years) for 4.5% prevalence; in contrast, 1.5% prevalence was found in 200 control subjects (p = 0.1). Each patient with bicuspid aortic valve is separately discussed. Presence of bicuspid aortic valve shows trends for association with aortic dilation (Odds ratio [OR] 4.2; 95% Confidence interval [95% CI] 0.2-81), and aortic dissection (OR 5.5; 95% CI 0.7-42), while negative association with the Walker-Murdoch sign (OR 0.07; 95% CI 0.006-0.73) was found. Prevalence of bicuspid aortic valve in patients with Marfan syndrome patients is 4.5%. While it is intriguing and even intuitive that the concurrence of both conditions would lead to more aggressive aortic disease, a true synergistic role for aortic wall weakening cannot be supported.