Patients with Marfan syndrome displayed higher rates of aortic complications in long-term follow-up after aortic valve replacement (AVR) than patients with bicuspid aortic valve disease, according to results of a study published June 1 in the Journal of the American College of Cardiology.

In a retrospective comparison, Shinobu Itagaki, MD, et al. assessed the long-term follow-up of thoracic aortopathy after AVR in patients with bicuspid aortic valve disease and those with Marfan syndrome. The study compared the outcomes of 13,205 patients—2,079 with bicuspid aortic valves and 73 with Marfan syndrome—who had AVR replacement between 1995 and 2010.

The results of the study showed that patients with Marfan syndrome were 14 times more likely to present with aortic dissection during long-term follow-up and five times more likely to undergo thoracic aortic surgery. The authors of the study note that these results “provide additional support for the discrete treatment algorithms for patients with bicuspid valve disease,” as the operative management of these patients has previously been extrapolated based on Marfan syndrome patients.

In an accompanying editorial comment, Himanshu J. Patel, MD, FACC, writes that “the fundamental lack of knowledge regarding bicuspid aortic valve disease suggests that there is a need for a prospectively-collected, clinically-robust database to further evaluate the natural history and genetic underpinnings of this complex, heterogeneous pathology.”

Keywords: Aorta, Aortic Aneurysm, Aortic Valve, Heart Valve Diseases, Marfan Syndrome, Mitral Valve

< Back to Listings