Aortic Valve Replacement in Bicuspid Aortic Valve Disease versus Marfan Syndrome

JACC in a Flash | Patients with Marfan syndrome have 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 JACC.

In this retrospective comparison, researchers compared long-term outcomes of 2,079 patients with bicuspid aortic valves, 73 patients with Marfan syndrome, and a control group of 11,053 patients with acquired aortic valves disease undergoing primary AVR without concomitant thoracic aortic surgery between 1995 and 2010. The primary outcome measure was the cumulative incidence of thoracic aortic dissection or rupture, while secondary outcome measures included the cumulative incidence of thoracic aortic aneurysm (TAA) and thoracic aortic surgery.

At the 15-year follow-up, patients with Marfan syndrome were 14 times more likely to present to the hospital with aortic dissection than those with bicuspid aortic valve disease. Marfan syndrome patients were also five times more likely to undergo thoracic aortic surgery and twice as likely to receive a new diagnosis of TAA.

“Our study provides comparative data that highlight the significant differences in long-term outcomes between these two very different clinical entities,” the authors write. They add that the “data suggest that a liberal approach to aortic resection extrapolated from experience in Marfan syndrome exposes the majority of patients with bicuspid valve to incremental operative risk without clear evidence of long-term benefit.”

In an ACC Journal Scan, David S. Bach, MD, adds: “This important study suggests that bicuspid aortopathy has fewer aortic complications compared to patients with Marfan syndrome, and that guidelines for management should be specific to the underlying condition rather than extrapolated from the Marfan population.”


  1. Itagaki S, Chikwe JP, Chiang YP, et al. J Am Coll Cardiol. 2015;65(22):2363-9.

Clinical Topics: Cardiac Surgery, Congenital Heart Disease and Pediatric Cardiology, Valvular Heart Disease, Aortic Surgery, Cardiac Surgery and CHD and Pediatrics, Cardiac Surgery and VHD, Congenital Heart Disease

Keywords: CardioSource WorldNews Interventions, Aortic Valve, Heart Valve Diseases, Bicuspid, Marfan Syndrome

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