Aortic event rate in the Marfan population: a cohort study

Area of Science:

• Cardiology
• Genetics
• Vascular Surgery

Background:

• Marfan syndrome management, particularly surgical timing, lacks robust data on aortic risks.
• Aortic complications are the primary cause of morbidity and mortality in Marfan syndrome.

Purpose of the Study:

• To evaluate aortic risk in Marfan syndrome patients under a standardized care protocol.
• To determine an optimal threshold for prophylactic aortic surgery in Marfan syndrome.

Main Methods:

• Retrospective analysis of 732 Marfan syndrome patients meeting international criteria.
• Standardized care included echocardiography every 2 years, beta-blockade, and activity counseling.
• Prophylactic surgery was recommended at a maximal aortic diameter of 50 mm.

Main Results:

• The overall event rate for death or aortic dissection was 0.17% per year.
• Aortic dissection risk increased significantly with aortic diameter, particularly at ≥50 mm.
• Risk was low (0.09%/year) for diameters <40 mm and 0.3%/year for 45-49 mm.

Conclusions:

• Aortic dissection risk remains low in Marfan syndrome patients with aortic diameters between 45 and 49 mm.
• An aortic diameter of 50 mm is a reasonable threshold for considering prophylactic aortic surgery.
• Standardized medical and surgical management can effectively mitigate aortic risks in Marfan syndrome.