Conclusion: Patients with Marfan syndrome who have ascending aorta diameters of Ͻ50 mm have a low risk of sudden death or aortic dissection. Fifty millimeters is a reasonable threshold for prophylactic aortic surgery in patients with Marfan syndrome. Summary: During the last 30 years, the life expectancy for patients with Marfan syndrome has increased by 30 years (Pyeritz RE, et al, Heart 2009;95:173-5). Improvement of survival in patients with Marfan syndrome is likely multifactorial but includes more widespread practice of routine imaging and timed prophylactic aortic surgery. Two recent task forces have proposed a 50-mm aortic diameter at the level of the sinus of Valsalva as the cutoff value for performing ascending aortic surgery; however, no value has been specifically proposed for patients with Marfan syndrome. All patients in this study fulfilled international criteria for Marfan syndrome and had not had previous aortic surgery or dissection. Patients must have come to the authors' center at least two times to be included in this study. Echocardiography was used to make aortic measurements every 2 years, and patients were given systemic  blockade and advised to avoid intense sports activity. When the aortic root maximum diameter reached 50 mm, prophylactic surgery was proposed. There were 732 patients with Marfan syndrome followed up for a mean of 6.6 years. During follow-up, two dissections and five deaths occurred. Event rate (death/aortic dissection) was 0.17% per year. Risk increased if the aortic diameter increased within 2 years of the event, from 0.09% per year (95% confidence interval [CI], 0.00%-0.20%) when the aortic diameter was Ͻ40 mm to 0.3% (95% CI, 0.00%-0.71%) with aortic diameters of 45 to 49 mm and to 1.3% (95% CI, 0.00%-3.93%) with aortic diameters of 50 to 54 mm. Aortic diameters Ͼ50 mm increased the risk four times. The annual risk was Ͻ0.05% when the aortic diameter was Ͻ50 mm after the authors excluded a neonatal patient, a woman who became pregnant, and a 72-year-old woman with previous myocardial infarction. Comment: The results suggest that aortic dissection in patients with Marfan syndrome can be almost entirely prevented with the use of systemic -blockers, avoidance of intense sporting activities, and scheduled surgery when the aortic root reaches 5 cm in diameter. Patients with Marfan syndrome can do very well with strict compliance with medical advice and timed prophylactic surgery.
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