Aortic Event Rate in the Marfan Population: A Cohort Study
Background—Optimal management, including timing of surgery, remains debated in Marfan syndrome (MFS), due to lack of data on aortic risk associated with this disease.
Methods and Results—We used our database to evaluate aortic risk associated with standardised care. Patients who fulfilled the international criteria, had not had previous aortic surgery or dissection and who came to our centre at least twice were included. Aortic measurements were made using echocardiography (every 2 years); patients were given systematic beta-blockade and advice about sports activities. Prophylactic aortic surgery was proposed when the maximal aortic diameter reached 50 mm. 732 patients with MFS were followed up for a mean of 6.6 years. Five deaths and two dissections of the ascending aorta occurred during follow-up. Event rate (death/aortic dissection) was 0·17% per year. Risk rose with increasing aortic diameter measured within 2 years of the event: from 0·09% per year (95% confidence interval 0.00-0·20) when the aortic diameter was <40 mm, to 0·3% (0·00-0·71) with diameters of 45-49 mm, and 1·33% (0.00-3·93) with diameters of 50-54 mm: the risk increased 4-times above 50 mm. The annual risk dropped below 0·05% when the aortic diameter was <50 mm, after exclusion of a neonatal patient, a woman who became pregnant against our recommendation, and a 72-year-old woman with previous myocardial infarction.
Conclusions—Risk of sudden death or aortic dissection remains low in patients with MFS and aortic diameter between 45 and 49 mm. 50 mm appears to be a reasonable threshold for prophylactic surgery.
- Received July 8, 2011.
- Accepted November 18, 2011.
- Copyright © 2011, American Heart Association, Inc. All rights reserved. Unauthorized use prohibited