Aortic Event Rate in the Marfan Population: A Cohort Study

Study Questions:

What is the annual aortic event rate as a function of aortic root diameter and the optimal timing for surgery?

Methods:

The investigators used their database to evaluate aortic risk associated with standardized care. Patients who fulfilled the international criteria, had not had previous aortic surgery or dissection, and came to their center at least twice were included. Aortic measurements were made with 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. The annual early aortic event rate was calculated as the ratio of the number of aortic events divided by the number of patient-years for each range of aortic diameter.

Results:

Seven hundred thirty-two patients with Marfan syndrome 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%/year. Risk rose with increasing aortic diameter measured within 2 years of the event: from 0.09%/year (95% confidence interval, 0.00-0.20) when the aortic diameter was <40 mm to 0.3% (95% confidence interval, 0.00-0.71) with diameters of 45-49 mm and 1.33% (95% confidence interval, 0.00-3.93) with diameters of 50-54 mm. The risk increased four times at diameters ≥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 their recommendation, and a 72-year-old woman with previous myocardial infarction.

Conclusions:

The authors concluded that the risk of sudden death or aortic dissection remains low in patients with Marfan syndrome and aortic diameter between 45 and 49 mm, and an aortic diameter of 50 mm appears to be a reasonable threshold for prophylactic surgery.

Perspective:

The primary finding of this study is the low rate of aortic events in a population diagnosed with Marfan syndrome according to the international criteria when current recommendations are applied (i.e., systematic beta-blockade, advice about sports and physical activity, regular aortic measurements with echocardiography, and prophylactic aortic root surgery for an absolute aortic diameter of 50 mm). Therefore, 50 mm aortic diameter is a reasonable threshold for prophylactic surgery in the absence of specific risk factors (e.g., family history of dissection with mild dilatation) and highlights the importance of early diagnosis based on familial screening to start optimal medical care.

Clinical Topics: Cardiac Surgery, Congenital Heart Disease and Pediatric Cardiology, Noninvasive Imaging, Cardiac Surgery and CHD and Pediatrics, Congenital Heart Disease, CHD and Pediatrics and Imaging, CHD and Pediatrics and Quality Improvement, Echocardiography/Ultrasound

Keywords: Myocardial Infarction, Follow-Up Studies, Death, Cardiology, Marfan Syndrome, Cardiovascular Diseases, Risk Factors, Echocardiography


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