Aortic Event Rate in the Marfan Population: A Cohort Study

Study Questions:

What is the event rate in a population of patients with the Marfan syndrome (MFS) without pre-existing aortic dissection when optimally managed and followed over time?

Methods:

Patients who fulfilled the international diagnostic criteria for MFS, attended a single-center multidisciplinary clinic at least twice, and had aortic imaging (most commonly echocardiography) at each visit were included. Most of these patients were managed per protocol with beta-blockade and advice on exercise/activity limitations, regardless of aortic size at presentation. Patients were offered surgery when aortic root diameter was >50 mm.

Results:

Of the 1,097 patients followed in this clinic who met diagnostic criteria for MFS, 732 patients (67%) met inclusion criteria. Of these, 47.1% were male and mean age was 24.5 ± 16.4. Mean duration of follow-up was 6.6 ± 4.3 years. Average increase in aortic root size was 0.5 ± 0.9 mm/y. The mean annual risk of death or aortic dissection in the entire cohort was 0.17% (with a 0.05%/year risk of dissection). When stratified by aortic diameter (Ao), the annual risk of death or dissection was 0.1% (Ao up to 39 mm), 0.12% (Ao = 40-44 mm), 0.31% (Ao = 45-49 mm), 1.37% (Ao = 50-54 mm), and 8.14% (Ao >55 mm). Excluded from this study were 92 patients followed in the clinic with a pre-existing dissection, three patients who had a dissection during a period when they were lost to clinical follow-up, and 156 patients with prior aortic surgery.

Conclusions:

The authors concluded that aortic event rate in patients with the MFS, when managed with beta-blockade, sports/physical activity limitations, and regular imaging follow-up, is low when the absolute aortic root diameter is <50 mm.

Perspective:

This analysis is a demonstration of the low event rate in a well-managed population of patients with MFS, and argues against the need for prophylactic surgery at aortic root diameter <50 mm in some patients. However, as the authors discuss, the decision to pursue surgery in higher-risk MFS patients (rapid rate of aortic growth, family history of dissection, desire for pregnancy) is more complex, and often these patients are operated on earlier. As many of these patients were excluded from this study, the reported event rate may be lower than expected. The study does underscore the importance and value of a multidisciplinary protocol approach to the management of these complex patients.

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

Keywords: Follow-Up Studies, Marfan Syndrome, Sports, Motor Activity, Echocardiography


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