Abstract 12688: Abdominal Aortic Aneurysm in Marfan Syndrome: Time for Screening Guidelines?

Abstract

Background: Marfan syndrome (MFS) leads to aortic root aneurysm, while descending thoracic aortic aneurysm (TAA) occurs less commonly. With imaging surveillance and prophylactic surgery, lifespan in MFS has increased to near normal. Abdominal aortic aneurysm (AAA) complicating Marfan syndrome is rarely reported in the literature. There are no guidelines for routine screening for AAA in MFS. We sought to characterize AAA disease in our MFS cohort. Methods: The records of 11 adults from our MFS Clinic with MFS (by Ghent or revised Ghent Criteria) and AAA disease were reviewed. Clinical features, imaging, operative reports, and outcomes were analyzed. Results: Eleven adults (9 male) with MFS and AAA, age at AAA diagnosis 43 + 15 yrs (range 18-63 yrs), were studied. Five patients smoked cigarettes. Nine of eleven patients were being treated with beta-blockers and angiotensin receptor blockers or ACE-inhibitors at the time of AAA diagnosis. Ten underwent prior aortic root replacement at age 31 + 15 yrs (range 8-62 yrs). The AAA was diagnosed 1-21 years after aortic root repair. The AAA was suprarenal in 5 patients, juxtarenal in 2, and infrarenal in 4. The size of the AAA ranged from 3.5 to 9.7 cm at time of diagnosis. Three patients had descending TAA. One patient had a prior type B dissection, not involving the abdominal aorta. Branch vessel aneurysms present in these patients included popliteal, intercostal, hepatic, subclavian and axillary. Four patients underwent open surgical repair (AAA size from 5.5 to 9.7 cm); one underwent endovascular repair; and five are being treated medically (aortic size 4.1 + 0.6 cm). One patient died suddenly with AAA size 5.7 cm at last measurement. Conclusions: Adults with MFS are at risk for developing AAA. Our cohort is the largest to date characterizing AAA in MFS. The AAAs were often very large at the time of initial diagnosis. MFS patients with AAA have a relatively high frequency of branch vessel aneurysms. Imaging to evaluate for AAA is recommended early after the diagnosis of MFS in adults and periodically thereafter to improve outcomes in this population.