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Abdominal Aortic Aneurysm in Marfan Syndrome
Tracy Hagerty1, Patrick Geraghty2, Alan C Braverman1
1Cardiovascular Division, Department of Medicine, Washington University School of Medicine, St. Louis, MO.
Annals of Vascular Surgery
|November 30, 2016
Summary
Adults with Marfan syndrome (MFS) have an increased risk of developing abdominal aortic aneurysms (AAAs). Screening for AAA is recommended in MFS patients with prior aortic root replacement, especially those with other aneurysms or a history of smoking.
Area of Science:
- Cardiovascular Medicine
- Genetics
- Vascular Surgery
Background:
- Marfan syndrome (MFS) commonly causes aortic root aneurysms.
- Abdominal aortic aneurysms (AAAs) are rarely reported in MFS patients.
- Risk factors and screening guidelines for AAA in MFS are not well-established.
Purpose of the Study:
- To characterize abdominal aortic aneurysms (AAAs) in adult patients with Marfan syndrome (MFS).
- To identify potential risk factors and clinical features associated with AAA in MFS.
Main Methods:
- Retrospective review of 12 adult patients with MFS and AAA.
- Analysis of clinical data, imaging, operative reports, and patient outcomes.
Main Results:
- The average age at AAA diagnosis was 44 years; 9/12 patients were smokers.
- Eleven patients had prior aortic root replacement; AAA size averaged 5.0 cm.
- Two patients had descending thoracic aortic aneurysms; 7 had branch vessel aneurysms.
Conclusions:
- Adults with MFS are at risk for developing AAA.
- AAA evaluation is recommended for MFS patients with prior root repair, especially if other aneurysms or smoking history are present.
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