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Aortic Branch Aneurysms in Marfan Syndrome
Quick Takes
- Aortic branch aneurysms are present in one quarter of patients with Marfan syndrome (MFS).
- The presence of aortic branch aneurysms in patients with MFS is independently associated with subsequent aortic surgery.
Study Questions:
What is the prevalence and clinical significance of aortic branch aneurysms in patients with Marfan syndrome (MFS)?
Methods:
This study evaluated the presence and clinical significance of aortic branch aneurysms in patients with MFS (FBN1 genetic variant) and prior magnetic resonance or computed tomography imaging of their aortic branches. Their prevalence, patterns, and relationship with subsequent aortic surgery was examined.
Results:
From a cohort of 187 patients with MFS, aortic branch aneurysms were identified in 50 (27%) patients. A total of 104 aneurysms were identified, and 62% of patients with aneurysms had more than one identified. The iliac artery was the most common site of peripheral aneurysms (n = 45). Peripheral aneurysms were observed more frequently in patients with increased age, dyslipidemia, and larger aortic roots (p < 0.05 for each). In a subgroup of 95 patients without prior aortic surgery or dissection, the presence of peripheral aneurysms was independently associated with increased aortic surgery (hazard ratio, 3.4; 95% confidence interval, 1.1-10.3; p = 0.03) over a mean follow-up of 3.3 ± 2.6 years.
Conclusions:
Aortic branch aneurysms were observed in one quarter of patients with MFS, and the presence of these was independently associated with subsequent aortic surgery.
Perspective:
The connective tissue abnormalities of MFS can result in abnormalities in the aortic branch vessels. However, the prevalence of aortic branch aneurysms is not established, and their clinical significance has not been clear. Their presence is not included in diagnostic criteria for MFS, and surveillance imaging that includes the aortic branches has not been included in guideline recommendations. This study finds that aortic branch aneurysms are common. Further, after adjusting for other characteristics, the presence of aortic branch aneurysms was independently associated with future aortic surgery. These findings suggest that surveillance imaging that includes the aortic branches may help identify higher-risk individuals, and these results should be considered in future guidelines. Larger studies with longer-term follow-up could better establish the relationship between aortic branch aneurysms and additional outcomes including potential risk for death and aortic dissection.
Clinical Topics: Cardiac Surgery, Congenital Heart Disease and Pediatric Cardiology, Diabetes and Cardiometabolic Disease, Dyslipidemia, Invasive Cardiovascular Angiography and Intervention, Noninvasive Imaging, Prevention, Vascular Medicine, Aortic Surgery, Cardiac Surgery and Arrhythmias, Cardiac Surgery and CHD and Pediatrics, Congenital Heart Disease, CHD and Pediatrics and Arrhythmias, CHD and Pediatrics and Imaging, CHD and Pediatrics and Interventions, Interventions and Imaging, Interventions and Structural Heart Disease, Interventions and Vascular Medicine, Computed Tomography, Nuclear Imaging
Keywords: Aneurysm, Dissecting, Aorta, Aortic Aneurysm, Cardiac Surgical Procedures, Connective Tissue, Diagnostic Imaging, Dissection, Dyslipidemias, Heart Defects, Congenital, Magnetic Resonance Spectroscopy, Marfan Syndrome, Secondary Prevention, Tomography, X-Ray Computed, Vascular Diseases
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