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Risk of Type B Dissection in Marfan Syndrome
Quick Takes
- In a large, single-center, retrospective and prospective registry of patients with Marfan syndrome (MFS) seen between 1982 and 2023, type B dissection occurred more commonly than did type A dissection (14% vs. 9%).
- Among patients with available pre-dissection surveillance imaging, type B dissection occurred in the setting of a normal or minimally dilated descending thoracic aorta in 87% of cases. Together with an association between type B dissection and elective aortic root replacement and mitral valve surgery, this is suggestive of a clinically more severe MFS phenotype.
Study Questions:
What are the determinants of type B aortic dissection risk in patients with Marfan syndrome (MFS)?
Methods:
The Cornell Aortic Aneurysm Registry is a large, single-center, retrospective and prospective registry based on data from medical records reviewed to identify patients with MFS seen between 1982 and 2023. Demographic and anthropometric features, cardiovascular disease, and surgical history were compared in patients with MFS with and without type B dissection.
Results:
Of 336 patients with MFS, 47 (14%) experienced a type B dissection and 30 (9%) experienced a type A dissection; after excluding patients with type I dissection or distal anastomosis dissection, the study population comprised 308 patients. Elective aortic root replacement (ARR) was performed in 163 patients. Patients with type B dissection more commonly underwent ARR before or after the type B dissection (37 of 47 patients [79%]) compared to patients without type B dissection (120 of 261 patients [46%], p < 0.001); of the 47 patients with type B dissection and elective ARR, the dissection occurred after ARR in 26 (55%; median duration 13.3 years after valve-sparing root and 20.8 years after valve replacing root repair) and before ARR in 10 (21%). Forty-one patients (87%) were aware of an MFS diagnosis before type B dissection. Among 24 patients with pre-dissection imaging, the descending aorta was normal (<3.0 cm, 10 patients) or minimally dilated (3.0-3.9 cm, 11 patients) in 88%. In multivariable analyses, patients with type B dissection were more likely to have undergone or undergo elective ARR or mitral valve surgery independent of elective ARR, to have had a type II dissection, and to have lived longer.
Conclusions:
In this cohort, type B dissections were more common than type A dissections and occurred below traditional surgical thresholds. The associations of type B dissection with ARR, mitral valve surgery independent of elective ARR, and type II dissection suggest a more severe phenotype in the setting of a longer life expectancy.
Perspective:
In the setting of more aggressive prophylactic intervention for ascending aorta repair in patients with MFS, this study found that type B dissection occurred more often than did type A dissection. The occurrence of type B dissection in the setting of a normal or minimally dilated descending thoracic aorta and the association of type B dissection with ARR and with mitral valve surgery is suggestive of a clinically more severe MFS phenotype.
Clinical Topics: Cardiac Surgery, Congenital Heart Disease and Pediatric Cardiology, Vascular Medicine, Cardiac Surgery and CHD and Pediatrics, Congenital Heart Disease
Keywords: AHA23, Aneurysm, Dissecting, Marfan Syndrome
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