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  5. Risk of Type B Dissection in Marfan Syndrome: The Cornell Aortic Aneurysm Registry

Risk of Type B Dissection in Marfan Syndrome: The Cornell Aortic Aneurysm Registry

File(s)
37930285.pdf (892.9 KB)
Permanent Link(s)
https://hdl.handle.net/1813/126857
Collections
Department of Cardiothoracic Surgery
Author
Narula, Nupoor
Devereux, Richard B.
Arbustini, Eloisa
Ma, Xiaoyue
Weinsaft, Jonathan W.
Girardi, Leonard
Malonga, Grace P.
Roman, Mary J.
Abstract

BACKGROUND: With preventive aortic grafting decreasing the incidence of type A dissections in Marfan syndrome (MFS), most dissections are now type B, for which risk factors remain largely uncertain. OBJECTIVES: We explored the determinants of type B dissection risk in a large, single-center MFS registry. METHODS: 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 (vs type A in 9%). Patients with type B dissection were more likely to have undergone elective aortic root replacement (ARR) (79 vs 46%; P < 0.001). Of the patients, 55% had type B dissection a mean of 13.3 years after ARR, whereas 45% experienced type B dissection before or in the absence of ARR; 41 patients (87%) were aware of their MFS diagnosis before type B dissection. Among those with predissection imaging, the descending aorta was normal or minimally dilated (<4.0 cm) in 88%. In multivariable analyses, patients with type B dissection were more likely to have undergone ARR and independent mitral valve surgery, to have had a type II dissection, and to have lived longer. CONCLUSIONS: In our contemporary cohort, type B dissections are more common than type A dissections and occur at traditional nonsurgical thresholds. The associations of type B dissection with ARR, independent mitral valve surgery, and type II dissection suggest a more severe phenotype in the setting of prolonged life expectancy.

Journal / Series
Journal of the American College of Cardiology
Date Issued
2023-10-18
Publisher
Elsevier
Keywords
WCM Library Coordinated Deposit
•
Marfan syndrome
•
aortic dissection
•
elective aortic root replacement
•
type B dissection
Related DOI
https://doi.org/10.1016/j.jacc.2023.08.055
Previously Published as
Narula N, Devereux RB, Arbustini E, Ma X, Weinsaft JW, Girardi L, Malonga GP, Roman MJ. Risk of Type B Dissection in Marfan Syndrome: The Cornell Aortic Aneurysm Registry. Journal of the American College of Cardiology. 2023. doi: 10.1016/j.jacc.2023.08.055. PMID: 37930285.
Rights
Attribution-NonCommercial-NoDerivatives 4.0 International
Rights URI
https://creativecommons.org/licenses/by-nc-nd/4.0/
Type
article

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