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

Nupoor Narula; Richard B Devereux; Eloisa Arbustini; Xiaoyue Ma; Jonathan W Weinsaft; Leonard Girardi; Grace P Malonga; Mary J Roman

doi:10.1016/j.jacc.2023.08.055

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

J Am Coll Cardiol. 2023 Oct 18:S0735-1097(23)07569-1. doi: 10.1016/j.jacc.2023.08.055. Online ahead of print.

Authors

Nupoor Narula¹, Richard B Devereux¹, Eloisa Arbustini², Xiaoyue Ma³, Jonathan W Weinsaft¹, Leonard Girardi⁴, Grace P Malonga¹, Mary J Roman⁵

Affiliations

¹ Division of Cardiology, Weill Cornell Medicine, New York, New York, USA.
² IRCCS Foundation Policlinico San Matteo, Pavia, Italy.
³ Division of Biostatistics and Epidemiology, Department of Health Care Policy and Research, Weill Cornell Medicine, New York, New York, USA.
⁴ Department of Cardiothoracic Surgery, Weill Cornell Medicine, New York, New York, USA.
⁵ Division of Cardiology, Weill Cornell Medicine, New York, New York, USA. Electronic address: mroman@med.cornell.edu.

PMID: 37930285
DOI: 10.1016/j.jacc.2023.08.055

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.

Keywords: Marfan syndrome; aortic dissection; elective aortic root replacement; type B dissection.