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Risk of Type B Dissection in Marfan Syndrome

2023; Elsevier BV; Volume: 82; Issue: 21 Linguagem: Inglês

10.1016/j.jacc.2023.08.055

1558-3597

Nupoor Narula, Richard B. Devereux, Eloisa Arbustini, Xiaoyue Ma, Jonathan W. Weinsaft, Leonard N. Girardi, Grace Malonga, Mary J. Roman,

Cardiac Valve Diseases and Treatments

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.We explored the determinants of type B dissection risk in a large, single-center MFS registry.Demographic and anthropometric features, cardiovascular disease, and surgical history were compared in patients with MFS with and without type B dissection.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.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.