Abstract 15187: Risk Factors for Type B Dissection in Marfan Syndrome: The Cornell Aortic Aneurysm Registry

Abstract

Introduction/Background: With improvements in the medical and surgical management of patients with Marfan syndrome (MFS), type A dissections are decreasing. As a corollary, most MFS dissections are now type B, for which risk factors remain largely uncertain. Research Questions/Hypothesis: In this prospective and retrospective observational study, we explore determinants of type B dissection risk in a large single-center MFS registry through analysis of systematic clinical and imaging data over four decades. Goals/Aim: Determinants of type B dissection risk in MFS will inform appropriate prognostication, management, and treatment of this patient population. Methods/Approach: Demographic and anthropometric features, cardiovascular disease, and surgical history were compared in MFS patients with and without type B dissection. Results/Data: Of 335 MFS patients, 56 (17%) experienced a type B dissection (versus type A in 9%). Patients with type B dissection were more likely to have undergone elective aortic root replacement (ARR; 75 vs. 46%, p<0.001). 55% of patients had type B dissection a mean of 14.1 years after ARR, whereas 45% experienced type B dissection before ARR. Valve replacement was more common than valve-sparing repair in those with post-operative type B dissection (27% vs. 12%, p=0.02), although this difference was eliminated after adjusting for duration of post-operative follow up. 50 (89%) patients were aware of their MFS diagnosis prior to type B dissection. Among those with pre-dissection imaging, the descending aorta was normal or minimally dilated in 87%. In multivariable analyses, patients with type B dissection were more likely to have undergone ARR, mitral valve surgery, had a type II dissection, and to have lived longer. Conclusions: In our contemporary cohort, type B dissections are more common than type A. The associations of type B dissection with ARR, mitral surgery, and type II dissection suggest a more severe phenotype in the setting of prolonged life expectancy.