The Risk for Type B Aortic Dissection in Marfan Syndrome

JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY

卷:65

Article

den Hartog, Alexander W.^1,2  Franken, Romy^1,2  Zwinderman, Aeilko H.^2,3  Timmermans, Janneke⁴  Scholte, Arthur J.⁵  van den Berg, Maarten P.⁶  de Waard, Vivian^6,7  Pals, Gerard⁸  Mulder, Barbara J. M.^1,2  Groenink, Maarten^1,2,9 

[1] Univ Amsterdam, Acad Med Ctr, Dept Cardiol, NL-1105 AZ Amsterdam, Netherlands

[2] Interuniv Cardiol Inst Netherlands, Utrecht, Netherlands

[3] Univ Amsterdam, Acad Med Ctr, Dept Clin Epidemiol & Biostat, NL-1105 AZ Amsterdam, Netherlands

[4] Radboud Univ Nijmegen, Med Ctr, Dept Cardiol, NL-6525 ED Nijmegen, Netherlands

[5] Leiden Univ, Med Ctr, Dept Cardiol, Leiden, Netherlands

[6] Univ Groningen, Univ Med Ctr Groningen, Dept Cardiol, Groningen, Netherlands

[7] Univ Amsterdam, Acad Med Ctr, Dept Med Biochem, NL-1105 AZ Amsterdam, Netherlands

[8] Vrije Univ Amsterdam Med Ctr, Dept Clin Genet, Amsterdam, Netherlands

[9] Univ Amsterdam, Acad Med Ctr, Dept Radiol, NL-1105 AZ Amsterdam, Netherlands

关键词: aortic diameter;    aortic dissection;    aortic distensibility;    aortic root replacement;    distal aorta;    losartan;    Marfan syndrome;   

DOI  :  10.1016/j.jacc.2014.10.050

来源: Elsevier

【 摘 要 】

BACKGROUND Aortic dissections involving the descending aorta are a major clinical problem in patients with Marfan syndrome. OBJECTIVES The purpose of this study was to identify clinical parameters associated with type B aortic dissection and to develop a risk model to predict type B aortic dissection in patients with Marfan syndrome. METHODS Patients with the diagnosis of Marfan syndrome and magnetic resonance imaging or computed tomographic imaging of the aorta were followed for a median of 6 years for the occurrence of type B dissection or the combined end point of type B aortic dissection, distal aortic surgery, and death. A model using various clinical parameters as well as genotyping was developed to predict the risk for type B dissection in patients with Marfan syndrome. RESULTS Between 1998 and 2013, 54 type B aortic dissections occurred in 600 patients with Marfan syndrome ( mean age 36 perpendicular to 14 years, 52% male). Independent variables associated with type B aortic dissection were prior prophylactic aortic surgery ( hazard ratio: 2.1; 95% confidence interval: 1.2 to 3.8; p = 0.010) and a proximal descending aorta diameter >= 27 mm ( hazard ratio: 2.2; 95% confidence interval: 1.1 to 4.3; p = 0.020). In the risk model, the 10-year occurrence of type B aortic dissection in low-, moderate-, and high-risk patients was 6%, 19%, and 34%, respectively. Angiotensin II receptor blocker therapy was associated with fewer type B aortic dissections ( hazard ratio: 0.3; 95% confidence interval: 0.1 to 0.9; p = 0.030). CONCLUSIONS Patients with Marfan syndrome with prior prophylactic aortic surgery are at substantial risk for type B aortic dissection, even when the descending aorta is only slightly dilated. Angiotensin II receptor blocker therapy may be protective in the prevention of type B aortic dissections. (C) 2015 by the American College of Cardiology Foundation.

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