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J Am Coll Cardiol. 2015 Jan 27;65(3):246-54. doi: 10.1016/j.jacc.2014.10.050.

The risk for type B aortic dissection in Marfan syndrome.

Author information

1
Department of Cardiology, Academic Medical Center, Amsterdam, the Netherlands; Interuniversity Cardiology Institute of the Netherlands, Utrecht, the Netherlands.
2
Interuniversity Cardiology Institute of the Netherlands, Utrecht, the Netherlands; Department of Clinical Epidemiology and Biostatistics, Academic Medical Center, Amsterdam, the Netherlands.
3
Department of Cardiology, Radboud University Medical Center, Nijmegen, the Netherlands.
4
Department of Cardiology, Leiden University Medical Center, Leiden, the Netherlands.
5
Department of Cardiology, University Medical Center, Groningen, the Netherlands.
6
Department of Cardiology, University Medical Center, Groningen, the Netherlands; Department of Medical Biochemistry, Academic Medical Center, Amsterdam, the Netherlands.
7
Department of Clinical Genetics, VU University Medical Center, Amsterdam, the Netherlands.
8
Department of Cardiology, Academic Medical Center, Amsterdam, the Netherlands; Interuniversity Cardiology Institute of the Netherlands, Utrecht, the Netherlands; Department of Radiology, Academic Medical Center, Amsterdam, the Netherlands. Electronic address: m.groenink@amc.uva.nl.

Abstract

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 ± 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.

KEYWORDS:

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

PMID:
25614422
DOI:
10.1016/j.jacc.2014.10.050
[Indexed for MEDLINE]
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