The risk for type B aortic dissection in Marfan syndrome

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

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.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Aneurysm, Dissecting / classification
  • Aneurysm, Dissecting / etiology*
  • Aneurysm, Dissecting / prevention & control
  • Angiotensin Receptor Antagonists / therapeutic use
  • Aorta, Thoracic / pathology
  • Aorta, Thoracic / surgery
  • Aortic Aneurysm, Thoracic / classification
  • Aortic Aneurysm, Thoracic / etiology*
  • Aortic Aneurysm, Thoracic / prevention & control
  • Dilatation, Pathologic
  • Female
  • Humans
  • Imaging, Three-Dimensional
  • Magnetic Resonance Imaging, Cine
  • Male
  • Marfan Syndrome / complications*
  • Multivariate Analysis
  • Risk Assessment*
  • Tomography, X-Ray Computed

Substances

  • Angiotensin Receptor Antagonists