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Aortic distensibility in Marfan syndrome: a potential predictor of aortic events?

Mitzi M van Andel1, Vivian de Waard2, Janneke Timmermans3

  • 1Cardiology, Amsterdam UMC - Location AMC, Amsterdam, The Netherlands.

Open Heart
|October 27, 2021
PubMed
Abstract

Insights

Aortic distensibility decreases over time in patients with Marfan syndrome (MFS), particularly in the descending aorta. While reduced distensibility correlates with aortic dilatation, it may not predict individual clinical events in this MFS cohort.

Area of Science:

  • Cardiovascular Medicine
  • Medical Imaging
  • Genetics

Background:

  • Marfan syndrome (MFS) patients are susceptible to aortic aneurysms due to elastic fiber fragmentation, leading to decreased aortic distensibility.
  • Previous research indicated that reduced aortic distensibility predicts progressive descending aorta dilatation.

Purpose of the Study:

  • To investigate longitudinal changes in aortic distensibility in patients with Marfan syndrome.
  • To assess the potential of aortic distensibility as a predictor of aortic events in MFS.

Main Methods:

  • Retrospective study of 35 Marfan syndrome patients with at least four cardiac MRI examinations (1996-2012).
  • Aortic distensibility was assessed at ascending, proximal descending, and distal descending aorta levels.
  • Longitudinal changes were analyzed using linear mixed-effects regression models.

Main Results:

  • Mean aortic distensibility was low at baseline and significantly decreased over time in the descending aorta (levels 2 and 3).
  • The rate of distensibility loss increased along the aorta (0.01, 0.03, and 0.06×10-3/mm Hg/year at levels 1, 2, and 3, respectively).
  • Reduced distensibility was associated with aortic dilatation rate, with men exhibiting lower baseline distensibility than women.

Conclusions:

  • Marfan syndrome patients exhibit reduced aortic distensibility early in life, inversely related to aortic dilatation rate.
  • In this selected MFS cohort, aortic distensibility appears less suitable as an individual predictor of clinical aortic events.
Keywords:
Marfan syndromeaneurysmdissectingmagnetic resonance imaging

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