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Ventricular-Vascular Coupling in Marfan and Non-Marfan Aortopathies

Farina Loeper1, Jantine Oosterhof2, Mark van den Dorpel2

  • 1Charite Hospital, Berlin, Germany.

Journal of the American Heart Association
|November 18, 2016
PubMed
Abstract

Insights

Marfan syndrome (MFS) impairs left ventricular (LV) contractility and ventricular-vascular coupling (VVI), unlike non-syndromal thoracic aortic aneurysm and dissection (ns-TAAD). VVI abnormalities in MFS are linked to increased mortality risk.

Area of Science:

  • Cardiology
  • Genetics
  • Aortic Diseases

Background:

  • Marfan syndrome (MFS) and familial non-syndromal thoracic aortic aneurysm and dissection (ns-TAAD) are genetic aortopathies characterized by aortic dilatation and increased stiffness.
  • These conditions affect the aorta, a major blood vessel, and can lead to serious cardiovascular complications.

Purpose of the Study:

  • To compare left ventricular (LV) contractility and ventricular-vascular coupling index (VVI) between MFS and ns-TAAD patients.
  • To investigate the determinants of VVI in these genetic aortopathies.

Main Methods:

  • Echocardiography was used to study patients with MFS and ns-TAAD, comparing them to a control group.
  • Key parameters assessed included aortic geometry, hemodynamics, LV work, LV contractility (end-systolic elastance [Ees]), and VVI.

Main Results:

  • Aortic sinuses were equally dilated in MFS and ns-TAAD compared to controls. Aortic stiffness was elevated in both MFS and ns-TAAD.
  • LV contractility (Ees) was reduced in MFS but increased in ns-TAAD compared to controls. VVI was abnormal in MFS patients.
  • A VVI of 0.8 or higher in MFS patients was associated with an increased risk of death and heart failure. Beta-blocker treatment showed partial VVI normalization in MFS.

Conclusions:

  • Left ventricular contractility and VVI are impaired in MFS, but preserved in ns-TAAD.
  • These findings suggest an intrinsic myocardial contractility impairment in MFS, independent of aortic stiffness.
  • Abnormal VVI in MFS is a significant predictor of adverse cardiovascular outcomes.
Keywords:
aneurysmmatrix metalloproteinasesmechanicsmyocardiumβ‐blocker

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