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Endothelial function in Marfan syndrome: selective impairment of flow-mediated vasodilation

D G Wilson1, M F Bellamy, M W Ramsey

  • 1Congenital Heart Disease Center, University Hospital of Wales Healthcare NHS Trust, Heath Park, Cardiff, Wales, UK.

Circulation
|February 23, 1999
PubMed
Abstract

Insights

Marfan syndrome impairs flow-mediated dilation, a function of fibrillin in endothelial cells, but preserves agonist-mediated dilation. This suggests fibrillin is key to endothelial mechanotransduction and nitric oxide release.

Area of Science:

  • Cardiovascular research
  • Vascular biology
  • Connective tissue disorders

Background:

  • Marfan syndrome involves fibrillin alterations, impacting vascular connective tissues.
  • Fibrillin microfibrils associate with arterial endothelial cells, suggesting a role in endothelial function.
  • Elevated endothelial cell products in Marfan subjects indicate endothelial dysfunction.

Purpose of the Study:

  • To directly assess flow- and agonist-mediated endothelium-dependent brachial artery reactivity in Marfan subjects.
  • To investigate the role of fibrillin in endothelial cell function and nitric oxide release.
  • To differentiate between flow-mediated and agonist-mediated endothelial responses in Marfan syndrome.

Main Methods:

  • Brachial artery diameter, blood flow, and blood pressure were measured using ultrasonic wall tracking, Doppler ultrasound, and photoplethysmography.
  • Vascular reactivity was assessed during hand hyperemia and after nitroglycerin administration.
  • Endothelium-dependent vasodilation was further evaluated using intra-arterial infusions of acetylcholine, bradykinin, and NG-monomethyl-L-arginine (L-NMMA).

Main Results:

  • Flow-mediated brachial artery responses were significantly impaired in Marfan subjects compared to controls (P<0.0001).
  • Nitroglycerin-induced vasodilation was similar in both groups, indicating preserved smooth muscle function.
  • Agonist-induced vasodilation to acetylcholine and bradykinin, and L-NMMA-induced vasoconstriction, were not significantly different between groups.

Conclusions:

  • Marfan subjects exhibit impaired flow-mediated endothelium-dependent vasodilation but preserved agonist-mediated vasodilation.
  • These findings suggest that basal nitric oxide release is likely preserved in Marfan syndrome.
  • The selective loss of flow-mediated dilation highlights a potential role for fibrillin in endothelial cell mechanotransduction.

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