Vascular reactivity modulation by exposure to serum from patients with Marfan syndrome in healthy rat aortic rings

Introduction Marfan syndrome is a rare disease in which different metabolic pathways and immune mediators such as transforming growth factor β1, interleukins and metalloproteinases participate. Material and methods Thoracic aortic rings were obtained from 20 healthy male Wistar rats weighing 350–400 g to evaluate vascular reactivity in the presence of serum from healthy subjects or patients with Marfan syndrome with or without transforming growth factor β1, verapamil, sodium nitroprusside and nitro-L-arginine methyl ester. Interleukins were determined by Enzyme-Linked Immunosorbent Assay in the serum, and the nitrates and nitrites ratio was evaluated by spectrometry. Metalloproteinases and inducible nitric oxide synthase were evaluated in the thoracic aorta aneurysm from patients with Marfan syndrome and healthy subjects by immunohistochemistry. Results There was increased vasoconstriction, (p = 0.008) and decreased vasodilation ( P = 0.01) in the presence of transforming growth factor β1, in aortas incubated with serum from patients with Marfan syndrome. Aortic rings from healthy subjects or Marfan syndrome incubated in the presence or absence of the combination of verapamil, transforming growth factor β1, sodium nitroprusside and nitro-L-arginine methyl ester ( P = 0.001) showed a decrease in vasoconstriction and an increase in vasorelaxation. The concentration of interleukins-6 and c reactive protein and of the nitrates and nitrites ratio was increased in serum from Marfan syndrome (p ≤ 0.03). The Metalloproteinases and inducible nitric oxide synthase were increase in thoracic aortic from Marfan syndrome versus healthy subjects or, ( P = 0.03 and P = 0.04 respectively). Conclusion The increase in circulating active transforming growth factor β1 and the subsequent activation of its downstream signaling pathway could be involved in the pathogenesis of aortic aneurysms in human patients with Marfan syndrome contributing to the modulation of vascular reactivity in healthy aortas.