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Marfan syndrome (MS) is of the most common connective tissue disorders. Although most patients have mutations in the fibrillin-1 gene (FBN1) and more than 1,700 mutations have been described, there are no mutations in less than 10% of patients. Aortic dilation is the most important complication; it involves chronic inflammatory processes and endothelial dysfunction. Prospective study from March 2015 to January 2017, in a cohort of 32 patients of MS confirmed by Ghent criteria and 35 controls of both genders, with a median age of 26 years (18–56). Patients had no comorbidities such as diabetes, hypertension, and/or neoplasms. They were not being treated with statin, NSAIDs, calcium antagonists, oral nitrates, and/or beta-blockers during 7 days prior to the study and patients with smoking history in the last 4 years. Controls were matched by age and gender. We analyzed endothelial dysfunction by flow-mediated vasodilation in the brachial artery, determining the maximum peak flow in the reactive hyperemia phase with a Philips Envisor device with Doppler capability. Its correlation with serum levels of biological markers that could participate in endothelial dysfunction pathways such asNO   3  −   /    NO   2  −ratio,NO   2  −, citrulline, TNFα, IL-1, IL-6, IL-10, IL-8, osteopontin, ICAM, VCAM, andNO   3  −   /    NO   2  −was determined. Endothelial dysfunction was found in 21 MS patients (65%). The aortic annulus (AAo) was of 27 mm (22–40) and 24 mm (22–30) ( p  = 0.04) in MS patients with and without dysfunction. The level ofNO   3  −   /    NO   2  −ratio, was of 108.95 ± 12.05 nM/ml in controls vs. 170.04 ± 18.76 nM/ml in MS ( p  = 0.002),NO   2  −was of 33.78 ± 3.41 vs. 43.95 ± 2.59 nM/ml ( p  = 0.03), citrulline 62.65 ± 3.46 vs. 72.81 ± 4.35 μMol/ml ( p  = 0.06). VCAM median was 39 pg/ml (0–86) vs. 32 pg/ml (11–66) ( p  = 0.03), respectively. The correlation of VCAM with triglycerides (TG) was of 0.62 ( p  = 0.005). There were no differences in TNFα, IL-1, IL-6, IL-8, IL-10, and osteopontin. MS endothelial dysfunction is related to aortic diameters, and increased levels of VCAM,  L -citrulline andNO   3  −   /    NO   2  −ratio,NO   2  −. VCAM-1 has a significant correlation with TG and could play a significant role in endothelial dysfunction.

frontiers in physiology

The Evaluation of Flow-Mediated Vasodilation in the Brachial Artery Correlates With Endothelial Dysfunction Evaluated by Nitric Oxide Synthase Metabolites in Marfan Syndrome Patients