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For many hereditary connective tissue disorders (HCTD), especially Marfan syndrome, remodeling of the heart and main vessels is described, which is manifested by a decrease in the systolic function of the left ventricle and expansion of the thoracic aorta. Evaluation of morphometric characteristics of the heart and main vessels in patients with other HCTD, in particular – marfanoid habitus (MH) has not been previously carried out.  Materials and methods . We examined 90 young men and 74 young women between the ages of 18 to 25 years, 111 patients older age groups with stable over coronary heart disease (mean age 64.6 ± 6.2 years) and 9 patients with verified Marfan syndrome (mean age 27.9 ± 9.3 years). All survey phenotypic and performed anthropometric survey identifying bone signs of dysembryogenesis as well as Echocardiography study on standard protocol.  The results.  Patients with MH as compared with control group revealed a relatively larger diameter of aortic root (30.4 ± 4.7 vs 28.0 ± 3.6 mm,  p  = 0.03) and the ascending aorta (26.6 ± 4.9 vs 24.6 ± 3.2 mm,  p  = 0.05). Also young with MH turned out to be significantly thicker myocardium of left ventricular posterior wall (8.3 ± 0.8 vs 7.7 ± 1.1 mm,  p  = 0.02) and interventricular septum (8.8 ± 1.2 vs 8.2 ± 1.1 mm,  p  = 0.04). When performing correlation analysis identified reliable positive correlation between such highly specialized bone signs as high palate ( r  = 0.31), infundibular deformation of the chest ( r  = 0.43), arachnodactyly ( r  = 0.45) and  Z -test ( p 2.0) have found 24% of older patients with MH.  Conclusion.  In patients with MH revealed significant structural changes of heart and main vessels which are progredient character – thickening of the left ventricular myocardium and expansion of the aortic root.

Remodeling of the heart and main vessels in patients with marfanoid habitus