A Marfanoid Habitus Dyagnostics’ Algorithm And Morfo-Functional Heart Singularities Relevent To This Dysplastic Phenotype

Official Russian guidelines for inherited connective tissue diseases distinguish a range of dysplastic phenotypes, with the marfanoid habitus being one of them. The prevalence of the marfanoid habitus and morfometrical heart singularities among the young practically healthy persons were not studied before. Materials and methods of the study: 560 practically healthy young persons aged from 18 to 25 years (average value 19.2 ± 1.4) were examined within the framework of the study. All the persons were subjected to phenotypical and anthropometrical examinations, with a group of 320 persons studied with the Echo-cardiographic procedure specifically aimed at small heart anomalies’ discovery. The study resulted in assessment of the marfanoid habitus among the young persons depending on sex and selected threshold of Dolichostenomelia and arachnodactylia coefficients. It shows that the use of the diagnostic algorithms offered by official Russian recommendations leads to the identification of this state of nearly half practically healthy young male persons. This could cause a hyper-diagnostics among this contingent group. The girls with marfanoid habitus demonstrate alternated morfometrical parameters - greater myocardia mass index, bigger thickness of myocardia, the trend to left cardiac ventricle hypertrophy. The persons with marfanoid habitus also tend to have some cardiac anomalies more frequently (mitral and tricuspidal valve prolapsed, left ventricular false tendons, foramen ovale).

inherited connective tissue diseases, bone signs of disemriogenius, dolichostenomelia, arachnodactylia, marfanoid habitus, cardiac anomalias