Систолическая дисфункция ЛЖ у лиц молодого возраста с марфаноидной внешностью

Background. In Marfan syndrome early impairment of left ventricular systolic function has been reported. Our aim was to evaluate the left ventricular systolic function in young adults with Marfanoid habitus (MH) (includes arachnodactylia, dolichostenomelia, high palate, deformations of the thorax). Methods: We studied 137 young subjects (mean age 21.3±1.5) - 58 male, 79 female. Transthoracic echocardiography (Vivid 7 Dimension, GE) was performed in 24 asymptomatic MH with excluded Marfan syndrome and 42 healthy control subjects. Radial and circumferential systolic strain and strain rate were determined using spackle tracking (EchoPAC»08, GE). Results: Ascending aorta diameters were larger in subjects with MH. LV mass index did not differ significantly between groups, but interventricular septum and posterior wall thickness were greater in MH group. Local LV radial and circumferential systolic deformation indices were significant decreased in MH group. Conclusions: Young adults with MH in the absence of major findings of Marfan syndrome (ascending aortic aneurysm and ectopia lentis) have decreased LV systolic function.