Tolerance for physical activity and speckle tracking-characteristics of right ventricle deformation in young patients with connective tissue dysplasia

In order to clarify the state of physical performance of young patients with signs of poorly differentiated connective tissue dysplasia (CTD) in comparison with morphometric and functional performance of the heart, an open comparative study was conducted. Speckle tracking (ST) echocardiography was applied for quantitative evaluation of deformation properties of the right ventricle (RV) free wall. The study included 549 patients with CTD (330 men, mean age 23.51 ±8.67). An average diagnostic coefficient of CTD was 28.84±10.76. The main symptoms were CTD changes in the musculoskeletal system (n = 549), cardiovascular system (n=409), skin (n=169) and respiratory system (n=119). 44 patients with CTD and low exercise tolerance and 13 healthy volunteers were tested by using a speckle tracking technique. In the group of patients with CTD a significantly higher incidence of low exercise tolerance and dystonic reactions to physical exertion were observed. Also recorded were changes in the ST-parameters of RV systolic strain.