Heart structure and function in systemic sclerosis

Introduction: Systemic sclerosis (SSc) is a multisystem disorder characterised by connective tissue fibrosing with vascular abnormalities and autoimmune changes. Heart involvement is one of the main factors shortening the survival of SSc patients. Aim of the study: (1) To assess structural and pathophysiological changes of the heart in SSc patients in standard echocardiographic examination. (2) To evaluate regional systolic and diastolic left ventricle disturbances in SSc patients by means of Tissue Doppler Imaging (TDI). Material and methods: In 19 SSc women aged 22-72 years and 16 control healthy women aged 19-57 years M-Mode, 2D, Continuous Doppler, Colour Doppler and Tissue Doppler examinations were performed. Tissue Doppler myocardial velocities in systole and early diastole were evaluated in 14 myocardial segments in parasternal and apical views. Results: No significant differences between both groups in left ventricle (LV) ejection fraction, LV end-diastolic and end-systolic diameter, LV wall thickness, valvular structure was observed. Mitral E/A ratio was significantly decreased in SSc women (p < 0.001), and pulmonary systolic pressure was significantly increased in this group (p < 0.001). In TDI examination no significant difference between the groups in systolic myocardial velocity was found. In SSc women early diastolic velocities were significantly lower than in controls (p < 0.00001). The detailed assessment of diastolic function in SSc group showed severe diastolic dysfunction of longitudinal myocardial fibers with normal function of circumflex myocardial fibers. Conclusions: In SSc patients significant left ventricle diastolic dysfunction was found. Diastolic dysfunction was observed only in longitudinal myocardial fibres and was not found in circumflex myocardial fibres. Thus, myocardial fibrosing and ischemia in SSc patients seem to be concentrated in the subendocardial region built by longitudinal muscle layer. Left ventricle diameter, walls thickness and systolic function in SSc patients are preserved.