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Heart structure and function in systemic sclerosis
Wojciech Plazak1, Ewa Zabinska-Plazak, Anna Wojas-Pelc
1Department of Cardiac and Vascular Diseases, Collegium Medicum, Jagiellonian University, Kopernika Str 19 31-501 Kraków, Poland.
Insights
Systemic sclerosis (SSc) patients exhibit significant left ventricle diastolic dysfunction, particularly in longitudinal myocardial fibers. Systolic function and cardiac structure remain preserved in these patients.
Area of Science:
- Cardiology
- Rheumatology
- Echocardiography
Background:
- Systemic sclerosis (SSc) is a multisystem autoimmune disease characterized by fibrosing connective tissue, vascular abnormalities, and autoimmune phenomena.
- Cardiac involvement is a major determinant of survival in SSc patients.
Purpose of the Study:
- To assess structural and pathophysiological cardiac changes in SSc patients using standard echocardiography.
- To evaluate regional left ventricle (LV) systolic and diastolic dysfunction in SSc patients with Tissue Doppler Imaging (TDI).
Main Methods:
- Standard echocardiography (M-Mode, 2D, Doppler) and TDI were performed on 19 SSc women and 16 healthy controls.
- Myocardial velocities in systole and early diastole were assessed in 14 LV segments using TDI.
Main Results:
- No significant differences in LV ejection fraction, dimensions, wall thickness, or valvular structure were observed between groups.
- SSc patients showed a significantly decreased mitral E/A ratio and increased pulmonary systolic pressure.
- TDI revealed significantly lower early diastolic myocardial velocities in SSc patients, indicating diastolic dysfunction, primarily in longitudinal fibers.
Conclusions:
- SSc patients exhibit significant left ventricle diastolic dysfunction, localized to longitudinal myocardial fibers.
- Myocardial fibrosing and ischemia in SSc appear concentrated in the subendocardial region.
- LV dimensions, wall thickness, and systolic function are preserved in SSc patients.
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.