Subclinical systolic dysfunction and higher stiffness in patients with scleroderma

Introduction: Scleroderma (SD) is an autoimmune disease, characterized by microvascular occlusive disease and various patterns of cutaneous and parenchymal fibrosis. Cardiac involvement includes pulmonary hypertension and myocardial fibrosis, but the mechanisms and onset of left ventricular (LV) systolic dysfunction in these patients are insuffi ciently studied. Aim: To assess 2D and 4D parameters of LV systolic function and to determine arterial stiffness in patients with scleroderma. Methods: 40 subjects (51 ± 9 years, 39 women) were studied: 20 patients with scleroderma (53% limited, 47% generalized form, mean time since diagnosis 5 ± 4 years), and 20 age and cardiovascular risk factors matched normals. LV systolic function was assessed by standard 2D echo (ejection fraction, 2DEF), and by speckle tracking to determine 2D longitudinal (2DLS), circumferential (2DCS), and radial (2DRS) strain. 4D auto LV quantification echo was used to assess LV geometry, 4D ejection fraction (4DEF), and systolic deformation (longitudinal, 4DLS; circumferential strain, 4DCS; radial, 4DRS; and area strain, 4DAS). Arterial function was assessed by “e-tracking” to measure the elastic modulus (Ep) and the β index. Results: 2DLS and all global and regional LV parameters by 4D echo were signifi cantly lower in the scleroderma group (2DLS -16.8 ± 2.4% in SD vs. -21.2 ± 1.6% control, p ≤ 0.01; 4DEF 51.9 ± 8.1% in SD vs. 63.5 ± 3.0% control, p ≤ 0.01; 4DLS -13.6 ± 2.2% in SD vs. -22.2 ± 2.8% control, p ≤ 0.01; 4DCS -11.9 ± 3.0% in SD vs.-19.3 ± 2.4% control, p ≤ 0.01; 4DRS 31.3 ± 8.8% in SD vs. 61.4 ± 6.2% control, p ≤ 0.01; 4DAS 22.8 ± 6.0% in SD vs.34.9 ± 2.5% control, p ≤ 0.01), whereas 2DEF, 2DCS, and 2DRS were similar. These changes were associated with increased arterial stiffness in patients with scleroderma (Ep 102.0 ± 38.4 in SD vs. 69.0 ± 27.0 control, p = 0.01;β index 8.5 ± 2.7 in SD vs. 5.2 ± 1.7 control, p = 0.01). Conclusions: Patients with scleroderma have LV subclinical systolic dysfunction and increased arterial stiffness, suggesting that cardiovascular screening in these patients should be extended beyond assessment of pulmonary hypertension and right ventricular function.

ISSN – online: 2734 – 6382
ISSN-L 1220-658X
ISSN – print: 1220-658X
The Romanian Journal of Cardiology is indexed by:
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CODE: 379
CME Credits: 10 (Romanian College of Physicians)