Progression of Left Ventricular Myocardial Dysfunction in Systemic Sclerosis: A Speckle-tracking Strain Echocardiography Study

Objective. Cardiac involvement is a main cause of mortality in systemic sclerosis (SSc). Its detection remains challenging using conventional echocardiography and little is known about its potential progression. This study assessed changes in cardiac performance over time in a prospective cohort of patients with SSc, including echocardiographic speckle-tracking strain analysis. Methods. The study included 234 patients with SSc [196 women, age 52 +/- 14 yrs, 165 limited SSc, time since diagnosis 5.2 yrs, interquartile range (IQR) 2.9-11.3]. Clinical variables, laboratory tests, pulmonary function tests, and echocardiographic measures were recorded at baseline and followup (median 2.3 yrs, IQR 1.3-3.9). Additionally, left ventricular (LV) systolic function was assessed with global longitudinal strain (GLS) by echocardiographic speckle-tracking analysis. Results. At followup, GLS had significantly worsened (-21% +/- 2 vs -19% +/- 2, p < 0.001) while LV ejection fraction had not changed (62% +/- 7 vs 61% +/- 8, p = 0.124). In particular, 39 patients showed a significant deterioration of GLS as defined by a >= 15% decrease, which was accompanied by a concomitant worsening of proximal muscle weakness, lung fibrosis, renal function, LV diastolic function, and right ventricular systolic function. Baseline variables associated with >= 15% deterioration in GLS were proximal muscle weakness (OR 3.437, 95% CI 1.13-10.43, p = 0.020), decreased DLCO (OR 3.621, 95% CI 1.25-10.51, p = 0.049), and LV diastolic dysfunction (OR 2.378, 95% CI 1.07-5.27, p = 0.033). Conclusion. In patients with SSc, progression of LV systolic dysfunction was demonstrated by GLS but not by LV ejection fraction. Proximal muscle weakness, DLCO, and LV diastolic dysfunction may identify patients at risk for progressive LV systolic dysfunction and in need of closer cardiac monitoring.