Background/Purpose: Cardiac involvement is a main cause of mortality in systemic sclerosis, although reported prevalence of cardiac involvement is low and detection of cardiac involvement remains challenging. Speckle-tracking strain analysis of echocardiographic images can detect subtle myocardial dysfunction in systemic sclerosis patients. Objectives: This study evaluated: 1. changes in cardiac performance over time including echocardiographic myocardial speckle tracking strain analysis in a large cohort of systemic sclerosis patients, not selected for disease severity,  and 2. baseline characteristics associated with deterioration of cardiac function.

Methods: 235 systemic sclerosis patients (197 female, 52±14 years), all fulfilling ACR 2013 or Leroy 2001 criteria for systemic sclerosis, were evaluated at baseline and follow-up (2.3 years, interquartile range 1.3-3.8), including complete physical examination and screening for organ involvement with at least high resolution computed tomography of the thorax, pulmonary function test, cardiopulmonary exercise test, electrocardiography and echocardiography.

Results: Left ventricular ejection fraction did not change significantly (62%±7 vs 61%±8,p=0.148) while global longitudinal strain decreased significantly (-21%±2 vs -19%±2, p<0.001). 39 patients showed progression of left ventricular dysfunction as defined by ≥15% decline in longitudinal strain. These patients showed significant worsening of left ventricular diastolic function and Tricuspid Annular Plane Systolic Excursion (TAPSE ; 22mm±4 versus 19mm±4, p=0.003) and 6% developed pulmonary hypertension. Multivariate analysis showed that at baseline proximal muscle weakness (Odds Ratio: 3.7; interquartile range:1.3–10.4), diffusing capacity of carbon monoxide (Odds Ratio: 0.97 [0.95–1.00]) and Left ventricular diastolic dysfunction (Odds Ratio: 2.25 [1.02–4.95]) were associated with progression of left ventricular dysfunction as reflected by strain analysis.

Conclusion: In this large cohort of systemic sclerosis patients, decline of left ventricular function was detected by speckle tracking strain analysis, and increase of percentage of patients with diastolic dysfunction. At baseline, proximal muscle weakness, diffusing capacity of carbon monoxide, and left ventricular diastolic function may identify patients at higher risk for deterioration of cardiac function and in need of closer cardiac monitoring by means of annual repeated echocardiography.    Table 1. Echocardiographic parameters at baseline and follow-up; n= 235 Abbreviations: LEVF= Left Ventricular Ejection Fraction; GLS= Global Longitudinal Strain; LV= Left Ventricular; sPAP= systolic Pulmonary Arterial Pressure; PAH= Pulmonary Arterial Hypertension; TAPSE = Tricuspid Annular Plane Systolic Excursion; PE= pericardial effusion

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/progression-of-left-ventricular-myocardial-dysfunction-in-systemic-sclerosis-using-speckle-tracking-strain-echocardiography-to-identify-patients-at-risk/