Abstract
Simple SummarySystemic Sclerosis (SSc) is a chronic connective tissue disorder with an inflammatory and autoimmune nature. The disease presents with microvascular changes, endothelial cell dysfunction, and fibrosis in visceral organs and tissues including lung, skin and heart. Cardiac involvement is a predictor of poor prognosis in this disease; therefore, early and pre-clinical diagnosis of cardiac involvement can be helpful in management of SSc. Two-dimensional Speckle Tracking Echocardiography (2D-STE) is a new method for the evaluation of myocardial strain in longitudinal, circumferential and radial planes. This study aimed at evaluating the mechanical performance of all cardiac chambers by using 2D-STE in patients with scleroderma, and its comparison with normal individuals. This study demonstrated that using 2D-STE can result in the diagnosis of impaired chamber mechanics and function in subclinical stages. Based on our findings, the simultaneous evaluation of all cardiac chambers by 2D-STE provides valuable information regarding myocardial involvement in patients with SSc.Objective: Cardiac involvement is a predictor of poor prognosis in patients with systemic sclerosis (SSc); therefore, preclinical diagnosis of heart involvement is crucial. Two-dimensional speckle tracking echocardiography (2D-STE), a method for evaluating the myocardial strain, could be helpful for the early diagnosis of cardiac mechanical function abnormalities. In this study, the simultaneous evaluation of all cardiac chambers was studied in patients with SSc, compared with normal individuals. Methods: The results of transthoracic echocardiography (TTE) and 2D-STE of 37 patients with SSc and 37 healthy individuals and the longitudinal strain (LS) of all chambers was precisely evaluated. The collected data were analyzed using SPSS version 16, and independent-sample t test and Chi-square test were used for comparison between the groups. Results: The mean ± SD of the participants’ age was 45.7 ± 11.54 (range of 17 to 68) years; most of them were women (75.7%). TTE showed higher left atrial (LA) volume (p < 0.001), right atrial (RA) area (p = 0.007), the severity of ventricular dysfunction (p < 0.05) and inferior vena cava diameter (p = 0.005), compared with the control group. Spectral and tissue Doppler echocardiography showed higher systolic pulmonary arterial pressure (sPAP) and mitral A wave velocity, and lower E/A ratio, E’ velocity of left ventricular (LV) septal and lateral wall in the case group (p < 0.05). Color Doppler echocardiography showed a higher frequency of valvular regurgitation in the case group (p < 0.05). The results of 2D-STE showed lower LA roof LS (p < 0.001), LA average LS (p = 0.015), LA global LS (p = 0.028), and LA ejection fraction (LAEF) (p = 0.001), lower mean RA left wall (p = 0.048) and EF (p < 0.001), and higher RV global LS in the case group (p = 0.025). Conclusions: Simultaneous evaluation of all cardiac chambers by 2D-STE provides valuable information about the myocardial involvement in patients with SSc. Therefore, it is suggested to use this method for the early diagnosis of cardiac involvement in such patients.
Highlights
Systemic Sclerosis (SSc) is a complex chronic connective tissue disorder, presenting with microvascular changes, endothelial cell dysfunction, and fibrosis in visceral organs and tissues, as well as complex autoimmune responses [1]
Patients who were diagnosed with Scleroderma by a rheumatologist and referred to us by Hafez Hospital, Shiraz, Iran, from November 2019 to May 2020 were considered as the case group, and the control group was conveniently selected from healthy volunteers, who were referred to the Shahid Faghihi Hospital echocardiography laboratory
The first important finding of the present study was the detection of cardiac changes in patients with SSc by 2D-STE, which were undetectable by transthoracic echocardiography (TTE); the second one is related to the simultaneous evaluation of the four cardiac chambers, considering the principal role of atria in cardiac forward stroke volume [19]
Summary
Systemic Sclerosis (SSc) is a complex chronic connective tissue disorder, presenting with microvascular changes, endothelial cell dysfunction, and fibrosis in visceral organs and tissues, as well as complex autoimmune responses [1]. The prevalence rates reported for the two major subtypes, SSc with limited or diffuse cutaneous involvement, vary according to geographical and racial risk factors, as well as the diagnostic criteria used in each study, while female predominance is a common statement [2]. Patients with SSc have a 2.7– to 3.4–fold increased mortality rate compared to the general population [3]. With poorer prognosis in patients with diffuse cutaneous SSc (dcSSc), pulmonary, renal, and cardiac involvements [4]. The cardiac involvement of SSc that results in a five–fold increased mortality rate [4] and sudden cardiac death in 21%–54% of patients [5], can be caused primarily by the inflammatory induced changes in cardiac structures, such as microvasculature, pericardium, conduction system, and valves, resulting in arrhythmia, pericarditis, valvular diseases, cardiac failure and cardiomyopathy, and/or secondarily by interstitial lung disease, pulmonary arterial hypertension (PAH) and kidney involvement [6]. Because of the low costs, availability and ease of performance of echocardiography, it is recommended as the primary step for detection and recommended is at least annual follow-up of myocardial, pericardial, and valvular involvements in the patients with SSc [8]
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