Cardiovascular Imaging for Systemic Sclerosis Monitoring and Management

Abstract

Systemic sclerosis (SSc) is a complex connective tissue disease with multiple clinical and subclinical cardiac manifestations. SSc can affect most structural components of the heart, including the pericardium, myocardium, valves, and conduction system through a damaging cycle of inflammation, ischemia, and fibrosis. While cardiac involvement is the second leading SSc-related cause of death, it is frequently clinically silent in early disease and often missed with routine screening. To facilitate identification of cardiac disease in this susceptible population, we present here a review of cardiac imaging modalities and potential uses in the SSc patient population. We describe well-characterized techniques including electrocardiography and 2D echocardiography with Doppler, but also discuss more advanced imaging approaches, such as speckle-tracking echocardiography, cardiovascular magnetic resonance imaging (CMR), and stress imaging, among others. We also suggest an algorithm for the appropriate application of these modalities in the workup and management of patients with SSc. Finally, we discuss future opportunities for cardiac imaging in SSc research to achieve early detection and to optimize treatment.

Diagnosis and management of primary heart involvement in systemic sclerosis

“…Transthoracic echocardiography (TTE) represents the cornerstone imaging modality for the evaluation of biventricular/biatrial function and wall motion abnormalities. It is widely available, bedside, cost effective, noninvasive and without radiation, and for these reasons is the first imaging approach for every cardiac disease [29] (Table 2).…”

“…To overcome these limitations, new speckle tracking indices were introduced, and speckle tracking echocardiography (STE) has recently emerged as a quantitative technique to accurately estimate myocardial function [29]. These indices include global longitudinal strain (GLS), global circumferential strain (GCS) and global radial strain (GRS), and mirror myocardial deformation in different motion directions: STE can be used to evaluate both global and regional LV and RV systolic function.…”

“…To date, TTE has also some limitations including operator and acoustic window dependency, which may do not allow accurate measurements [29]. Furthermore, it cannot perform tissue characterization, which may influence the risk stratification and treatment decision making in these patients [29].…”