Cardiac Intravoxel Incoherent Motion Diffusion‐Weighted Magnetic Resonance Imaging With T1 Mapping to Assess Myocardial Perfusion and Fibrosis in Systemic Sclerosis: Association With Cardiac Events From a Prospective Cohort Study

Abstract

Objective Myocardial involvement may occur during systemic sclerosis (SSc) and can lead to impaired myocardial contraction and/or arrhythmia. Cardiac magnetic resonance imaging (MRI) is used for noninvasive characterization of the myocardium. The aim of this study was to evaluate the utility of cardiac MRI with intravoxel incoherent motion (IVIM) diffusion‐weighted imaging (DWI) and longitudinal relaxation time (T1) sequence mapping for assessment of myocardial microvascular and interstitium impairment in SSc. Methods In this single‐center prospective cohort study, 40 consecutive patients with SSc and 20 healthy controls were assessed by cardiac MRI with IVIM DWI and T1 mapping sequences on a 3T scanning system. Images were analyzed independently by 2 assessors, and Bland‐Altman plots were used to assess interreader concordance and reproducibility. Characteristics of the patients were compared according to quartiles of T1 and perfusion fraction (f‐coefficient) values, using exact Cochran‐Ermitage trend tests for qualitative variables and analysis of variance for quantitative variables. Kaplan‐Meier cardiac events–free survival curves were plotted and compared with a log‐rank test for trend. Results T1 values were higher in SSc patients than in healthy controls, and were higher in the diffuse cutaneous SSc (dcSSc) subset (P = 0.02). Higher T1 values were associated with the immunologic pattern seen in patients with the dcSSc form (P = 0.0001), a higher modified Rodnan skin thickness score (MRSS) (P = 0.003), and a higher frequency of interstitial lung disease (P = 0.03). Moreover, higher T1 values were correlated with higher MRSS scores (r = +0.32, P = 0.04) and reduced forced vital capacity (r = −0.34, P = 0.048), and tended to be correlated with reduced total lung capacity (r = −0.30, P = 0.07). Lower f‐coefficient values, as a measure of decreased tissue perfusion, were associated with less frequent use of vasodilators (P = 0.02 for angiotensin‐converting enzyme inhibitors and P = 0.06 for calcium‐channel blockers) and more frequent use of glucocorticoids (P = 0.02). The f‐coefficients were inversely correlated with the T1 values (r = −0.31, P = 0.02). Furthermore, higher T1 values were associated with higher incidence of cardiac events (log‐rank test for trend P = 0.03). Conclusion Increased T1 values, potentially suggesting microscopic fibrosis, were observed more frequently in patients with dcSSc, and higher T1 values were associated with interstitial lung disease and more frequent cardiac events during follow‐up. The results of this study show that cardiac MRI with T1 mapping sequences and IVIM DWI may be useful in assessing myocardial involvement in patients with SSc.

Sudden Cardiac Death in Systemic Sclerosis: Diagnostics to Assess Risk and Inform Management

“…It is increasingly appreciated that there is a significant burden of myocardial fibrosis in SSc that is not manifest by either diastolic or systolic cardiac dysfunction [15,48,49]. CMR measures of both focal and diffuse myocardial fibrosis have been shown to predict the development of heart failure and arrhythmia, and a large burden of fibrosis is associated with increased mortality [14,15,50]. The prevalence of myocardial fibrosis is much greater than that of systolic dysfunction and reduced LVEF in SSc, despite the observed link between fibrosis and impaired cardiac function [50].…”

“…The risk of death from SSc, and in particular heart disease in SSc, has remained largely unchanged for over 40 years [5]. This includes the risk of sudden cardiac death (SCD) which is reported to occur in up to 9% of individuals with SSc, [8][9][10][11][12][13][14][15] increasing to 67% in patients with clinically overt SHI [10].…”

“…the risk of sudden cardiac death (SCD) which is reported to occur in up to 9% of individuals with SSc, [8][9][10][11][12][13][14][15] increasing to 67% in patients with clinically overt SHI [10].…”

Cardiovascular outcomes in systemic sclerosis with abnormal cardiovascular MRI and serum cardiac biomarkers

“… 34 36 The Scleroderma Arrhythmia Clinical Utility Study included 150 patients with SSc and reported predictive value of increased T2 ratio, a marker of myocardial oedema and LGE% for the development of ventricular rhythm disturbances and sudden cardiac death in SSc. Another study in 40 consecutive patients with SSc that had T1 mapping and diffusion-weighted imaging CMR showed association of T1 native with the occurrence of CV events 37 while Bordonaro et al , looking retrospectively at a symptomatic SSc cohort reported LGE, T1 native and ECV as independent predictors of CV events. 38 These studies included patients with both primary and secondary CVD, capturing real-life cohorts.…”

Quantitative assessment of left ventricular myocardial involvement in patients with connective tissue disease: a 3.0T contrast-enhanced cardiovascular magnetic resonance study

“…Multiparametric imaging derived from CMR has been increasingly used to evaluate a variety of cardiac diseases including ischemic cardiomyopathy and non-ischemic cardiomyopathy resulting from diabetes mellitus, amyloidosis, and hypertension [12][13][14]. However, to the best of our knowledge, few studies have simultaneously evaluated myocardial strain, microcirculation perfusion and myocardial fibrosis in patients with CTD [15,16]. Therefore, the present study aimed to quantitatively assess the multiple parameters derived from CMR including LV global myocardial deformation, microvascular perfusion, and late gadolinium enhancement (LGE) in CTD patients and investigated the association between LV deformation and myocardial perfusion, as well as LGE.…”