CMR feature tracking in cardiac asymptomatic systemic sclerosis: Clinical implications

Abstract

Background Impaired myocardial deformation has been sporadically described in cardiac asymptomatic systemic sclerosis (SSc). We aimed to study myocardial deformation indices in cardiac asymptomatic SSc patients using cardiac magnetic resonance feature tracking (CMR-FT) and correlate these findings to the phenotypic and autoimmune background. Methods Fifty-four cardiac asymptomatic SSc patients (44 females, 56±13 years), with normal routine cardiac assessment and CMR evaluation, including cine and late gadolinium enhancement (LGE) images, were included. SSc patients were compared to 21 sex- and age- matched healthy controls (17 females; 54±19 years). For CMR-FT analysis, a mid-ventricular slice for LV peak systolic radial and circumferential strain and a 4-chamber view for LV/RV peak systolic longitudinal strain were used. Results Twenty-four patients had diffuse cutaneous SSc and 30 limited cutaneous SSc. Thirteen patients had digital ulcers. Median disease duration was 3.6 years. LV ejection fraction was higher in SSc patients compared to controls (62±6% vs. 59±5%, p = 0.01). Four patients had no LGE examination; in the remaining patients LGE was absent in 74%, while 18% had RV insertion fibrosis and 8% evidence of subendocardial infarction. LV longitudinal strain differed in those with insertion fibrosis (-18.0%) and infarction (-16.7%) compared to no fibrosis (-20.3%, p = 0.04). Patients with SSc had lower RV longitudinal strain and strain rate compared to controls (p<0.001 and p = 0.01, respectively). All other strain and strain rate measurements were non-significant between patients and controls. Conclusions In cardiac asymptomatic SSc patients with normal routine functional indices, CMR-FT identifies subclinical presence of insertion fibrosis and/or myocardial infarction by impaired LV longitudinal strain. RV derived longitudinal indices were impaired in the patient group. CMR FT indices did not correlate to the patients’ phenotypic and autoimmune features.

Cardiac Magnetic Resonance Imaging in Pulmonary Arterial Hypertension: Ready for Clinical Practice and Guidelines?

“…Eisenmenger with right-to-left shunt and PAH), the fibrosis in the right ventricle and septum does not fully resemble that of non-congenital PAH, as in idiopathic PAH or PAH associated with connective tissue disorders, such as scleroderma [38,42]. Furthermore, patients with sclerodermas have been shown to have intrinsic myocardial involvement besides PAH-related alterations [29, [43][44][45][46][47][48]. In addition, localized LV fibrosis and infarctions have been shown even in cardiac asymptomatic patients with scleroderma suggesting a more complex fibrosis pattern in this population of PAH [43,44].…”

“…Furthermore, as many of the studies comprise diverse groups of patients, pooling the values into a uniform conclusive value could be considered infeasible. As an example, patients with scleroderma, including those cardiac asymptomatic, have been shown to have increased T1 values [44]. However, the distribution can stand in contrast to patients with congenital heart disease with fibrosis in the right ventricle and septum that does not resemble that of non-congenital PAH [42].…”

“…Furthermore, patients with sclerodermas have been shown to have intrinsic myocardial involvement besides PAH-related alterations [ 29 , 43 – 48 ]. In addition, localized LV fibrosis and infarctions have been shown even in cardiac asymptomatic patients with scleroderma suggesting a more complex fibrosis pattern in this population of PAH [ 43 , 44 ].…”

Underfilling decreases left ventricular function in pulmonary arterial hypertension

“…Patients with SSc have been shown to be prone to having myocardial fibrosis and could therefore be expected to have impaired myocardial stiffness, increased LV filling pressures and hence increased LA volumes. However, in a previous CMR study, patients with SSc did not have lower LV-GLS unless infarction or localised fibrosis was present [ 36 ]. Furthermore, low LV-GLS was shown to be due to PAH rather than SSc in itself [ 12 ].…”

Quantification of Myocardial Deformation Applying CMR-Feature-Tracking—All About the Left Ventricle?

“…In other relatively rare diseases like Marfan syndrome, Fabry disease or systemic sclerosis that can potentially affect myocardial structures, the use of CMR-FT including biventricular strain assessments and myocardial dyssynchrony was also demonstrated to sensitively detect myocardial dysfunction [87][88][89]. For example, subtle attenuations of LV and RV longitudinal function were found in patients with Marfan syndrome, while RV GCS values were increased reflecting a potential compensation mechanism and indicating the existence of a Marfan-related cardiomyopathy that is detectable by applying CMR-FT deformation analyses [90].…”