Abstract
Aims: T1-mapping is considered a surrogate marker of acute myocardial inflammation. However, in diffuse cutaneous systemic sclerosis (dcSSc) this might be confounded by coexisting myocardial fibrosis. We hypothesized that T1-based indices should not by themselves be considered as indicators of myocardial inflammation in dcSSc patients. Methods/Results: A cohort of 59 dcSSc and 34 infectious myocarditis patients was prospectively evaluated using a 1.5-Tesla system for an indication of suspected myocardial inflammation and was compared with 31 healthy controls. Collectively, 33 (97%) and 57 (98%) of myocarditis and dcSSc patients respectively had ≥1 pathologic T2-based index. However, 33 (97%) and 45 (76%) of myocarditis and dcSSc patients respectively had ≥1 pathologic T2-based index. T2-signal ratio was significantly higher in myocarditis patients compared with dcSSc patients (2.5 (0.6) vs. 2.1 (0.4), p < 0.001). Early gadolinium enhancement, late gadolinium enhancement and T2-mapping did not differ significantly between groups. However, both native T1-mapping and extracellular volume fraction were significantly lower in myocarditis compared with dcSSc patients (1051.0 (1027.0, 1099.0) vs. 1120.0 (1065.0, 1170.0), p < 0.001 and 28.0 (26.0, 30.0) vs. 31.5 (30.0, 33.0), p < 0.001, respectively). The original Lake Louise criteria (LLc) were positive in 34 (100%) myocarditis and 40 (69%) dcSSc patients, while the updated LLc were positive in 32 (94%) and 44 (76%) patients, respectively. Both criteria had good agreement with greater but nonsignificant discordance in dcSSc patients. Conclusions: ~25% of dcSSc patients with suspected myocardial inflammation had no CMR evidence of acute inflammatory processes. T1-based indices should not be used by themselves as surrogates of acute myocardial inflammation in dcSSc patients.
Highlights
Systemic sclerosis (SSc) is an autoimmune disease characterized by microvascular abnormalities, inflammation and fibrosis of all organs including the heart [1]
We aimed to evaluate a cohort of SSc patients with suspicion of myocardial inflammation using Cardiovascular magnetic resonance (CMR) in order to examine the relationship between T1- and T2-based indices and to characterize the constellation of CMR findings in this group
We aimed to evaluate patients referred for CMR due to suspected infectious myocarditis and healthy controls for comparison
Summary
Systemic sclerosis (SSc) is an autoimmune disease characterized by microvascular abnormalities, inflammation and fibrosis of all organs including the heart [1]. Primary cardiac disease in SSc patients is either caused by coronary microvasculopathy or by primary myocardial inflammation, with the end effect being myocardial fibrosis [4]. The early detection of myocardial inflammation in particular is of great clinical significance in SSc, because, in contrast to microvasculopathy, it is an acute process and should be managed with short-term immunosuppressive treatment [4]. CMR can detect myocardial oedema and replacement fibrosis using T2-weighted short tau imaging (STIR-T2) and late gadolinium enhancement (LGE) imaging respectively [9,10]. Native T1- and T2-mapping are highly sensitive to myocardial water content and more accurate than STIR-T2 in the detection of myocardial oedema [11,12]. T2-mapping can make up for the propensity of STIR-T2 for identifying artefacts as nonexistent oedema [13]
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