Quantification of Myocardial Extracellular Volume Fraction with Cardiac MR Imaging for Early Detection of Left Ventricle Involvement in Systemic Sclerosis

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.

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Diffuse cardiac fibrosis quantification in early systemic sclerosis by magnetic resonance imaging and correlation with skin fibrosis

Journal of Scleroderma and Related Disorders ◽

10.1177/2397198318762888 ◽

2018 ◽

Vol 3 (2) ◽

pp. 159-169 ◽

Cited By ~ 8

Author(s):

Daniel C Lee ◽

Monique E Hinchcliff ◽

Roberto Sarnari ◽

Madeline M Stark ◽

Jungwha Lee ◽

...

Keyword(s):

Magnetic Resonance Imaging ◽

Cardiac Magnetic Resonance ◽

Systemic Sclerosis ◽

Magnetic Resonance ◽

T1 Mapping ◽

Volume Fraction ◽

Extracellular Volume ◽

Extracellular Volume Fraction ◽

Resonance Imaging ◽

Early Systemic Sclerosis

Purpose: To evaluate the utility of cardiac magnetic resonance T1 mapping in early systemic sclerosis and its association with skin score. Methods: In total, 24 consecutive patients with early systemic sclerosis referred for cardiovascular evaluation and 12 controls without systemic sclerosis were evaluated. All patients underwent cine, T1 mapping, and late gadolinium–enhanced cardiac magnetic resonance imaging. T1 mapping indices were compared between systemic sclerosis patients and controls (extracellular volume fraction, gadolinium partition coefficient (λ), pre-contrast T1, and post-contrast T1). The association between T1 mapping parameters and the modified Rodnan skin score was determined. Results: There were no significant differences in cardiac structure/function between systemic sclerosis patients and controls on cine imaging, and 8 of 24 (33%) systemic sclerosis patients had evidence of late gadolinium–enhanced cardiac magnetic resonance imaging (i.e. focal myocardial fibrosis). Of the T1 mapping parameters (indices indicative of diffuse myocardial fibrosis), extracellular volume fraction differentiated systemic sclerosis patients from controls the best, followed by λ, even when the eight systemic sclerosis patients with late gadolinium–enhanced cardiac magnetic resonance imaging were excluded. Extracellular volume fraction had a sensitivity and specificity of 75% and 75% for diffuse myocardial fibrosis (optimal abnormal cutoff value of >27% (area under receiver operating characteristic curve = 0.85)). In the 16 patients without evidence of late gadolinium–enhanced cardiac magnetic resonance imaging, each of the four cardiac magnetic resonance T1 mapping parameters (extracellular volume fraction, λ, Pre-T1 and Post-T1) correlated with modified Rodnan skin score ( R = 0.51–0.65, p = 0.007–0.043), indicating a correlation between systemic sclerosis cardiac and skin fibrosis. Conclusion: The four T1 mapping indices are significantly correlated with modified Rodnan skin score in patients with early systemic sclerosis. Quantification of diffuse myocardial fibrosis using extracellular volume fraction should be considered as a marker for cardiac involvement in systemic sclerosis clinical studies.

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