Abstract
Background: Acute cardiac events are a significant contributor to mortality in systemic sclerosis (SSc). However, echocardiographic evaluation may be deceptively normal during an acute presentation. We hypothesized that in diffuse SSc patients presenting with acute cardiac events and a normal echocardiogram, cardiovascular magnetic resonance (CMR) would have incremental diagnostic/prognostic value. Methods. 50 consecutive diffuse SSc patients with normal echocardiograms were evaluated using a 1.5T system. A total of 27 (63%) had experienced an acute cardiac event three to tendays before CMR evaluation (rhythm disturbances, angina pectoris, shortness of breath). Left/right ventricular (LV/RV) volumes and ejection fractions (EF), as well as LV mass, the T2-signal ratio, early/late gadolinium enhancement (EGE/LGE), native/post-contrast T1-mapping, T2-mapping and extracellular volume fraction (ECV) were compared between the event and no-event groups. Results: No differences were identified in LV/RV volumes/EF/mass. In logistic regression analyses, independent predictors of belonging to the event group were EGE (odds ratio (95% CI): 1.55 (1.06–2.26), p = 0.024), LGE (1.81 (1.23–2.67), p = 0.003), T2 mapping (1.20 (1.06–1.36), p = 0.004) and native/post-contrast T1 mapping (1.17 (1.04–1.32), p = 0.007 and 0.86 (0.75–0.98), p = 0.025). At a median follow-up of ~1.2 years, 42% vs. 11% of the event/no-event group respectively reached a combined endpoint of event occurrence/recurrence or cardiovascular mortality. Of the independent predictors resulting from logistic regression analyses, only LGE (hazard ratio (95% CI): 1.20 (1.11–1.30), p < 0.001), T2-mapping (1.07 (1.01–1.14), p = 0.025) and native T1-mapping (1.08 (1.01–1.15), p = 0.017) independently predicted the combined endpoint. Conclusions: A normal echocardiogram does not preclude myocardial lesions in diffuse SSc patients, which can be detected by CMR especially in symptomatic patients.
Highlights
Systemic sclerosis (SSc) is an autoimmune disease (ARD) of unknown etiology, characterized by fibrosis of the skin and internal organs, typically microvascular vasculopathy and systemic inflammation with presence of circulating autoantibodies and pro-inflammatory cytokines [1,2]
A dose of 0.1 mmol/kg gadobenate dimeglumine paramagnetic contrast agents was injected for early gadolinium enhancement (EGE) and another 0.1 mmol/kg for late gadolinium enhancement (LGE), according to the protocol suggested by the Lake
Patients in the event group had noticeably higher LGE values compared to the no-event group (median (IQR): 6.0 (5.0–12.0) vs. 3.0 (0.0–5.0), p
Summary
Systemic sclerosis (SSc) is an autoimmune disease (ARD) of unknown etiology, characterized by fibrosis of the skin and internal organs, typically microvascular vasculopathy and systemic inflammation with presence of circulating autoantibodies and pro-inflammatory cytokines [1,2]. Microvascular disease of the myocardium can result in angina pectoris, acute myocardial infarction (MI) or both in SSc patients. Inflammation presenting either as myocarditis [10] or as acute, diffuse subendocardial vasculopathy leading to diffuse subendocardial fibrosis [11] may contribute to increased cardiovascular mortality. Acute cardiac events are a significant contributor to mortality in systemic sclerosis (SSc). Echocardiographic evaluation may be deceptively normal during an acute presentation. We hypothesized that in diffuse SSc patients presenting with acute cardiac events and a normal echocardiogram, cardiovascular magnetic resonance (CMR) would have incremental diagnostic/prognostic value. 50 consecutive diffuse SSc patients with normal echocardiograms were evaluated using a 1.5T system. A total of 27 (63%) had experienced an acute cardiac event three to tendays before CMR evaluation (rhythm disturbances, angina pectoris, shortness of breath).
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