Cardiovascular Magnetic Resonance Identifies High-Risk Systemic Sclerosis Patients with Normal Echocardiograms and Provides Incremental Prognostic Value

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.

Elevated interleukin-6 levels are associated with impaired outcome in cardiac transthyretin amyloidosis

“…In the large BIOSTAT-CHF cohort ( n = 2329 patients) IL-6 levels were elevated in patients with heart failure and HFpEF, atrial fibrillation or elevated NTproBNP levels[ 5 ]. These clinical findings are often present in ATTR-cardiomyopathy patients as well.…”

“…In the last years the role of systemic inflammation for progression of cardiovascular disease in general has been established, especially in coronary heart disease. Elevated interleukin (IL)-6 levels are described in patients suffering from heart failure with preserved ejection fraction (HFpEF), atrial fibrillation and elevated N-terminal pro-brain natriuretic peptide (NTproBNP)[ 5 ]. Furthermore, on a molecular level increased cardiac IL-6 and IL-6 receptor messenger ribonucleic acid (mRNA) levels in myocardial tissue have been associated with worsening of heart failure[ 6 ].…”

Ventricular Tachycardia Has Mainly Non-Ischaemic Substrates in Patients with Autoimmune Rheumatic Diseases and a Preserved Ejection Fraction

“…Further studies in uniform populations with individual ARDs should be performed before final conclusions can be drawn. Again, our group has already published similar work in patients with SSc [ 20 ]. The evaluation of myocardial strain in non-contrast CMR scans was not a part of this investigation.…”

“…Evaluation of de novo VT occurrence after CMR is required to provide more robust evidence in support of the findings observed in this study. Our group has already published similar work in patients with SSc [ 20 ]. Potential differences between groups in some CMR indices might have not been identified due to insufficient statistical power, as in the case of LGE.…”

Imaging in Diagnosis of Systemic Sclerosis

“…Moreover, T1-mapping offers incremental diagnostic value in the identification of patients with high risk of cardiovascular incidents and in addition to independently predict adverse outcomes at follow-up. Thus, CMR may be considered in cases where echocardiographic results are equivocal but the clinical picture warrants a higher index of suspicion, in order to identify high-risk patients early on and to institute prompt changes in the therapeutic management of SSc-related cardiac involvement [ 92 ].…”