Background/Purpose: Cardiac involvement is a leading cause of death in systemic sclerosis (SSc). Cardiac MRI (CMR) is useful in the early assessment of cardiac disease. Our aim was to describe the characteristics of patients with inflammatory and/or fibrotic abnormalities on CMR and their clinical outcomes in a large retrospective SSc cohort.

Methods: We identified SSc patients with CMR done from 2010 to 2020 by searching the electronic database of a tertiary-care university center. CMR abnormalities of interest were edema (T2 hyperintensity) and fibrosis (late gadolinium enhancement, LGE). Outcomes included heart failure, atrial and ventricular arrhythmias, need for pacemaker or defibrillator and cardiac death (not attributable to other causes). Student’s, Mann-Whitney U and chi-square tests were used to compare patient characteristics.

Results: A total of 114 CMRs performed in 99 patients were identified (95% met ACR-EULAR criteria). Mean (SD) age was 57.8 (11.7) years and median (IQR) disease duration was 5.9 (2.6-12.8) years. Patients were mostly female (80%) and white (92%). Half of CMRs were performed as screening tests (asymptomatic), 25% were done to investigate cardiac symptoms and 25% were done following abnormal cardiac investigations.

After excluding two patients with coronary artery disease-related changes, 12 (12%) patients had inflammatory and/or fibrotic CMR abnormalities (LGE, n=11; T2 hyperintensity, n=5), of whom 4 were asymptomatic. LGE was most often described as heterogeneous, linear or focal/nodular areas of enhancement affecting the left ventricular mid-wall and/or subendocardial layers of the basal and/or mid-cavity segments, in non-ischemic distributions involving the antero-septal (n=5), inferior (n=5), infero-lateral (n=5), anterior (n=3), antero-lateral (n=3) and/or infero-septal (n=2) segments.

Baseline characteristics are presented in Table 1. Patients with abnormal CMR more often had cardiac symptoms/signs or abnormal investigations (80% vs 44%, p=0.02), lower left ventricular ejection fractions (p=0.007) and higher troponin levels (p=0.013). They were also numerically more often males (40% vs 18%) with hypertension (50% vs 27%), myositis (20% vs 11%), digital ulcers/scars (50% vs 34%), interstitial lung disease (80% vs 54%) and anti-topoisomerase I antibodies (30% vs 18%).

Over a mean (SD) follow-up of 4.3 (2.9) years, no patient developed new-onset heart failure. One patient with normal CMR developed syncope with frequent premature ventricular contractions (6% of QRS complexes) on Holter and inflammation/fibrosis on follow-up CMR, leading to implantable defibrillator placement 3 years after the initial CMR. Two patients with symptomatic myocarditis died from sudden death within one year of the initial abnormal CMR. None of the four asymptomatic patients with CMR abnormalities developed clinically apparent cardiac complications.

Conclusion: In this real-world SSc cohort, CMR abnormalities were found in 12% of patients, most of whom had clinically apparent cardiac disease. Abnormalities in asymptomatic patients were infrequent. Further study is required to determine the value of CMR screening in asymptomatic SSc patients.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/retrospective-study-on-the-prognostic-value-of-cardiac-magnetic-resonance-imaging-abnormalities-in-systemic-sclerosis/