Cardiac involvement in systemic sclerosis: a cardiac magnetic resonance centred multimodality work-up
T Constantino, I Cardoso, P Bras, I Almeida, J Viegas, V Ferreira, R Ferreira, S Pinheiro, S Aguiar RosaAbstract
Introduction
Systemic sclerosis (SSc) is characterised by microvascular injury and progressive fibrosis. Cardiac involvement is often subclinical, yet it carries major prognostic significance. However, the underlying myocardial substrate and its relationship with autoantibody profile remain incompletely defined.
Purpose
To characterize SSc-related myocardial involvement using cardiac magnetic resonance (CMR), echocardiography (Echo), biomarkers and 24-h Holter monitoring, and to explore associations with autoantibodies.
Methods
We conducted a retrospective, single-centre cohort study of patients with confirmed SSc who underwent CMR between 2019 and 2024. Clinical phenotype and autoantibodies (anti–Scl-70, anti-centromere, anti–RNA polymerase III) were recorded. CMR assessed biventricular volumes, ejection fraction (EF), native T1/T2 mapping, late gadolinium enhancement (LGE) and pericardial effusion. Echo evaluated left ventricular (LV) and right ventricular (RV) function, global longitudinal strain (GLS) and diastolic indices. High-sensitivity troponin T (cTnT), NT-proBNP and 24-h Holter were reviewed when available. Myocardial edema was defined as T2 ≥55 ms and diffuse fibrosis as native T1 ≥1000 ms.
Results
Ten patients were included (49±17 years; 70% female), 60% diffuse and 40% limited cutaneous SSc. Autoantibody positivity: anti–Scl-70 in 6/10 (60%), anti-centromere in 1/10 (10%), and anti–RNA polymerase III in 1/10 (10%). NT-proBNP was elevated (≥125 pg/mL) in 7/10 (70%; median 834 pg/mL [IQR 50–1329]). cTnT exceeded the 99th-percentile limit (≥14 ng/L) in 7/10 (70%). LV and RV EF were preserved, with mildly increased indexed volumes. LGE occurred in 8/10 (80%), with a non-ischaemic pattern (Table 1 and Figure 1C)—intramural in 6/10 (60%) and subepicardial in 2/10 (20%)—most frequently involving the basal anteroseptal segment (4/8 LGE-positive, 50%). Native T1 (Figure1B) was elevated in 9/10 (90%; mean 1096±112 ms). T2 mapping showed myocardial edema in 7/10 (70%; mean 54.3±4.7 ms), with concomitant elevation of T1 and T2 in 7/10 (70%). Mild pericardial effusion was present in all patients (Figure 1A). Echo showed preserved systolic function (LVEF 63 [47–63]%, n=7; tricuspid annular plane systolic excursion (TAPSE) 21 [19.2–23.5] mm, n=6; RV S′ 12 [10.5–15] cm/s, n=6). LA volume index was 20 [18.5–32.8] mL/m² (n=6) with enlargement (>34 mL/m²) in 2/6 (33%). Holter showed no tachyarrhythmias and low ectopy.
Conclusions
CMR revealed predominantly subclinical myocardial involvement in SSc, with coexisting diffuse fibrosis and myocardial oedema, consistent with active myocardial inflammation. Despite preserved systolic function, parametric mapping abnormalities suggest early fibrotic–inflammatory remodelling and underscore the incremental value of mapping techniques for early phenotyping.For image description, please refer to the figure legend and surrounding text.For image description, please refer to the figure legend and surrounding text.