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Introduction Background: Cardiac involvement in Systemic Sclerosis (SSc) is widely recognized as heterogeneous and, when clinically evident, it is associated with a poor prognosis. Recently, five cardiac magnetic resonance (CMR) phenotypes in SSc have been identified (Knight DS et al. European Heart Journal 2023). These phenotypes do not align with the existing clinical subgroup classifications or autoantibody statuses, yet each has a distinct 5-year prognosis. Our objective is to test the long-term prognostic significance of this classification system in an external cohort and to compare ECG Holter monitor parameters, NT-proBNP, and troponin T levels across these groups.Material and Methods Methods: CMR assessments were conducted on consecutive SSc patients who presented symptoms of dyspnoea, palpitations, or chest pain. Based on the CMR findings, patients were categorized into five distinct groups: those with dilated right hearts with right ventricular failure (RVF); biventricular failure with dilatation and dysfunction (BVF); and those with normal function, categorized further based on cavity sizes as average cavity (NF-AC), small cavity (NF-SC), and large cavity (NF-LC). The study focused on comparing clinical characteristics, serum troponin and NT-proBNP levels, ECG Holter monitor parameters, and 10-year survival outcomes across these groups.Results Table 1 presents the clinical characteristics and assessed parameters of the 65 enrolled patients across the CMR subsets. The distributions for NF-AC, BVF, NF-SC, NF-LC, and RVF were 43.1%, 21.5%, 16.9%, 15.5%, and 3.1%, respectively. The duration of SSc was the only disease characteristic that showed statistically significant differences across the subsets. Troponin T and NT-proBNP values were similar across all subsets. The NF-LC and RVF groups exhibited Left Bundle Branch Block (LBB) more frequently and tended to present more ventricular ectopic beats (VEB) compared to other groups. There was a variation in 10-year survival rates across the groups, with patients in the RVF, NF-LC, and BVF categories showing poorer prognosis (figure 1).Conclusions This data confirms the prognostic value of the proposed CMR subsets in an another European SSc cohort, highlighting that subsets with poorer prognosis are associated with a higher arrhythmic burden.Conclusions Initial therapy was associated with improved survival and reduced PAH progression in SSc-PAH, with consistent effects across haemodynamic thresholds and risk strata. These findings support guideline-recommended early intervention, highlight the importance of high-quality observational data in rare diseases, and underscore the need for RCTs to clarify treatment effects in patients with milder haemodynamic impairment.Abstract OC.05 Table 1Clinical features, serum TnI and NT-proBNP, and ECG Holter parameters of the enrolled patientsAbstract OC.05 Figure 1Ten-year survival across the five CMR subsets