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Introduction Cardiac manifestations in Systemic Sclerosis are common, majority remain subclinical. Significant involvement seen in some cases present with arrhythmia, myocarditis, endocardial or pancardiac involvement leading to ventricular dysfunction with overt heart failure. Isolated right ventricular involvement is rarer.Material and Methods 33-yr Asian male with dcSSc with Scl-70 antibodies since 2017, presented in late 2022 with mRSS score 11/51 but did not have any cardio-respiratory symptoms.PFTs showed normal spirometry with reduced DLCO (63%pred) and KCO (70%pred). ECG showed right bundle branch block and ECHO revealed relatively normal LV, but RV was dilated with reduced function. Estimated-RVSP was 21mmHg. HRCT Scan confirmed mild ILD.He was commenced on mycophenolate mofetil, amlodipine and omeprazole. Mid-2023 he presented with shortness of breath on walking about 50 meters with palpitations along with flu-like illness, raised CRP of 31mg/l (negative for covid). Troponin-I were 112 and repeated 94. He had a presyncope with short-lived VT (figure 1a).Repeat ECHO revealed LV size normal but flattening with RV volume overload, EF 64% and RV is significantly dilated, reduced function but no pericardial effusion. CTPA excluded PE.Left & right-heart catheter studies ruled out CAD and PHT. His cMRI was as in figure 2a,b,c and table 1.Results cMRI findings are as tabulated (table1). Also, his PET CT scan correlated with cMRI findings.AICD was inserted. His diagnosis was severe dcSSc (Scl-70 positive) with significant predominant involvement of RV.Arrhythmogenic RV dysplasia (with de novo mutation) was considered as a differential as well as amyloidosis, ‘post-flu’ myopathy but after MDM review it was felt strongly due to his dcSSc.Conclusions Cardiac disease is seen in 15-35% of SSc. Most have subclinical disease. It has significant morbidity and mortality (2.8-fold increase*) and 4th overall leading cause of death^.Myocarditis can be silent yet be detectable as illustrated by Mavrogeni etal by cMRI utilizing the Lake Louise Criteria. Myocarditis tends to occur in early stage, however our patient presented with symptoms at a later stage.There does not exist any specific treatment guidelines regarding severe myocarditis which are usually treated with ISA such as MMF, cyclophosphamide, low dose steroids and IV Ig also been tried based on clinical experience, case reports and observational data. There is a single case report of combination of rituximab and MMF which showed benefit.Treatment of cardiac manifestations remains an unmet need. In appropriately screened cases cardiac transplantation may possibly need to be considered in the centre of excellence.Abstract P.143 Figure 1–2Abstract P.143 Table 1Cardiac MRI findings