Ab1212 Presentation, Treatment and Outcome of Systemic Sclerosis-Primary Heart Involvement: Data From a Monocentric Cohort

Background: Systemic sclerosis (SSc) carries a significant burden of morbidity and mortality. SSc-primary heart involvement (pHI) is characterized by inflammation and small-vessel vasculopathy leading to myocardial fibrosis, and accounts for a great share of disease-related deaths. Scant evidence is available on its presentation, follow-up and treatment strategies, and definite guidelines are lacking. Objectives: To describe a real-life cohort of SSc-pHI patients in terms of clinical, laboratory and imaging features at pHI onset, therapeutic interventions and outcome. Methods: Adults patients followed up in our center in the years 2018-2023, fulfilling the ACR/EULAR 2013 criteria for SSc and with evidence of pHI according to the latest definition1, were retrospectively enrolled. Data on clinical and serological presentation, EKG, echocardiographic and cardiac MRI (c-MRI) features and treatment were collected for all patients at pHI onset. As inclusion criteria, a minimum follow-up of 12 months from pHI onset was required. During follow-up, cardiac function was evaluated through serum biomarkers, EKG, echocardiographic and c-MRI imaging, whenever available. We also assessed the mortality rate and the prevalence of severe pHI-related complications: major arrhythmias, ICD implantation, heart failure (HF) and cardiac transplant. Results: Fourteen patients (64% males) were enrolled. The majority of patients had diffuse cutaneous SSc (71%) and anti-topoisomerase I antibodies (61%). The mean age at pHI onset was 53.1 ±13.6 years, with a median disease duration of 4.5 (1,25-11,3) years. At pHI diagnosis, 71% of patients had elevated troponin and 42% had BNP/NT-proBNP elevation. Only 36% of patients were symptomatic at pHI onset (1 patient had chest pain, 1 had palpitations, 2 had worsening dyspnea and 1 had syncope). Nearly all patients had abnormal EKG findings (72%) with the most frequent disorder being intraventricular conduction delay (63%). On Holter EKG monitoring, 3 patients showed >500 premature ventricular contractions over 24 hours, one of them with a run of non-sustained ventricular tachycardia. Five patients had abnormal findings on echo: 2 patients had right ventricular (RV) dysfunction without pulmonary hypertension, 3 had hypokinetic left ventricle (LV), 2 of them with reduced ejection fraction; 4 of these patients also had LV diastolic dysfunction. C-MRI was performed at baseline in 13 patients: all of them presented myocardial fibrosis (isolated LV in 9 patients, isolated RV in 1 and biventricular in 2) and 4 had myocardial edema. Pulse i.v. glucocorticoids were used in 4 patients; all patients were treated with mycophenolate mofetil, either alone or in combination with rituximab (5 patients), or tocilizumab (2 patients). At 12 months, troponin levels decreased significantly (>50%) compared to baseline in 6 patients, while follow-up echocardiographic and c-MRI findings, when available, showed no significant change. The mortality rate was 28%, with one death (7%) secondary to pHI; 2 patients received ICD implantation for primary prevention purposes; 1 patient had a major arrhythmia and 1 patient acute HF. Conclusion: Presentation of SSc-pHI varies broadly among affected patients and a multi-parametric assessment is required. Immunosuppressive treatment appears to prevent progression of pHI and stabilize heart function. REFERENCES: 1 Bruni C. et al, J Scleroderma Relat Disord. 2022. Acknowledgements: NIL. Disclosure of Interests: None declared.