Cardiac involvement in systemic sclerosis - the added value of cardiac magnetic resonance

Background Cardiac complications of systemic sclerosis (SSc) are frequently subclinical or insidious and may affect up to 80% of patients [1], remaining one of the leading causes of death in, usually in the mechanism of life-threating arrythmia, heart failure or sudden cardiac arrest [2]. Diagnostic tools that can be used for heart involvement in SSc include ECG, echocardiography, serum markers (troponin, CK-MB, BNP, NT-proBNP) and, recently, magnetic resonance imaging. Cardiac magnetic resonance (CMR) with advanced tissue characterization techniques (including parametric mapping of T1 and T2 relaxation times as well as extracellular volume [ECV] fraction of the myocardium) can provide unique non-invasive insight into early (inflammation, oedema) and late (fibrosis, necrosis) stages of heart involvement in those patients [3]. Objectives To assess cardiac involvement in unselected systemic sclerosis patients in both diffuse (DSSc) and localized (LSSc) clinical subgroups, using contemporary cardiac magnetic resonance imaging techniques. Methods In this prospective single-centre study, we included 20 consecutive systemic sclerosis patients (51+/- 14 years, 14 [70%] female) in whom CMR was performed between 2013 and 2022. Their baseline CMR results, clinical presentation of SSc, medical history, laboratory tests, lung function tests, and follow-up were analyzed with special attention to CMR features of heart involvement in the course of SSc in both clinical subgroups. Moreover, CMR findings were compared to a group of healthy controls (N= 37). Results Systemic sclerosis patients had significantly lower LVEF (56,6% vs 61,6%, p<0,002), higher LVESVI (38,9 ml/m2 vs 31,6 ml/m2, p=0,002), longer T1 and T2 relaxation times (1029,3 ms vs 993 ms and 48,1 ms vs 44 ms, respectively; p<0,001), and higher ECV (27,9% vs 26,0%, p<0,05) as compared to healthy controls. Overall, 13 (65%) patients had at least one abnormal finding in CMR, of which in 7 (35%) baseline ECG and echocardiogram were normal or borderline normal. Additionally, heart involvement was more frequent among DSSc patients (11/13), with more severe manifestations including greater extent of dysfunction and more pronounced tissue alterations than in LSSc patients (2/7). Of note, CMR allowed for detection of subtle tissue abnormalities in 3 (15%) of patients with normal ECG and normal echocardiogram, as well as added new tissue information (edema/abnormal T1/T2/ECV and/or non-ischemic fibrosis) in 4 (20%) others, in whom borderline unspecific echo abnormalities were detected. During a median follow up of 3,4 (1,9-5,5) years, three patients (15%) died (heart failure - 1, gastrointestinal complications - 1 and breast cancer - 1). Treatment escalation and deescalation were required in 8 and 5 patients, respectively. Two patients had their treatment changed over the follow up period, in 5 the treatment remained unchanged. Conclusion SSc patients frequently present heart involvement, especially in DSSc type. This complication is often late diagnosed or misdiagnosed by conventional methods. Cardiac magnetic resonance with modern tissue characterization techniques, provides detailed insight in heart involvement in systemic sclerosis. Modern quantitative techniques allow for early diagnosis, thus prompting closer follow up and/or treatment decisions in a proportion of patients in whom other diagnostic tests were inconclusive. References [1]D’Angelo WA et al. Am J Med . 1969;46(3):428-440. doi:10.1016/0002-9343(69)90044-8 [2]Bruni C et al. Best Pract Res Clin Rheumatol . 2021;35(3):101668. doi:10.1016/j.berh.2021.101668 [3]De Luca G et al. Clin Rev Allergy Immunol . 2022;10.1007/s12016-022-08923-3. doi:10.1007/s12016-022-08923-3 Acknowledgements: NIL. Disclosure of Interests None Declared.