Consensus on the assessment of systemic sclerosis–associated primary heart involvement: World Scleroderma Foundation/Heart Failure Association guidance on screening, diagnosis, and follow-up assessment

Abstract

Introduction: Heart involvement is a common problem in systemic sclerosis. Recently, a definition of systemic sclerosis primary heart involvement had been proposed. Our aim was to establish consensus guidance on the screening, diagnosis and follow-up of systemic sclerosis primary heart involvement patients. Methods: A systematic literature review was performed to investigate the tests used to evaluate heart involvement in systemic sclerosis. The extracted data were categorized into relevant domains (conventional radiology, electrocardiography, echocardiography, cardiac magnetic resonance imaging, laboratory, and others) and presented to experts and one patient research partner, who discussed the data and added their opinion. This led to the formulation of overarching principles and guidance statements, then reviewed and voted on for agreement. Consensus was attained when the mean agreement was ⩾7/10 and of ⩾70% of voters. Results: Among 2650 publications, 168 met eligibility criteria; the data extracted were discussed over three meetings. Seven overarching principles and 10 guidance points were created, revised and voted on. The consensus highlighted the importance of patient counseling, differential diagnosis and multidisciplinary team management, as well as defining screening and diagnostic approaches. The initial core evaluation should integrate history, physical examination, rest electrocardiography, trans-thoracic echocardiography and standard serum cardiac biomarkers. Further investigations should be individually tailored and decided through a multidisciplinary management. The overall mean agreement was 9.1/10, with mean 93% of experts voting above 7/10. Conclusion: This consensus-based guidance on screening, diagnosis and follow-up of systemic sclerosis primary heart involvement provides a foundation for standard of care and future feasibility studies that are ongoing to support its application in clinical practice.

Digital Ulcers and Ventricular Arrhythmias as Red Flags to Predict Replacement Myocardial Fibrosis in Systemic Sclerosis

“…The association between a history of DUs and replacement myocardial fibrosis may suggest a common pathological substrate pointing to an abnormal vascular function underlying cutaneous and cardiac complications, whereas the presence of ventricular arrhythmias can be the undesirable consequence of the disarray induced by collagen deposition in the myocardial interstitial space [ 48 , 49 ]. There is consensus on the added value of CMR in the detection of SSc-related cardiac involvement, even in the early, asymptomatic phases [ 9 ]. Given the difficulty of accessing CMR in certain areas, supporting patient selection for this evaluation is an unmet need.…”

“…Myocardial fibrosis is the pathological hallmark of myocardial involvement and has been reported in >50% of cases in necropsy studies [ 5 ]. Myocardial involvement significantly affects the prognosis of the disease [ 6 ], but it is often clinically underestimated [ 7 ]; thus, early detection and monitoring are crucial in SSc management [ 8 , 9 ]. Transthoracic echocardiography (TTE) is the routine imaging tool used to assess cardiac involvement, but it is not able to detect myocardial fibrosis, unless more advanced tools such as integrated backscatter and speckle tracking are employed.…”

Heterogeneity of determining disease severity, clinical course and outcomes in systemic sclerosis-associated interstitial lung disease: a systematic literature review

“…In summary, Medline, Embase, Web of Science databases and Cochrane Library were searched up to 1 August 2023. A single author (CB) with established experience [35][36][37] performed the literature search using the search, strings detailed in online supplemental file 1.…”