International expert agreement on World Scleroderma Foundation/Heart Failure Association consensus-based definition and guidance on screening, diagnosis, and follow-up assessment of systemic sclerosis-associated primary heart involvement.

Objective: The aim of this study was to evaluate the level of agreement among international experts on the World Scleroderma Foundation/Heart Failure Association consensus-based definition of systemic sclerosis-associated primary heart involvement (SSc-pHI) and on the guidance for its screening, diagnosis, and follow-up assessment, including feasibility and applicability assessments.

Methods: An online survey was conducted to assess the level of agreement and feasibility/applicability using a 10-point scale (0 = not at all, 10 = completely agree or completely feasible/applicable). The weblink to the survey was shared with 1199 participants worldwide. A high level of agreement was defined by an average score over 7/10.

Results: In total, 161 external experts completed the survey. Most of them were rheumatologists (80.7%), working in Europe (81.4%), and had > 10 years of clinical experience in managing SSc patients (59%). Overall agreement among external experts was high (mean 8.27, SD 1.86). The highest scores regarded items emphasizing the involvement of a multidisciplinary team, personalization of patient management, and initial evaluation techniques. A lower level of agreement was obtained in questions related to cardiac magnetic resonance imaging and endomyocardial biopsy. No factors associated with low level of agreement and feasibility/applicability were identified.

Conclusion: The consensus-based definition and guidance on screening, diagnosis, and follow-up assessment of SSc-pHI provides a basis for application by the wider community. A lower level of agreement on the use of advanced or more invasive diagnostic techniques likely reflects regional differences in access and the need for more evidence on its use. This emphasizes the importance of involving a multidisciplinary team.