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Sarcoidosis is a progressive disease of unclear aetiology with most evidence supporting an aberrant immune response to an unidentified antigen(s) in genetically predisposed individuals. Clinical cardiac manifestations occur in 5% of patients with systemic sarcoidosis. Subclinical cardiac involvement has been described in 20%–25% of patients in autopsy studies with further validation from cardiovascular MRI studies. Cardiac sarcoidosis (CS) is characterised by inflammatory non-caseating myocardial granulomas and myocardial fibrosis. Importantly, this condition is associated with various cardiac manifestations, ranging from heart failure to conduction defects, ventricular arrhythmias and sudden cardiac death (SCD). Recent registry findings of 351 patients in the MIDFIN registry from Ekström et al suggest that up to 14% of patients with CS had a presenting manifestation of SCD.1 Furthermore, of all the deaths recorded in this registry, 64% (n=54/84) were unexpected SCD and cardiac involvement detected at autopsy. The emerging data on the association of ventricular arrhythmias with myocardial scar in patients with CS, diagnosed by cardiac MRI, have led to its incorporation into the 2017 American College of Cardiology/American Heart Association/Heart Rhythm Society (ACC/AHA/HRS) guidelines for primary prevention of SCD.2 As a result, a large proportion of patients diagnosed with CS now fulfil class I or IIa criteria for implantable cardioverter defibrillator (ICD) implantation. A recent meta-analysis of 19 studies found crude rates of SCD/aborted SCD are high in primary prevention (23%) and secondary prevention (58%) of patients with CS.3 In this condition that is already challenging to diagnose and detect, the risk of SCD is high, and an appropriate selection of patients for appropriate ICD therapy is crucial.
Despite this, over the years, risk stratification for SCD …
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Twitter @KyleBFranke, @Rajiv_EP
Contributors Initial conception or design: KBF. Drafting the work and revising it critically: RM. Final drafting: KBF and RM. Final approval: KBF. Both authors are in agreement to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Competing interests Financial disclosures: Dr RM is supported by the Mid-Career Fellowship by The Hospital Research Fund. The University of Adelaide reports receiving on behalf of Dr RM lecture and/or consulting fees from Abbott, Medtronic, Bayer and Pfizer. The University of Adelaide reports receiving on behalf of Dr RM research funding from Bayer, Abbott and Medtronic.
Patient and public involvement Patients and/or the public were not involved in the design, or conduct, or reporting or dissemination plans of this research.
Provenance and peer review Not commissioned; internally peer reviewed.
Linked Articles
- Arrhythmias and sudden death