Abstract
Cardiac disease is a leading cause of death in systemic sclerosis (SSc) and sudden cardiac death (SCD) is thought to occur more commonly in SSc than in the general population. Diffuse myocardial fibrosis, myocarditis and ischaemic heart disease are all prevalent in SSc and can be reasonably hypothesised to contribute to an increased risk of SCD. Despite this, SCD remains a relatively understudied area of SSc with little understood about SSc-specific risk factors and opportunities for primary prevention. In this review, we present an overview of the possible mechanisms of SCD in SSc and our current understanding of how each of these mechanisms may contribute to cardiac death. This review highlights the need for a future research agenda that addresses the underlying epidemiology of SCD in SSc and identifies opportunities for intervention to modify the disease course of heart disease in SSc.
Highlights
Systemic sclerosis (SSc) is a rare multi-system autoimmune disease with significant cardiac complications
The risk of death from SSc, and in particular heart disease in SSc, has remained largely unchanged for over 40 years [5]. This includes the risk of sudden cardiac death (SCD) which is reported to occur in up to 9% of individuals with
SCD is a frequent cause of death for patients with heart failure with reduced ejection fraction (HFrEF), rates have reduced with optimization of medical therapy, cardiac resynchronisation therapy and use of implantable cardioverter-defibrillators (ICD) [40,41]
Summary
Systemic sclerosis (SSc) is a rare multi-system autoimmune disease with significant cardiac complications. The risk of death from SSc, and in particular heart disease in SSc, has remained largely unchanged for over 40 years [5]. This includes the risk of sudden cardiac death (SCD) which is reported to occur in up to 9% of individuals with. Sudden cardiac arrest or SCD is the the risk of sudden cardiac death (SCD) which is reported to occur in up to 9% of individuals with SSc, [8,9,10,11,12,13,14,15] increasing to 67% in patients with clinically overt SHI [10]. Search agenda to better understand the risk and primary prevention of SCD in SSc
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