Abstract

The growing interest in the prevention of sudden cardiac death (SCD) has drawn attention to MVP as a possible substrate of cardiac arrest. Although poorly understood, the current understanding of arrhythmogenesis in MVP involves the development of a substrate for arrhythmias (cardiac fibrosis) combined with a trigger for arrhythmias. In general, MVP has a low prevalence; its prevalence in patients who died from sudden cardiac death (SCD) remains unclear. We aimed to assess the prevalence of MVP in patients with autopsy-proven SCD. A systematic search, without language restriction, using PubMed, EMBASE, SCOPUS, Google Scholar, and ClinicalTrials.gov was performed. The meta-analysis was performed using a meta-package for R version 4.0/RStudio version 1.2 and Freeman Tukey double arcsine method to establish the variance of raw proportions. The outcomemeasured was the prevalence of MVP in patients who died with SCD, proven by autopsy. We also aimed to evaluate prevalence stratified by region. This systematic review of nineteen studies (seven from North America, eleven from Europe, and one from Asia) incorporated a total of 6,642 patients with autopsy-proven SCD (mean age 38.4±16.1 years, 80% men). Fifteen studies exclusively incorporated the general population, three included young athletes, and one included military recruits. Of all SCDs, the prevalence of MVP was 2.2% (95% CI 1.2-3.5%) with a high degree of heterogeneity I2=88%, Χ2 p<0.01). When stratified by region, the prevalence of MVP among SCD patients was 6.7% (95% CI 3.5-10.9%) in Italy, 2.85% (95% 1-5.6%) in the United States, 2.4% (95% CI 1.6-3.3%) in the United Kingdom and 1.7% (95% CI 0.2-6%) in Israel. The overrepresentation of MVP in unexplained SCD cohorts indicates an ‘at-risk’ subset and poses significant concern in light of the large MVP population in the general population.

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