A Systematic Analysis of the Clinical Outcome Associated with Multiple Reclassified Desmosomal Gene Variants in Arrhythmogenic Right Ventricular Cardiomyopathy Patients

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  Graphical Abstract

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  Fig. 1. Study workflow and scheme of publication selection with information on patients with two or more ARVC-related desmosomal variants and subsequent patient selection for the database. *61 out of the 65 articles overlapped with the systematic PubMed search, ^number of patients after including information from contacted authors. Patient groups are based on the number of (likely) pathogenic ARVC variants after reclassification; P, pathogenic variant; LP, likely pathogenic variant; VUS, variant with uncertain significance; LB, likely Benign variant; B, benign variant

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  Fig. 2. Kaplan–Meier survival analysis. Group 1 (double P/LP variant carriers), Group 2 (one P/LP variant carrier), and Group 3 (no P/LP variant carrier). A All. B, C All, stratified for sex. D, E All, stratified for proband status

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  Fig. 3. Kaplan–Meier survival analysis of homozygous Naxos and homozygous Hutterite founder variant carriers. A Kaplan–Meier curves of homozygous Naxos variant carriers, Group 2 (one P/LP variant carrier) and Group 3 (no P/LP carrier). B Kaplan–Meier curves of homozygous Hutterite variant carriers, Group 2 (one or none P/LP variant carrier), and Group 3 (no P/LP variant carrier)