Long-Term Arrhythmic Follow-Up and Risk Stratification of Patients With Desmoplakin-Associated Arrhythmogenic Right Ventricular Cardiomyopathy

Figures

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  Figure 1. Freedom From Ventricular Arrhythmia Events in the Overall Study Cohort Kaplan-Meier Curve Displaying Overall Cohort Freedom From Ventricular Arrhythmias OverFollow-Up

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  Figure 2. Differences in Arrhythmic Outcomes Depending on LV Involvement Freedom from ventricular arrhythmia in the overall (right panel) and primary prevention (left panel) cohorts, stratified by LV involvement both panels show a higher occurrence of ventricular arrhythmia events in those patients in which LV involvement is present. Shaded area around the KM curve represents the 95% freedom from ventricular arrhythmia CI. Numbers reported below represents patients at risk (top row: no LV involvement; bottom row: LV involvement); KM = Kaplan Meier; LV = left ventricular.

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  Figure 3. Performance of the ARVC Risk Score in DSP-TFC+ Patients Performance of the ARVC risk calculator in DSP-TFC+ patients calibration plot for the performance of the ARVC risk calculator in the primary prevention cohort overall (A) and stratified on the presence (B) or absence (C) of left ventricular involvement. Average risk predicted by the ARVC risk calculator is plotted against the observed VA risk and associated 95% CI as estimated by the Kaplan-Meier method for each quintile (blue line). The linearity of this relationship is assessed using linear regression, and the coefficient of determination (r2) reflects the degree to which this relationship is appropriately characterized by a linear model. The red line reflects perfect, linear risk-prediction; risk predictions falling to the upper left of this line represent model underprediction, while risk prediction falling to the lower right of this line represent model overpredictions. ARVC = arrhythmogenic right ventricular cardiomyopathy; LV = left ventricular; VA = ventricular arrhythmias.

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  Central Illustration. Arrhythmia Risks in Patients With Desmoplakin-Associated Arrhythmogenic Right VentricularCardiomyopathy