PLN Mutation Arrhythmic Risk Model (PLN Risk Calculator)

PLN p.Arg14del arrhythmic risk model — explanation and clinical context This tool implements the primary phenotypic predictors selected in the multivariable PLN p.Arg14del prediction model published by Verstraelen et al. (European Heart Journal, 2021): left ventricular ejection fraction (LVEF; model parameterized per 1% decrease), 24-hour premature ventricular contraction (PVC) count (log-transformed), number of leads with negative T-waves (count of precordial + inferior leads with T-wave inversion), and the presence of a low-voltage ECG. The published model demonstrated excellent discrimination (C-statistic ≈ 0.83) and improved performance compared with the earlier PLN model. This page computes a transparent relative hazard index (exp(linear predictor)) using the published multivariable hazard ratios (converted to log-coefficients).

It is intended for clinician use as a comparative risk index across patients but is not a substitute for the exact 5-year absolute risk output from the authors' supplementary model (which requires the baseline hazard/intercept to translate the linear predictor to absolute risk). Clinical interpretation summary: The model shows that lower LVEF, higher (log) PVC burden, more leads with negative T waves, and presence of low-voltage ECG are independently associated with higher malignant ventricular arrhythmia risk among PLN p.Arg14del carriers. Shared decision-making about primary-prevention ICD implantation should consider the absolute predicted risk (authors suggest a 5-year risk threshold of >5% as a reasonable minimum to consider ICD), comorbidities, patient preferences, and local practice.

For precise absolute risk use the original online calculator or consult the paper's supplementary material.