2-year risk of sustained ventricular tachycardia in newly diagnosed ARVC patients
The ARVC risk calculator is based on clinical data of patients fulfilling ARVC diagnosis from 14 academic centers worldwide. It estimates the risk of sustained ventricular tachycardia in newly diagnosed patients who fulfill 2010 TFC for definite diagnosis of ARVC but have not experienced prior sustained ventricular arrhythmias.

The aim of the individualized predictions is to aid patients and clinicians in their decision to implant an implantable cardioverter-defibrillator for primary prevention of sudden cardiac arrest.
Research authors: Cadrin-Tourigny J, Bosman LP, Nozza A, Wang W, Tadros R, Bhonsale A, et al.
Details Custom formula Study characteristics Files & References
★★★
Model author
Model ID
1785
Version
1.3
Revision date
2019-03-28
MeSH terms
  • Ventricular Tachycardia
  • Cardiomyopathy
  • Arrythmia
  • Implantable Defibrillators
  • Sudden Cardiac Death
  • Model type
    Custom model (Calculation)
    Status
    public
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    Formula

    Additional information

    Aims:
    Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVC) is characterized by ventricular arrhythmias (VAs) and sudden cardiac death (SCD). We aimed to develop a model for individualized prediction of incident VA/SCD in ARVC patients.

    Methods and results:
    Five hundred and twenty-eight patients with a definite diagnosis and no history of sustained VAs/SCD at baseline, aged 38.2 ± 15.5 years, 44.7% male, were enrolled from five registries in North America and Europe. Over 4.83 (interquartile range 2.44–9.33) years of follow-up, 146 (27.7%) experienced sustained VA, defined as SCD, aborted SCD, sustained ventricular tachycardia, or appropriate implantable cardioverter-defibrillator (ICD) therapy. A prediction model estimating annual VA risk was developed using Cox regression with internal validation. Eight potential predictors were pre-specified: age, sex, cardiac syncope in the prior 6 months, non-sustained ventricular tachycardia, number of premature ventricular complexes in 24 h, number of leads with T-wave inversion, and right and left ventricular ejection fractions (LVEFs). All except LVEF were retained in the final model. The model accurately distinguished patients with and without events, with an optimism-corrected C-index of 0.77 [95% confidence interval (CI) 0.73–0.81] and minimal over-optimism [calibration slope of 0.93 (95% CI 0.92–0.95)]. By decision curve analysis, the clinical benefit of the model was superior to a current consensus-based ICD placement algorithm with a 20.6% reduction of ICD placements with the same proportion of protected patients (P < 0.001).

    Conclusion:
    Using the largest cohort of patients with ARVC and no prior VA, a prediction model using readily available clinical parameters was devised to estimate VA risk and guide decisions regarding primary prevention ICDs. 

    Study Population

    Total population size: 528
    Males: {{ model.numberOfMales }}
    Females: {{ model.numberOfFemales }}

    Continuous characteristics

    Name Mean SD Unit
    Age at diagnosis 38.16 15.47 years
    RVEF 43.80 10.4 %
    LVEF 57.66 8.42 %
    Name LL Q1 Median Q3 UL Unit
    24 h PVC count 278 1007 3731 PVCs

    Categorical characteristics

    Name Subset / Group Nr. of patients
    Proband status Yes 263
    No 265
    Pathogenic mutation PKP2 258
    DSP 23
    DSG2 17
    PLN 26
    Multiple mutations 6
    Other 10
    Ethnicity Caucasian 485
    Other 43
    NVST Yes 231
    No 297
    ICD Yes 218
    No 310
    Beta-blockers Yes 200
    No 328
    Anti-arrhythmic drugs Yes 82
    No 446

    Risk of sustained ventricular tachycardia within 2 years:
    ...
    %

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    Result
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    Risk of sustained ventricular tachycardia within 2 years: %

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    Outcome stratification

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    Conditional information

    Result interpretation

    How this model should be used:1
    This model estimates the risk of sustained ventricular tachycardia in newly diagnosed patients who fulfill 2010 TFC for definite diagnosis of ARVC but have not experienced prior sustained ventricular arrhythmias. The aim of the individualized predictions is to aid patients and clinicians in their decision to implant an implantable cardioverter-defibrillator for primary prevention of sudden cardiac arrest.

    Please consider the following limitations:1

    • This model was has not (yet) been validated in external cohorts.
    • The calculator should not be used in patients with prior sustained ventricular arrhythmia or sudden cardiac arrest.
    • The calculator is designed to provide predictions based on the clinical characteristics of ARVC patients at time of their diagnosis.2
    • Caution should be exercised when interpreting the result for pediatric patients <14 years of age.
    References:
    1. Cadrin-Tourigny J, Bosman LP, Nozza A, , etl al. A new prediction model for ventricular arrhythmias in arrhythmogenic right ventricular cardiomyopathy. European Heart J 2019. 
    2. Marcus FI, McKenna WJ, Sherrill D, et al. Diagnosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia: proposed modification of the Task Force Criteria. Eur Heart J 2010;31:806-814. 

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    Calculations alone should never dictate patient care, and are no substitute for professional judgement. See our full disclaimer.

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