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Prediction Model for Ventricular Arrhythmias in ARVC
Study Questions:
Can a model developed based on data from five registries predict the risk of sustained ventricular arrhythmia (VA) in patients with arrhythmogenic right ventricular dysplasia (ARVC)?
Methods:
Patients with a definite diagnosis and no history of sustained VAs or sudden cardiac death (SCD) were enrolled in five registries in North America and Europe. A prediction model estimating annual VA risk was developed using Cox regression with internal validation. Eight potential predictors were prespecified: age, sex, cardiac syncope in the prior 6 months, nonsustained ventricular tachycardia, number of premature ventricular complexes in 24 hours, number of leads with T-wave inversion, and right and left ventricular ejection fractions (LVEFs).
Results:
Among 528 patients with average age of 38 years followed for 4.8 years, 146 (27.7%) experienced sustained VA, defined as SCD, aborted SCD, sustained ventricular tachycardia, or appropriate implantable cardioverter-defibrillator (ICD) therapy. All potential predictors 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 overoptimism (calibration slope of 0.93; 95% CI, 0.92–0.95). The clinical benefit of the model was superior to a current consensus-based ICD placement algorithm with a 21% reduction of ICD placements with the same proportion of protected patients (p < 0.001).
Conclusions:
A simple prediction model using readily available clinical parameters may estimate VA risk and help to guide decisions regarding primary prevention ICDs in patients with ARVC.
Perspective:
The present study estimates the rate of VA events in ARVC patients without a pre-existing history of sustained VA (5.6% per year). This level of risk is consistent with prior reports highlighting the arrhythmic event rates in ARVC being higher than in other types of nonischemic cardiomyopathies. It is believed that the majority of patients with definite ARVC benefit from ICD placement. However, ICD placement is associated with a significant risk of inappropriate shocks in this usually young, active, and otherwise healthy population. The decision curve analysis comparing the utility of the present model to the International Task Force Consensus Statement algorithm suggests that the new model reduces the need for an ICD in about 20% of patients without increasing the proportion of patients with subsequent VA/SCD. The new prediction model may be found at www:arvcrisk.com.
Clinical Topics: Arrhythmias and Clinical EP, Cardiovascular Care Team, Heart Failure and Cardiomyopathies, Prevention, Implantable Devices, EP Basic Science, Genetic Arrhythmic Conditions, SCD/Ventricular Arrhythmias, Atrial Fibrillation/Supraventricular Arrhythmias, Acute Heart Failure
Keywords: Arrhythmias, Cardiac, Arrhythmogenic Right Ventricular Dysplasia, Cardiomyopathies, Death, Sudden, Cardiac, Defibrillators, Implantable, Heart Failure, Primary Prevention, Risk Assessment, Stroke Volume, Syncope, Tachycardia, Ventricular, Ventricular Premature Complexes
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