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Arrhythmic Risk Prediction in ARVC
Quick Takes
- The https://ARVCrisk.com calculator was successfully validated on an external cohort and adequately predicts sustained ventricular arrhythmias within 5 years of diagnosis.
- The net clinical benefit of ICD use was better predicted by ARVCrisk than other published clinical statements.
Study Questions:
Does the arrhythmogenic right ventricular cardiomyopathy (ARVC) risk calculator (https://ARVCrisk.com) adequately predict sustained ventricular arrhythmias, and how does it compare to other guidelines or consensus indications for implantable cardioverter-defibrillator (ICD) implantation?
Methods:
This was an observational, retrospective, longitudinal cohort study applying the ARVCrisk calculator to patients diagnosed by 2010 Task Force criteria but not yet having had ventricular arrhythmias (VAs) at the time of diagnosis. Clinical data from initial diagnosis were used for risk prediction. The primary outcome was first VA event. The ARVCrisk calculator was then compared against several guidelines and consensus statements using decision curve analysis to determine utility of ICD use at 5 years between the various decision models.
Results:
The study population was 429 patients, mean age 43 ±15 years; with a 70% incidence of plakophilin-2 (PKP2) mutations in patients with available genetic information. Over 5 years of follow-up, 24% of the cohort experienced a VA event. The annual VA event rate was 5%. Most events were ICD treated. The incidences of sudden cardiac arrest and sudden cardiac death were 6.8% and 2.9%, respectively. Model validation showed good agreement (Harrell C-index, 0.7) between predicted and observed at 1 and 5 years. The ARVCrisk model outperformed Task Force, American Heart Association, and Heart Rhythm Society guideline/consensus statements to predict net clinical benefit of ICD use.
Conclusions:
The ARVCrisk calculator provides good prognostic data to predict VAs and outperforms other published clinical statements on predicted ICD benefit.
Perspective:
Genetic arrhythmia syndromes such as ARVC, with varying genotypes and severities of phenotypic manifestations, present a prognostic challenge where the risk of a life-threatening event must be weighed against the risk and cost of the treatment. Risk calculators such as ARVCrisk provide a tool to better assess that risk; and in this case, may demonstrate the superiority of data-derived algorithms to other evidence-based or consensus agreement.
Clinical Topics: Arrhythmias and Clinical EP, Cardiovascular Care Team, Heart Failure and Cardiomyopathies, Prevention, Implantable Devices, 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, Genotype, Heart Arrest, Heart Failure, Mutation, Risk Assessment, Secondary Prevention
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