Arrhythmic Risk Stratification for Arrhythmogenic Right Ventricular Cardiomyopathy
Should We Ask Who Is at High Risk or Who Is at Low Risk?
This article requires a subscription to view the full text. If you have a subscription you may use the login form below to view the article. Access to this article can also be purchased.
- arrhythmogenic right ventricular dysplasia
- defibrillators, implantable
- tachycardia, ventricular
- ventricular fibrillation
See Article by Orgeron et al
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a progressive disorder characterized by morphological changes in the myocardium that place the patient at risk for lethal ventricular arrhythmias. A genetic mutation involving desmosomes is seen in most patients1 that affects myocyte cell-to-cell adhesion. Athletes are likely to express this disease at an earlier age as exercise promotes progression of the disease. Because either ventricle can be affected, the term arrhythmogenic cardiomyopathy has been proposed.1,2 Diagnosis is based on criteria as set out in the 2010 Task Force document,3 encompassing ventricular structure, histology, electrocardiographic features, arrhythmias, and genetic features.
In 2015, an International Task Force published a consensus statement to provide clinical practice guidance based on available evidence and expert opinion.4 For implantable cardioverter defibrillator (ICD) implantation, the Task Force assigned a class I recommendation to patients who had been resuscitated from hemodynamically unstable ventricular tachycardia (VT) or ventricular fibrillation (VF) and for patients with severe right ventricular or left ventricular systolic dysfunction because these patients were felt to have life-threatening arrhythmic events >10% per year.4 The Task Force advised that an ICD should be considered (class IIa) for patients with hemodynamically stable sustained VT, syncope, nonsustained VT, or moderate ventricular dysfunction.4 A class IIb (may be considered) designation was assigned for those with minor risk factors, such as male sex, proband status, inducibility on electrophysiological study, or at least 3 precordial leads with T-wave inversion.4 Finally, class III (not recommended) was assigned to asymptomatic patients with no risk factors or healthy gene carriers.4
Since the publication of the International Task Force consensus statement, larger observational studies have added to our understanding of this disease. In 416 …