Arrhythmogenic Right Ventricular Cardiomyopathy Recommendations for Arrhythmogenic Right Ventricular Cardiomyopathy References that support the recommendations are summarized in Online Data Supplement 30. COR LOE Recommendations I B-NR In selected first-degree relatives of patients with arrhythmogenic right ventricular cardiomyopathy, clinical screening for the disease is recommended along with genetic counseling and genetic testing, if the proband has a disease causing mutation.S7.3-1 S7.3-4 I B-NR In patients with suspected arrhythmogenic right ventricular cardiomyopathy and VA or electrocardiographic abnormalities, cardiac MRI is useful for establishing a diagnosis and for risk stratification.S7.3-5 S7.3-8 I B-NR In patients with arrhythmogenic right ventricular cardiomyopathy and an additional marker of increased risk of SCD (resuscitated SCA, sustained VT, significant ventricular dysfunction with RVEF or LVEF 35%), an ICD is recommended if meaningful survival greater than 1 year is I B-NR In patients with arrhythmogenic right ventricular cardiomyopathy and VA, a beta blocker is recommended.S7.3-11,S7.3-14,S7.3-15 CLINICAL STATEMENTS AND GUIDELINES I B-NR In patients with a clinical diagnosis of arrhythmogenic right ventricular cardiomyopathy, avoiding intensive exercise is IIa B-NR In patients with clinically diagnosed or suspected arrhythmogenic right ventricular cardiomyopathy, genetic counseling and genetic testing can be useful for diagnosis and for gene-specific targeted family IIa B-NR In patients with arrhythmogenic right ventricular cardiomyopathy and syncope presumed due to VA, an ICD can be useful if meaningful survival greater than 1 year is IIa B-NR In patients with clinical evidence of arrhythmogenic right ventricular cardiomyopathy but not VA, a beta blocker can be useful.S7.3-14,S7.3-15 IIa B-NR In patients with arrhythmogenic right ventricular cardiomyopathy and recurrent symptomatic sustained VT in whom a beta blocker is ineffective or not tolerated, catheter ablation with availability of a combined endocardial/epicardial approach can be beneficial.S7.3-27 S7.3-33 IIa B-NR In patients with suspected arrhythmogenic right ventricular cardiomyopathy, a signal averaged ECG can be useful for diagnosis and risk stratification.S7.3-14,S7.3-34,S7.3-35 IIb B-NR In asymptomatic patients with clinical evidence of arrhythmogenic right ventricular cardiomyopathy, an electrophysiological study may be considered for risk Synopsis Arrhythmogenic right ventricular cardiomyopathy is an inherited cardiomyopathy that predominantly af- fects the right ventricle but can affect the left ven- tricle causing areas of myocardial replacement with fibrosis and adipose tissue that frequently causes VA and SCD. 