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Circulation. 2018;138:e272–e391. DOI: 10.1161/CIR.0000000000000549 September 25, 2018 e311 CLINICAL STATEMENTS AND GUIDELINES

Figure 6. Secondary and primary prevention of SCD in patients with NICM. Colors correspond to Class of Recommendation in Table 1. See Section 7.2 for discussion. *ICD candidacy as determined by functional status, life expectancy or patient preference. 2° indicates secondary; EP, electrophysiological; GDMT, guideline-directed management and therapy; HF, heart failure; ICD, implantable cardioverter-defibrillator; LVEF, left ventricular ejection fraction; NICM, nonischemic cardiomyopathy; SCA, sudden cardiac arrest; SCD, sudden cardiac death; VA, ventricular arrhythmia; and WCD, wearable cardioverter-defibrillator.

subendocardial, subepicardial, or intramyocardial, and all locations may be affected and require endocardial and epicardial ablation. In the HELP-VT (Heart Center of Leipzig VT) study,S7.2.3-2 successful ablation of all VT morphologies was achieved in 66.7% of patients with NICM, compared with the 77.4% success rate in ischemic cardiomyopathy. An epicardial approach to ablation was required in 30.2% of NICM patients, compared with only 1.2% with ischemic cardiomyopathy. Epicardial ablation was an independent predictor of successful ablation. Acute and long-term success of ablation is lower for NICM, compared with post-MI patients. The long-term survival-free of VT recurrence after catheter ablation appears to be better for patients with ischemic than NICM (57% versus 40.5% at 1 year).S7.2.3-2 Risks are similar to those observed for post-MI VT ablation, with additional risks of epicardial access and ablation when required. Although any NICM can produce scar-related VT, cardiac sarcoidosis (see Section 7.6) and Lamin mutations are particularly associated with sustained monomorphic VT.S7.2.3-6 7.3 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 1. 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 2. 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 3. 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 expected.S7.3-9–S7.3-13

I B-NR 4. In patients with arrhythmogenic right ventricular cardiomyopathy and VA, a beta blocker is recommended.S7.3-11,S7.3-14,S7.3-15

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