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September 25, 2018 Circulation. 2018;138:e272–e391. DOI: 10.1161/CIR.0000000000000549 e310

CLINICAL STATEMENTS AND GUIDELINES therapy arms. Therefore, the results of DANISH should not be generalized to patients with NICM who are ineligible for CRT. During a median follow-up of 5.6 years, ICD reduced SCD from 8.4% to 4.3%, but there was no difference in all-cause mortality.S7.2.2-11 Several meta-analyses have been published.S7.2.2-12,S7.2.2-13 One provided data on ICDs with and without CRT and showed survival benefit from the ICD.S7.2.2-13 The second used patient level data from 2 trials and adopted a more robust approach to reducing heterogeneity by excluding patients with CRT and those randomized to antiarrhythmic medications; a 25% relative risk reduction in mortality with an ICD was shown.S7.2.2-12

2.	 Laminopathies are diseases caused by mutations mainly in the Lamin A/C gene that produce various inherited diseases including subtypes of muscular dystrophy and progeria. Isolated cardiac involvement is also observed and is an important cause of familial cardiomyopathy.S7.2.2-9 The disease is highly penetrant such that all affected individuals have evidence of disease by 60 years of age. Cardiac manifestations may include atrial fibrillation, conduction disturbances, VA, and NICM. A number of observational studies reported a high risk of SCD when cardiac involvement is present.S7.2.2-7–S7.2.2-10 One study reported SCD as the most frequent mode of death (46%) in both the isolated cardiac and the neuromuscular phenotypes of Lamin diseases.S7.2.2-9 In a cohort of 269 LMNA mutation positive individuals,S7.2.2-10 NSVT during ambulatory electrocardiographic monitoring, LVEF <45% at first evaluation, male sex, and nonmissense mutations were independent risk factors for VA. Malignant VA were observed only in persons with ≥2 of these risk factors.S7.2.2-10 No studies have tested the effect of the ICD on longterm survival. 3.	 Patients with NICM and class I HF symptoms were not included in SCD-HeFT or DANISH.S7.2.2-1,S7.2.2-11 Although such patients were included in the DEFINITE trial, only 99 (21.6%) of 458 patients in the DEFINITE trial had class I HF.S7.2.2-5 Therefore, it is uncertain whether a primary prevention ICD in such patients improves survival. 4.	 There are insufficient data from RCTs regarding the value of the ICD in patients with NYHA class IV. Ambulatory class IV HF patients were included in the COMPANION trial that, overall, showed improved functional status and survival with a CRT defibrillator.S7.2.2-3 Unless such a patient is a candidate for CRT or advanced HF therapies such as heart transplantation or an LVAD, an ICD is not expected to meaningfully prolong survival.S7.2.2-3 7.2.3 Treatment of Recurrent VA in Patients With NICM

Recommendations for Treatment of Recurrent VA in Patients With NICM References that support the recommendations are summarized in Online Data Supplement 29.

COR LOE Recommendations

IIa B-R 1. In patients with NICM and an ICD who experience spontaneous VA or recurrent appropriate shocks despite optimal device programming and treatment with a beta blocker, amiodarone or sotalol can be beneficial.S7.2.3-1

IIa B-NR 2. In patients with NICM and recurrent sustained monomorphic VT who fail or are intolerant of antiarrhythmic medications, catheter ablation can be useful for reducing recurrent VT and ICD shocks.S7.2.3-2,S7.2.3-3

Recommendation-Specific Supportive Text

1.	 ICDs reduce mortality from VA, yet ICD shocks are painful and associated with significant morbidity and poor QoL. Although ICDs are highly programmable and provide antitachycardia pacing therapy that can terminate most VT episodes without the need for a shock, prevention of shocks, both appropriate and inappropriate, remains an important concern. In the OPTIC (Optimal Pharmacological Therapy in Cardioverter Defibrillator Patients) study, 412 patients with documented VT and VF who received an ICD within 21 days of the documented arrhythmiaS7.2.3-1 were randomized to amiodarone plus beta blocker, sotalol alone, or beta blocker alone. Over 1 year, shocks occurred in 38.5% assigned to beta blocker alone, 24.3% assigned to sotalol, and 10.3% assigned to amiodarone plus beta blocker. The rates of study medication discontinuation at 1 year were 18.2% for amiodarone, 23.5% for sotalol, and 5.3% for beta blocker alone. Adverse pulmonary and thyroid events and symptomatic bradycardia were more common among patients randomized to amiodarone. Thus, amiodarone plus beta blocker were more effective than sotalol in preventing ICD shocks but at the expense of increased risk of medication-related adverse effects.S7.2.3-1 Sotalol should not be used in patients with an LVEF <20% due to its negative inotropic effects. 2.	 Sustained monomorphic VT due to NICM is most often due to scar-related reentry. Cardiac MRI often indicates scar location, which tends to be basal along the mitral annulus or in the septum.S7.2.3-4,S7.2.3-5 The VT substrate can be

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