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September 25, 2018 Circulation. 2018;138:e272–e391. DOI: 10.1161/CIR.0000000000000549 e318 CLINICAL STATEMENTS AND GUIDELINES benign.S7.4-34,S7.4-35 Data from series of unselected consecutive outpatients suggest that most mutations are novel and limited to particular families.S7.4-34,S7.4-35 Therefore, routine mutation screening would appear to be of little prognostic value in HCM.S7.4-52 The short-term risk of sudden death in patients who are genotype positive but have no other manifestations of the disease appears to be low.S7.4-53 Therefore, an ICD is not indicated in these individuals. 7.5 Myocarditis Recommendations for Myocarditis References that support the recommendations are summarized in Online Data Supplement 32. COR LOE Recommendations I C-LD 1. In patients with life-threatening VT or VF associated with confirmed or clinically suspected myocarditis, referral to centers with mechanical hemodynamic support and advanced arrhythmia management is recommended.S7.5-1 IIb C-LD 2. In patients with giant cell myocarditis with VF or hemodynamically unstable VT treated according to GDMT, an ICD and/or an antiarrhythmic medication may be considered if meaningful survival of greater than 1 year is expected.S7.5-2–S7.5-4 Recommendation-Specific Supportive Text 1. Myocarditis is an inflammatory process often related to infection.S7.5-1,S7.5-5–S7.5-9 When patients are treated in centers with the availability of mechanical hemodynamic support procedures, cardiac catheterization, endomyocardial biopsy, advanced cardiac imaging procedures, and arrhythmia management including ICD implantation, outcomes appear improved.S7.5-1 The acute course of myocarditis varies ranging from an asymptomatic finding of transient ST-T changes noted on ECG to cardiogenic shock and recurrent VA.S7.5-10–S7.5-12 Acute management is largely supportive and can rapidly advance to requiring mechanical support.S7.5-13,S7.5-14 Cardiac arrhythmias range from conduction abnormalities to life-threatening VT and VF.S7.5-15–S7.5-17 Arrhythmias may require antiarrhythmic medications and/or device therapy.S7.5-18 Giant cell myocarditis is fairly uncommon, but it is of particular importance because it typically affects young individuals and is usually fatal if untreated.S7.5-2–S7.5-4,S7.5-19 VT may require antiarrhythmic medications such as amiodarone and/ or an ICD that in some instances can be used as a bridge to more advanced HF therapies such as LVAD or transplant. Myocarditis and SCD have been reported with HIV infection.S7.5- 20,S7.5-21 Systemic lupus erythematous can cause myocarditis but only rarely VT or VF.S7.5-8,S7.5-22 In patients with Chagas disease, acute myocarditis is rare but more than one third of affected patients develop late myocardial damage with progressive HF. Conduction defects with progression to complete heart block and VT or VF are common. Amiodarone appears to be effective in treating VA.S7.5-23 An ICD is frequently used in the late phase of myocarditis,S7.5-24 and radiofrequency catheter ablation has been successfully used to control recurrent VA in some patietnts.S7.5-25 2. Giant cell myocarditis is fairly uncommon, but it is of particular importance as it typically affects young individuals and is usually fatal if untreated. The diagnosis is confirmed by endomyocardial biopsy. Patients may develop heart block, requiring a temporary or a permanent pacemakers. An ICD and antiarrhythmic medications, such as amiodarone are often used in the acute phase to treat VT or VF and reduce the risk of SCD.S7.5-2–S7.5-4,S7.5-19,S7.5-26–S7.5-28 7.6 Cardiac Sarcoidosis Recommendations for Cardiac Sarcoidosis References that support the recommendations are summarized in Online Data Supplement 33. COR LOE Recommendations I B-NR 1. In patients with cardiac sarcoidosis who have sustained VT or are survivors of SCA or have an LVEF of 35% or less, an ICD is recommended, if meaningful survival of greater than 1 year is expected.S7.6-1–S7.6-5 IIa B-NR 2. In patients with cardiac sarcoidosis and LVEF greater than 35% who have syncope and/ or evidence of myocardial scar by cardiac MRI or positron emission tomographic (PET) scan, and/or have an indication for permanent pacing, implantation of an ICD is reasonable, provided that meaningful survival of greater than 1 year is expected.S7.6-6–S7.6-10 IIa C-LD 3. In patients with cardiac sarcoidosis and LVEF greater than 35%, it is reasonable to perform an electrophysiological study and to implant an ICD, if sustained VA is inducible, provided that meaningful survival of greater than 1 year is expected.S7.6-11,S7.6-12 IIa C-LD 4. In patients with cardiac sarcoidosis who have an indication for permanent pacing, implantation of an ICD can be beneficial.S7.6-13 IIa C-LD 5. In patients with cardiac sarcoidosis with frequent symptomatic VA and evidence of myocardial inflammation, immunosuppression in combination with antiarrhythmic medication therapy can be useful to reduce VA burden.S7.6-14–S7.6-16 Downloaded from http://ahajournals.org by on October 7, 2026