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Circulation. 2018;138:e272–e391. DOI: 10.1161/CIR.0000000000000549 September 25, 2018 e319 CLINICAL STATEMENTS AND GUIDELINES

Recommendation-Specific Supportive Text

1.	 Sarcoidosis is a systemic granulomatous disease of unknown cause. Pulmonary involvement is most frequent but any organ can be affected. Cardiac involvement, diagnosed by cardiac MRI or positron emission tomography (PET), has been reported in up to 55% of patients with extracardiac disease, while isolated cardiac sarcoidosis was seen in most patients diagnosed with cardiac sarcoidosis in 1 report.S7.6-17 Cardiac manifestations include conduction abnormalities, VA, and depressed ventricular function with or without HF, and these contribute greatly to a higher mortality in cardiac sarcoidosis compared with sarcoidosis without cardiac involvement.S7.6-2 In a 25-year study of 110 patients with cardiac sarcoidosis in Finland with HF at presentation, marked LV dysfunction at diagnosis (LVEF <35%), and isolated cardiac sarcoidosis predicted an adverse outcome.S7.6-1 VA can also occur in patients with relatively normal LV function, some of whom have RV involvement that can mimic arrhythmogenic right ventricular cardiomyopathy. Several reports of patients with cardiac sarcoidosis and ICDs implanted for either primary or secondary prevention of SCD show a high frequency of appropriate ICD therapies,S7.6-3–S7.6-5 supporting use of ICDs for primary and secondary prevention of SCD according to the indications applied for other cardiomyopathies. The frequency of conduction abnormalities often warrants a device that provides bradycardia pacing as well. 2.	 Patients with cardiac sarcoidosis can experience VA and SCD, even if the LVEF is normal, and approaches to identification of patients at risk of SCD despite preserved LV function are not well defined. A number of studies have evaluated the role of cardiac MRI for predicting VA and SCD. A meta-analysis,S7.6-6 which included 760 patients in 10 studies, found that late gadolinium enhancement was associated with increased all-cause mortality and more VA compared with those without late gadolinium enhancement. Applicability is limited by the lack of precise quantification of late gadolinium enhancement burden that may allow for more nuanced risk stratification. Some studies suggested that a threshold effect exists, with extensive LV and RV involvement being a particularly high-risk feature.S7.6-7,S7.6-8 However, late gadolinium enhancement can be present even if the LVEF is >50% and was associated with a risk of death or VT of 4.9% per year compared to 0.24% per year when late gadolinium enhancement was absent in 1 observational study.S7.6-7 PET for assessing inflammation and scar is also being increasingly used, but data are limited. In 1 report, the

Figure 8. prevention of SCD in patients with cardiac sarcoidosis. Colors correspond to Class of Recommendation in Table 1. See Section 7.6 for discussion. *ICD candidacy as determined by functional status, life expectancy, or patient preference. †For recurrent sustained monomorphic VT, refer to Figure 2. CEP indicates electrophysiological; GDMT, guideline-directed management and therapy; ICD, implantable cardioverter-defibrillator; LVEF, left ventricular ejection fraction; MRI, magnetic resonance imaging; PET, positron emission tomography; SCA, sudden cardiac arrest; SCD, sudden cardiac death; VA, ventricular arrhythmia; and VT, ventricular tachycardia.

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