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Background: The 2014 Heart Rhythm Society consensus statement defines histological (definite) and clinical (probable) diagnostic categories of cardiac sarcoidosis (CS), but few studies have compared their arrhythmic phenotypes and outcomes., Objective: The purpose of this study was to evaluate the electrophysiological/arrhythmic phenotype and outcomes of patients with definite and probable CS., Methods: We analyzed the arrhythmic/electrophysiological phenotype in a single-center North American cohort of 388 patients (median age 56 years; 39% female, n = 151) diagnosed with definite (n = 58) or probable (n = 330) CS (2000-2022). The primary composite outcome was survival to first ventricular tachycardia/fibrillation (VT/VF) event or sudden cardiac death. Key secondary outcomes were also assessed., Results: At index evaluation, in situ cardiac implantable electronic devices and antiarrhythmic drug use were more common in definite CS. At a median follow-up of 3.1 years, the primary outcome occurred in 22 patients with definite CS (38%) and 127 patients with probable CS (38%) (log-rank, P = .55). In multivariable analysis, only a higher ratio of the 18 F-fluorodeoxyglucose maximum standardized uptake value of the myocardium to the maximum standardized uptake value of the blood pool (hazard ratio 1.09; 95% confidence interval 1.03-1.15; P = .003, per 1 unit increase) was associated with the primary outcome. During follow-up, patients with definite CS had a higher burden of device-treated VT/VF events (mean 2.86 events per patient-year vs 1.56 events per patient-year) and a higher rate of progression to heart transplant/left ventricular assist device implantation but no difference in all-cause mortality compared with patients with probable CS., Conclusion: Patients with definite and probable CS had similarly high risks of first sustained VT/VF/sudden cardiac death and all-cause mortality, though patients with definite CS had a higher overall arrhythmia burden. Both CS diagnostic groups as defined by the 2014 Heart Rhythm Society criteria require an aggressive approach to prevent arrhythmic complications., Competing Interests: Disclosures No relevant disclosures by any of the authors., (Copyright © 2024 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.)

Background: Current guidelines present varying classes of recommendations for implantable cardioverter-defibrillator (ICD) utilization in patients with cardiac sarcoidosis (CS) and left ventricular ejection fraction (LVEF) <50%., Objective: The purpose of this study was to investigate the ventricular arrhythmia risk in CS patients with ICDs and varying degrees of left ventricular systolic dysfunction., Methods: The study included CS patients with an ICD and LVEF <50% at index evaluation. The primary outcome was survival free of sustained ventricular tachycardia (VT)/ventricular fibrillation (VF) after ICD implantation and was assessed comparatively for LVEF ≤35% vs 36%-49% and for primary vs secondary prevention ICD indication., Results: The study included 61 patients (median age 57 years; 61% male) with LVEF 36%-49% (n = 23) or LVEF ≤35% (n = 38). An ICD was implanted for secondary prevention in 24% and 44% of the LVEF ≤35% and 36%-49% groups, respectively (P = .11). The primary outcome did not differ between the 2 groups in univariable analysis (LVEF ≤35% vs 36%-49%: hazard ratio [HR] 0.85; 95% confidence interval [CI] 0.39-1.82; P = .67). In multivariable analysis, secondary prevention ICD indication was the only significant predictor of incident sustained VT/VF (HR 2.86; 95% CI 1.23-6.67; P = .015). Mean sustained VT/VF event burden was higher in the secondary compared with the primary prevention ICD patients (0.47 vs 0.11 events per patient-year; P = .005) but did not differ significantly between LVEF ≤35% and 36%-49% patients., Conclusion: CS patients with ICD indications and LVEF 36%-49% carry similarly high arrhythmic risk as those with LVEF ≤35%. Patients with secondary prevention ICDs have the highest overall risk., Competing Interests: Disclosures The authors have no conflicts to disclose., (Copyright © 2024 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.)

Competing Interests: Disclosures Drs Friedman, Attia, Kapa, and Siontis are coinventors of the artificial intelligence–enhanced electrocardiogram (AI-ECG) algorithms other than the one presented herein. Mayo Clinic has licensed such algorithms to Anumana Inc., with potential for commercialization. The AI-ECG algorithm for cardiac sarcoidosis detection has not been licensed to a commercial entity.

Background: Myocardial inflammation contributes to the pathogenesis of arrhythmogenic cardiomyopathy (ACM), a clinically and genetically heterogenous disorder. Due to phenotypic overlap, some patients with genetic ACM may be evaluated for an underlying inflammatory cardiomyopathy. However, the cardiac fludeoxyglucose (FDG) positron emission tomography (PET) findings in ACM patients have not been elucidated., Methods: All genotype-positive patients in the Mayo Clinic ACM registry (n = 323) who received a cardiac FDG PET were included in this study. Pertinent data were extracted from the medical record., Results: Collectively, 12/323 (4%; 67% female) genotype-positive ACM patients received a cardiac PET FDG scan as part of their clinical evaluation (median age at scan 49 ± 13 years). Amongst these patients, pathogenic/likely pathogenic variants were detected in LMNA (n = 7), DSP (n = 3), FLNC (n = 1) and PLN (n = 1). Of note, 6/12 (50%) had abnormal myocardial FDG uptake, including diffuse (entire myocardium) uptake in 2/6 (33%), focal (1-2 segments) uptake in 2/6 (33%) and patchy (3+ segments) in 2/6 (33%). Median myocardial standardized uptake value ratio was 2.1. Interestingly, LMNA-positive patients accounted for 3 out of 6 (50%) positive studies (diffuse uptake in 2 and focal uptake in 1)., Conclusion: Abnormal myocardial FDG uptake is common in genetic ACM patients undergoing cardiac FDG PET. This study further supports the role of myocardial inflammation in ACM. Further investigation is needed to determine role of FDG PET in diagnosis and management of ACM and investigate the role of inflammation in ACM., Competing Interests: Conflict of interest Dr. Ackerman is a consultant for Abbott, Boston Scientific, Bristol Myers Squibb, Daichii Sankyo, Invitae, Medtronic, Tenaya Therapeutics, and UpToDate. MJA and Mayo Clinic have license agreements with AliveCor, Anumana, ARMGO Pharma, Pfizer, and Thryv Therapeutics. However, none of these entities were involved in this study. Other authors declare no conflicts., (Copyright © 2023 Elsevier B.V. All rights reserved.)

Background: Histologic evidence is required for a definitive diagnosis of cardiac sarcoidosis (CS) by published guidelines; however, the sporadic nature of the disease may produce false negative biopsy results, causing CS to be underdiagnosed. We sought to establish a clinical category of CS absent histologic findings., Methods: Patients evaluated for CS were stratified into 3 groups: probable CS and definite CS based on Heart Rhythm Society (HRS) criteria and presumed CS, ie, patients without any histologic evidence of sarcoidosis, but with unexplained high-grade atrioventricular block or ventricular arrhythmia and findings suggestive of CS on either cardiac magnetic resonance imaging or positron emission tomography. The primary end point was hospitalization-free and overall survival at 10 years., Results: A total of 383 patients were included in the study: 59, definite CS; 223, probable CS; and 101, presumed CS (62, isolated CS and 39, systemic CS). Compared with patients meeting HRS criteria for CS, patients with presumed CS had lower odds of New York Heart Association class III or IV symptoms (odds ratio [OR], 0.44 [95% CI, 0.23-0.83]; P = .01) but greater odds of previous ventricular tachycardia (OR, 2.4 [95% CI, 1.4-4.0]; P = .001) or history of resuscitated sudden cardiac arrest (OR, 2.9 [95% CI, 1.0-8.6]; P = .05). Hospitalization-free and overall survival were similar among groups (P = .51 and P = .71, respectively)., Conclusions: Clinical categorization of patients with presumed CS identified a high-risk cohort comparable to patients with histologic evidence of disease, although caution should be exercised in reaching this diagnosis without paying due diligence to the differential diagnosis., (Copyright © 2021 Elsevier B.V. All rights reserved.)

Background and Aims: Perivascular epicardial adipose tissue (pEAT) plays a key role in the progression of atherosclerosis, plaque rupture, and thrombosis. However, the relationship between pEAT and prognosis after revascularization of ST-segment elevation myocardial infarction (STEMI) is unknown. This study aimed to investigate the relationship between pEAT thickness and prognosis after STEMI., Methods: We studied 180 STEMI patients (mean age 59.4 ± 13.3 years, 78.9% male) who underwent cardiac magnetic resonance (CMR) imaging within 1 week of prompt infarct-related artery revascularization and 52 age/sex/body mass index-matched controls (mean age 59.9 ± 13.5 years, 78.9% male). pEAT thickness indexed to body surface area at five locations, infarct size, left ventricular ejection fraction (LVEF), and coronary microvascular obstruction (MVO) were evaluated by CMR. Associations between pEAT index and 1-year composite major adverse cardiovascular events (MACE), infarct size, LVEF, and MVO were analyzed., Results: Mean pEAT indices were significantly higher in STEMI patients than controls. In STEMI patients, higher pEAT indices at the superior and inferior interventricular groove (SIVG and IIVG, respectively) were significantly associated with larger infarct size, higher prevalence of MVO, and inversely correlated with post-infarct LVEF. SIVG pEAT index was an independent predictor of composite MACE in post-STEMI patients with an odds ratio of 2.26 (95% confidence interval 1.63-3.13; p < 0.0001) after adjustment for age, sex, LVEF, and 2.71 (95% confidence interval 1.93-3.80; p < 0.0001) after adjustment for age, sex, previous myocardial infarction, diabetes mellitus, and renal function., Conclusions: STEMI patients have significantly higher pEAT indices than controls. SIVG pEAT index independently predicts composite MACE in revascularized STEMI patients, underscoring the potentially prognostic value of this variable., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2020. Published by Elsevier B.V.)

Radionuclide imaging provides both established and emerging diagnostic and prognostic tools to assist clinicians in the management of patients with ischemic cardiomyopathy, cardiac sarcoidosis, and cardiac amyloidosis. This review highlights the underlying pathophysiology of each entity and associated diagnostic and clinical challenges, and describes the available radionuclide imaging techniques. Specific protocols, advantages and disadvantages, comparison with other noninvasive imaging modalities, and discussion of the evolving role of hybrid imaging are also included., (Copyright © 2016 Elsevier Inc. All rights reserved.)