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Introduction: While cardiac sarcoidosis (CS) carries a risk of ventricular arrhythmias (VAs) and sudden cardiac death (SCD), risk stratification of patients with CS and preserved left ventricular/right ventricular (LV/RV) systolic function remains challenging. We sought to evaluate the role of electrophysiologic testing and programmed electrical stimulation of the ventricle (EPS) in patients with suspected CS with preserved ventricular function., Methods: One hundred twenty consecutive patients with biopsy-proven extracardiac sarcoidosis and preserved LV/RV systolic function underwent EPS. All patients had either probable CS defined by an abnormal cardiac positron emission tomography or cardiac magnetic resonance imaging, or possible CS with normal advanced imaging but abnormal echocardiogram (ECG), SAECG, Holter, or clinical factors. Patients were followed for 4.5 ± 2.6 years for SCD and VAs., Results: Seven of 120 patients (6%) had inducible ventricular tachycardia (VT) with EPS and received an implantable cardioverter defibrillator (ICD). Three patients (43%) with positive EPS later had ICD therapies for VAs. Kaplan-Meier analysis stratified by EPS demonstrated a significant difference in freedom from VAs and SCD (P = 0.009), though this finding was driven entirely by patients within the cohort with probable CS (P = 0.018, n = 69). One patient with possible CS and negative EPS had unrecognized progression of the disease and unexplained death with evidence of CS at autopsy., Conclusions: EPS is useful in the risk stratification of patients with probable CS with preserved LV and RV function. A positive EPS was associated with VAs. While a negative EPS appeared to confer low risk, close follow-up is needed as EPS cannot predict fatal VAs related to new cardiac involvement or disease progression., (© 2019 Wiley Periodicals, Inc.)

Introduction: Multiple ablations are often necessary to manage ventricular arrhythmias (VAs) in nonischemic cardiomyopathy (NICM) patients. We assessed characteristics and outcomes and role of adjunctive, nonstandard ablation in repeat VA ablation (RAbl) in NICM., Methods and Results: Consecutive NICM patients undergoing RAbl were analyzed, with characteristics of the last VA ablations compared between those undergoing 1 versus multiple-repeat ablations (1-RAbl vs. >1RAbl), and between those with or without midmyocardial substrate (MMS). VA-free survival was compared. Eighty-eight patients underwent 124 RAbl, 26 with > 1RAbl, and 26 with MMS. 1-RAbl and > 1-RAbl groups were similar in age (57 ± 16 vs. 57 ± 17 years; P = 0.92), males (76% vs. 69%; P = 0.60), LVEF (40 ± 17% vs. 40 ± 18%; P = 0.96), and amiodarone use (31% vs. 46%, P = 0.22). One-year VA freedom between 1-RAbl vs. > 1RAbl was similar (82% vs. 80%; P = 0.81); adjunctive ablation was utilized more in >1RAbl (31% vs. 11%, P = 0.02), and complication rates were higher (27% vs. 7%, P = 0.01), most due to septal substrate and anticipated heart block. >1-RAbl patients had more MMS (62% vs. 16%, P < 0.01). Although MMS was associated with worse VA-free survival after 1-RAbl (43% vs. 69%, P = 0.01), when >1RAbl was performed, more often with nonstandard ablation, VA-free survival was comparable to non-MMS patients (85% vs. 81%; P = 0.69). More RAbls were required in MMS versus non-MMS patients (2.00 ± 0.98 vs. 1.16 ± 0.37; P < 0.001)., Conclusion: For NICM patients with recurrent, refractory VAs despite previous ablation, effective arrhythmia control can safely be achieved with subsequent ablation, although >1 repeat procedure with adjunctive ablation is often required, especially with MMS., (© 2018 Wiley Periodicals, Inc.)

Some patients diagnosed with arrhythmogenic right ventricular cardiomyopathy (ARVC) are eventually found to have cardiac sarcoidosis (CS). Accurate differentiation between these 2 conditions has implications for immunosuppressive therapy and familial screening. We sought to determine whether cardiac magnetic resonance imaging (MRI) could be used to identify the characteristic findings to accurately differentiate between CS and ARVC. Consecutive patients with a diagnostic MRI scan indicating CS and/or ARVC constituted the cohort. All patients diagnosed with CS had histologic confirmation of sarcoidosis, and all patients with ARVC met the diagnostic task force criteria. The cardiac MRI data were retrospectively analyzed to identify possible differentiating characteristics. Of the patients, 40 had CS and 21 had ARVC. Those with CS were older and had more left ventricular scar. The presence of mediastinal lymphadenopathy or left ventricular septal involvement was seen exclusively in the patients with CS (p <0.001). A family history of sudden cardiac death was seen only in the ARVC group (p = 0.012). The right ventricular ejection fraction and ventricular volumes were also significantly different between the 2 groups. In conclusion, patients with CS have significantly different cardiac MRI characteristics than patients with ARVC. The cardiac volume, in addition to the degree and location of cardiac involvement, can be used to distinguish between these 2 disease entities. The presence of mediastinal lymphadenopathy and left ventricular septal scar favors a diagnosis of CS and not ARVC. Consideration of CS should be given if these MRI findings are observed during the evaluation for possible ARVC., (Copyright © 2012 Elsevier Inc. All rights reserved.)

Introduction: Sarcoidosis is a multisystem granulomatous disease that can affect the heart. Early identification of cardiac sarcoidosis (CS) is critical because sudden death can be the initial presentation. We sought to evaluate the potential role of the ECG for identification of cardiac involvement in a cohort of patients with biopsy-proven pulmonary sarcoidosis., Methods: Our cohort consisted of referred patients with biopsy-proven pulmonary sarcoidosis who demonstrated symptoms consistent with cardiac involvement. The ECG characteristics collected were PR, QRS duration, QT interval, rate, bundle branch block (BBB), fragmented QRS (fQRS). QRS fragmentation was defined as 2 anatomically contiguous leads demonstrating RSR' patterns in the absence of BBB., Results: There were 112 subjects included in the cohort. Of the 52 subjects eventually diagnosed with CS, 39 had an ECG demonstrating fQRS while 21 of the 60 of non-CS patients had fQRS (75% vs 33.9%, P < 0.01). A RBBB or LBBB pattern were both more prevalent in the CS population (RBBB: 23.1% vs 6.7%, P = 0.016; LBBB: 3.8% vs 1.7%, P = 0.6). QRS duration remained significantly associated with CS after exclusion of those with BBB (93.5 +/- 10.6 vs 88 +/- 11 ms; P = 0.04). When fQRS and bundle branch block were combined, 90.4% of CS patient's ECGs contained at least one of the features, compared to 36.7% of noncardiac CS (P < 0.01)., Conclusions: The presence of fQRS or BBB pattern in patients with pulmonary sarcoidosis is associated with cardiac involvement and therefore should prompt further evaluation., (© 2011 Wiley Periodicals, Inc.)

Introduction: Cardiac sarcoidosis (CS) occurs in up to 25% of patients with pulmonary involvement. Early diagnosis is critical because sudden death from ventricular arrhythmias can be the initial presentation. We sought to evaluate the diagnostic utility of signal-averaged ECG (SAECG) for detection of cardiac involvement of sarcoidosis., Methods: Subjects with biopsy proven sarcoidosis and symptoms suggestive of possible cardiac involvement were included in the cohort. Standard criteria for SAECG were used. Subjects were considered to have CS if they met criteria established by the Japanese Ministry of Health and Welfare modified to include cardiac MRI., Results: Of the 88 patients in the cohort 27 had evidence of CS independent of the SAECG results. The SAECG was abnormal in 14 of these 27 patients and 11 of the 61 of the subjects without cardiac involvement (P < 0.01). The sensitivity of SAECG detection of CS was 52% with a specificity of 82%. For the entire cohort, SAECG had a positive predictive value (PPV) of 0.56 and a negative predictive value (NPV) of 0.79. Within a subgroup of 67 patients with an unfiltered QRS duration of <100 ms, the specificity for diagnosing cardiac sarcoidosis improves to 100% with a reduced sensitivity of 36.8. Of the SAECG parameters, LAS40 was significantly associated with the diagnosis of cardiac sarcoidosis for the entire cohort (P < 0.01) and among the subgroup of patients with an unfiltered QRS duration of <100 ms (P < 0.01)., Conclusions: SAECG is a useful screening tool in the evaluation of sarcoidosis for detection of cardiac involvement., (©2011, Wiley Periodicals, Inc.)

ICD Shocks in Cardiac Sarcoidosis. Background: An implantable cardioverter defibrillator (ICD) is indicated for some patients with cardiac sarcoidosis (CS) for prevention of sudden death. However, there are little data regarding the event rates of ICD therapies in these patients. We sought to identify the incidence and characteristics of ICD therapies in this patient population. Methods: We performed a cohort study of patients with ICDs at 3 institutions. Cases were those patients with CS and an ICD implanted for primary or secondary prevention of sudden death. Additionally, we included a comparison with historical controls of ICD therapy rates reported in clinical trials evaluating the ICD for primary and secondary prevention of sudden death. Results: Of the 112 CS subjects identified, 36 (32.1%) received appropriate therapies for ventricular tachyarrhythmias (VT) over a mean follow-up period of 29.2 months. VT storm (>3 episodes in 24 hours) occurred in 16 (14.2%) CS subjects. Inappropriate therapies occurred in 13 CS subjects (11.6%). Covariates associated with appropriate ICD therapies included left ventricular ejection fraction (LVEF) <55% (OR 6.52 [95% CI 2.43-17.5]), right ventricular dysfunction (OR 6.73 [95% CI 2.69-16.8]), and symptomatic heart failure (OR 4.33 [95% CI 1.86-10.1]). Conclusions: In our cohort of patients with CS and ICDs, almost one-third receive appropriate therapies. This may be due to a myocardial inflammatory process leading to increased triggered activity and subsequent scarring leading to reentrant tachyarrhythmias. Adjusted predictors of ICD therapies in this population include left or right ventricular dysfunction. (J Cardiovasc Electrophysiol, Vol. 23, pp. 925-929, September 2012) [ABSTRACT FROM AUTHOR]

Background: Ventricular tachycardia (VT) ablation is often avoided in patients with advanced heart failure (HF) and this group of patients is often excluded from research trials. Objective: To evaluate VT recurrence rates among patients with severe HF and VT treated with ablation (Abl) versus medications (Med) alone. Methods: We performed a retrospective analysis of patients presenting to a tertiary referral hospital between 2006 and 2017 with ventricular tachycardia and severe heart failure (New York Heart Association functional Class IV or any Interagency Registry for Mechanically Assisted Circulatory Support score) for whom electrophysiology consult was requested. Baseline characteristics and VT recurrence were compared based on method of arrhythmia treatment. Results: Of 129 patients, 47% underwent ablation while 53% were managed medically. In this cohort, mean age was 60 ± 12 years, 84% were males, ischemic cardiomyopathy was present in 55%, mean LVEF was 24±12%, and 77% had a preexisting ICD.One-year mortality was 23%, 40% of whom were treated with Abl versus 60% with Med, p=0.196. Abl patients were more likely to have an ICD prior to presentation (85% vs 69%, p=0.037) and had shorter length of hospital stay (14±16 vs 21±16 days, p=0.021) compared to Med patients. Other baseline characteristics were not significantly different between groups. Abl patients overall had better VT-free survival compared to those treated with medications alone (OR 0.36, 95% CI [0.18, 0.80], Figure). Conclusions: Ablation of ventricular arrhythmia even in patients with severe heart failure is associated with decreased length of hospitalization and improved VT-free survival compared to medical therapy alone. These findings warrant additional, prospective study to verify and possibly improve management of this high-risk group of patients. [ABSTRACT FROM AUTHOR]

Background: Ventricular tachycardia (VT) is common in patients with severe heart failure (HF). Objective: We sought to determine whether early recurrence of VT is associated with worse survival. Methods: We performed retrospective analysis of patients presenting to a tertiary referral hospital between 2006-2017 with VT and severe HF (defined as New York Heart Association functional Class IV or any Interagency Registry for Mechanically Assisted Circulatory Support score) for whom electrophysiology consultation was requested. A comparison was made in baseline characteristics and outcomes between those with early VT recurrence (within 30 days of initial consult) and those without early VT recurrence. Results: Of 129 patients, 47% underwent ablation while 53% were managed medically. Mean age was 60±12 years, 84% were males, ischemic cardiomyopathy was present in 55%, mean LVEF was 24±12%, and 77% had a preexisting ICD. Early recurrence of VT was observed in 25 patients (19%). Those with early recurrence had lower mean LVEF (20±7% vs 25±12%, p =0.032), were less likely to have an ICD prior to presentation (56% vs 82%, p =0.015), were more likely to have been treated with only medications (80% vs 20%, p =0.003), had greater in-hospital mortality (28% vs 5%, p =0.002) and experienced a longer hospitalization (23±19 vs 17±16 days), compared to those without early recurrence. There was no significant difference in other baseline characteristics between groups. Patients with early recurrence had significantly worse 1-year survival 56% vs 82% (Figure), with OR 3.5, 95% CI [1.3,8.9]. Conclusion: Early VT recurrence among patients with severe heart failure is associated with increased mortality within the year following presentation. VT ablation should be considered to help mitigate risk of early recurrence and associated morbidity in such patients. [ABSTRACT FROM AUTHOR]