IMPORTANCE Ventricular tachycardia (VT) is associated with high mortality in patients with cardiac sarcoidosis (CS), and medical management of CS-associated VT is limited by high failure rates. The... Show moreIMPORTANCE Ventricular tachycardia (VT) is associated with high mortality in patients with cardiac sarcoidosis (CS), and medical management of CS-associated VT is limited by high failure rates. The role of catheter ablation has been investigated in small, single-center studies.OBJECTIVE To investigate outcomes associated with VT ablation in patients with CS.DESIGN, SETTING, AND PARTICIPANTS This cohort study from the Cardiac Sarcoidosis Consortium registry (2003-2019) included 16 tertiary referral centers in the US, Europe, and Asia. A total of 158 consecutive patients with CS and VT were included (33% female; mean [SD] age, 52 [11] years; 53% with ejection fraction [EF] <50%).EXPOSURES Catheter ablation of CS-associated VT and, as appropriate, medical treatment.MAIN OUTCOMES AND MEASURES Immediate and short-term outcomes included procedural success, elimination of VT storm, and reduction in defibrillator shocks. The primary long-term outcome was the composite of VT recurrence, heart transplant (HT), or death.RESULTS Complete procedural success (no inducible VT postablation) was achieved in 85 patients (54%). Sixty-five patients (41%) had preablation VT storm that did not recur postablation in 53 (82%). Defibrillator shocks were significantly reduced from a median (IQR) of 2 (1-5) to 0 (0-0) in the 30 days before and after ablation (P < .001). During median (IQR) follow-up of 2.5 (1.1-4.9) years, 73 patients (46%) experienced VT recurrence and 81 (51%) experienced the composite primary outcome. One- and 2-year rates of survival free of VT recurrence, HT, or death were 60% and 52%, respectively. EF less than 50% and myocardial inflammation on preprocedural F-18-fluorodeoxyglucose positron emission tomography were significantly associated with adverse prognosis in multivariable analysis for the primary outcome (HR, 2.24; 95% CI, 1.37-3.64; P = .001 and HR, 2.93; 95% CI, 1.31-6.55; P = .009, respectively). History of hypertension was associated with a favorable long-term outcome (adjusted HR, 0.51; 95% CI, 0.28-0.92; P = .02).CONCLUSIONS AND RELEVANCE In this observational study of selected patients with CS and VT, catheter ablation was associated with reductions in defibrillator shocks and recurrent VT storm. Preablation LV dysfunction and myocardial inflammation were associated with adverse long-term prognosis. These data support the role of catheter ablation in conjunction with medical therapy in the management of CS-associated VT. Show less
Hoogendoorn, J.C.; Venlet, J.; Out, Y.N.J.; Man, S.; Kumar, S.; Sramko, M.; ... ; Zeppenfeld, K. 2021
BACKGROUND Cardiac sarcoidosis (CS) with right ventricular (RV) involvement can mimic arrhythmogenic right ventricular cardiomyopathy (ARVC). Histopathological differences may result in disease... Show moreBACKGROUND Cardiac sarcoidosis (CS) with right ventricular (RV) involvement can mimic arrhythmogenic right ventricular cardiomyopathy (ARVC). Histopathological differences may result in disease-specific RV activation patterns detectable on the 12-lead electrocardiogram. Dominant subepicardial scar in ARVC leads to delayed activation of areas with reduced voltages, translating into terminal activation delay and occasionally (epsilon) waves with a small amplitude. Conversely, patchy transmural RV scar in CS may lead to conduction block and therefore late activated areas with preserved voltages reflected as preserved R' waves.OBJECTIVE The purpose of this study was to evaluate the distinct terminal activation patterns in precordial leads V-1 through V-3 as a discriminator between CS and ARVC.METHODS Thirteen patients with CS affecting the RV and 23 patients with gene-positive ARVC referred for ventricular tachycardia ablation were retrospectively included in a multicenter approach. A non-ventricular-paced 12-lead surface electrocardiogram was analyzed for the presence and the surface area of the R' wave (any positive deflection from baseline after an S wave) in leads V-1 through V-3.RESULTS An R' wave in leads V-1 through V-3 was present in all patients with CS compared to 11 (48%) patients with ARVC (P=.002). An algorithm including a PR interval of >= 220 ms, the presence of an R' wave, and the surface area of the maximum R' wave in leads V-1 through V-3 of similar to 1.65 mm(2) had 85% sensitivity and 96% specificity for diagnosing CS, validated in a second cohort (18 CS and 40 ARVC) with 83% sensitivity and 88% specificity.CONCLUSION An easily applicable algorithm including PR prolongation and the surface area of the maximum R' wave in leads V-1 through V-3 of similar to 1.65 mm(2) distinguishes CS from ARVC. This QRS terminal activation in precordial leads V-1 through V-3 may reflect disease-specific scar patterns. Show less
Siontis, K.C.; Kim, H.M.; Vergara, P.; Peretto, G.; D.H. do; Riva, M. de; ... ; Bogun, F.M. 2020
Aims Catheter ablation is an effective treatment for post-infarction ventricular tachycardia (VT). However, some patients may experience a worsened arrhythmia phenotype after ablation. We aimed to... Show moreAims Catheter ablation is an effective treatment for post-infarction ventricular tachycardia (VT). However, some patients may experience a worsened arrhythmia phenotype after ablation. We aimed to determine the prevalence and prognostic impact of arrhythmia exacerbation (AE) after post-infarction VT ablation.Methods and results A total of 1187 consecutive patients (93% men, median age 68 years, median ejection fraction 30%) who underwent post-infarction VT ablation at six centres were included. Arrhythmia exacerbation was defined as post ablation VT storm or incessant VT in patients without prior similar events. During follow-up (median 717 days), 426 (36%) patients experienced VT recurrence. Events qualifying as AE occurred in 67 patients (6%). Median times to VT recurrence with and without AE were 238 [interquartile range (IQR) 35-640] days and 135 (IQR 22-521) days, respectively (P= 0.25). Almost half of the patients (46%) who experienced AE experienced it within 6 months of the index procedure. Patients with AE had had longer ablation times during the ablation procedures compared to the rest of the patients (median 42 vs. 34 min, P = 0.02). Among patients with VT recurrence, the risk of death or heart transplantation was significantly higher in patients with than without AE (hazard ratio 1.99, 95% CI 1.28-3.10; P= 0.002) after adjusting for age, gender, ejection fraction, cardiac resynchronization therapy, post-ablation non-inducibility, and post-ablation amiodarone use.Conclusion Arrhythmia exacerbation after ablation of infarct-related VT is infrequent but is independently associated with an adverse long-term outcome among patients who experience a VT recurrence. The mechanisms and mitigation strategies of AE after catheter ablation require further investigation. Show less
Hoogendoorn, J.C.; Sramko, M.; Venlet, J.; Siontis, K.C.; Kumar, S.; Singh, R.; ... ; Zeppenfeld, K. 2020
OBJECTIVES This study sought to investigate the value of electroanatomical voltage mapping (EAVM) to distinguish cardiac sarcoidosis (CS) from arrhythmogenic right ventricular cardiomyopathy (ARVC)... Show moreOBJECTIVES This study sought to investigate the value of electroanatomical voltage mapping (EAVM) to distinguish cardiac sarcoidosis (CS) from arrhythmogenic right ventricular cardiomyopathy (ARVC) in patients with ventriculartachycardia from the right ventricle (RV).BACKGROUND CS can mimic ARVC. Because scar in ARVC is predominantly subepicardial, this study hypothesized that the relative sizes of endocardial low bipolar voltage (BV) to low unipolar voltage (UV) areas may distinguish CS from ARVC.METHODS Patients with CS affecting the RV (n = 14), patients with gene-positive ARVC (n = 13), and a reference group of patients without structural heart disease (n = 9) who underwent RV endocardial EAVM were included. RV regionspecific BV and UV cutoffs were derived from control subjects. In CS and ARVC, segmental involvement was determined and low-voltage areas were measured, using <1.5 mV for BV and <3.9 mV, <4.4 mV, and <5.5 mV for UV. The ratio between low BV and low UV area was calculated generating 3 parameters: Ratio3.9, Ratio4.4 and Ratio5.5, respectively.RESULTS In control subjects, BV and UV varied significantly among RV regions. The basal septum was involved in 71% of CS patients and in none of ARVC patients. Ratio5.5 discriminated CS from ARVC the best. An algorithm including Ratio5.5≥0.45 and basal septal involvement identified CS with 93% sensitivity and 85% specificity. This was validated in a separate population (CS [n = 6], ARVC [n = 10]) with 100% sensitivity and 100% specificity.CONCLUSIONS EAVM provides detailed information about scar characteristics and scar distribution in the RV. An algorithm combining Ratio5.5 (area BV <1.5 mV/area UV <5.5 mV) and bipolar basal septal involvement allows accurate diagnosis of (isolated) CS in patients presenting with monomorphic ventricular tachycardia from the RV. Show less
Siontis, K.C.; Kim, H.M.; Stevenson, W.G.; Fujii, A.; Bella, P. della; Vergara, P.; ... ; Bogun, F.M. 2016
