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AimsDesmoplakin (DSP) cardiomyopathy is an increasingly recognized form of arrhythmogenic cardiomyopathy. With a genotype-specific approach, we characterized the diagnosis, natural history, and risk for ventricular arrhythmia and heart failure in DSP cardiomyopathy.Methods and resultsWe followed 91 individuals [45 probands, 34% male, median age 27.5 years (interquartile interval 20.0–43.9)] with pathogenic or likely pathogenic DSP variants for a median of 4.3 years. Regarding the ventricular involvement, left predominance was most common (n = 22, 28%) followed by bi-ventricular in 12 (15%) and right predominance in 5 (6%). Myocardial injury (chest pain, elevated troponin, normal coronary angiogram) occurred in 20 (22%) individuals. Incidence rates of sustained ventricular arrhythmia and heart failure (ventricular dysfunction ± symptoms) were 5.9 [95% confidence interval (CI): 3.9–9.1] and 6.7 (95% CI: 4.5–9.8) per 100 person-years, respectively. In univariate regression, myocardial injury was associated with sustained ventricular arrhythmia [hazard ratio (HR) 2.53, 95% CI: 1.05–6.11] and heart failure (HR 7.53, 95% CI: 3.10–18.26). After adjustment, left ventricular ejection fraction ConclusionDSP cardiomyopathy affects both ventricles and carries high risk for ventricular arrhythmia and heart failure. Myocardial injury is associated with worse disease outcomes. Both diagnosis and risk stratification of DSP cardiomyopathy need refinement.

Objectives This study sought to explore the long-term arrhythmic outcomes of bilateral cardiac sympathetic denervation (BCSD). Background BCSD has been associated with improved arrhythmic outcomes in patients with refractory ventricular arrhythmias. However, whether BCSD antiarrhythmic effects are sustained long after the procedure is still uncertain. Methods We included consecutive patients who underwent BCSD because of refractory ventricular tachycardia (VT) and had at least 18 months of follow-up. VT recurrence after BCSD was evaluated to assess arrhythmic outcomes. The occurrence of VT episodes within the first 12 weeks after the procedure was assessed to explore the impact of early VT recurrence on late arrhythmia-free survival. Results Twenty patients (42 ± 16 years; 55% male) were included in the analysis. Nineteen (95%) patients had structural heart disease (left ventricular ejection fraction: 0.46 ± 0.14). Class I or class III drugs failed for all patients, and the mean number of VT ablation procedures was 2.5 ± 1.6. Over a mean follow-up of 1,300 ± 321 days (median: 1,276 days [Interquartile range (IQR): 1,181 to 1,480 days), 11 (55%) patients remained VT free after sympathectomy. Freedom from sustained VT or implantable cardioverter-defibrillator shock was 60% (95% confidence interval: 0.35 to 0.77) and 54.5% (95% confidence interval: 0.31 to 0.73) after BCSD at 1 and 4 years. Early VT recurrence was not associated with worse late arrhythmia-free survival rates. Conclusions BCSD was associated with longstanding antiarrhythmic effects in patients with refractory ventricular arrhythmias. The occurrence of VT episodes early after the procedure was not associated with worse late arrhythmic outcomes.

Patients with arrhythmogenic right ventricular cardiomyopathy (ARVC) classically initially present with ventricular arrhythmias or, less commonly, heart failure. Myocardial inflammation has been implicated in pathogenesis, but clinical myocarditis in ARVC is less described. We therefore studied clinical myocarditis as an initial ARVC presentation, and hypothesized that these patients have distinct clinical and genetic characteristics. Using the Johns Hopkins ARVC Registry, we identified 12 patients (all female, median age 20) referred between 2014 and 2019 diagnosed with myocarditis at presentation who were subsequently diagnosed with ARVC by Task Force Criteria. Majority presented with chest pain (n = 7, 58%) or ventricular arrhythmia (n = 3, 25%). All patients had troponin elevations and left ventricular (LV) function was reduced in 5 (42%). Magnetic resonance imaging demonstrated LV delayed gadolinium enhancement and/or pericardial enhancement in 10 (83%); only 3 (25%) patients had right ventricular abnormalities. Pathogenic genetic variants were identified in 11 (92%) patients: 10 desmoplakin (DSP) and 1 desmoglein-2 (DSG2). Thus, nearly 1/3 (10/32, 31%) of overall DSP ARVC patients were originally diagnosed with myocarditis. Patients were diagnosed with ARVC 1.8 years (IQR 2.7 years) after presentation and 8 (75%) patients did not meet Task Force Criteria without genetic testing. ARVC diagnosis led to an additional 5 (42%) patients referred for implantable cardiac defibrillator and 17 family member diagnoses. In conclusion, ARVC may initially present as myocarditis and these patients have distinct characteristics including female gender, LV involvement and DSP gene variants. Genetic testing is key to ARVC diagnosis and should be considered in select myocarditis patients.

Introduction We recently developed two noninvasive methodologies to help guide VT ablation: population-derived automated VT exit localization (PAVEL) and virtual-heart arrhythmia ablation targeting (VAAT). We hypothesized that while very different in their nature, limitations, and type of ablation targets (substrate-based vs. clinical VT), the image-based VAAT and the ECG-based PAVEL technologies would be spatially concordant in their predictions. Objective The objective is to test this hypothesis in ischemic cardiomyopathy patients in a retrospective feasibility study. Methods Four post-infarct patients who underwent LV VT ablation and had pre-procedural LGE-CMRs were enrolled. Virtual hearts with patient-specific scar and border zone identified potential VTs and ablation targets. Patient-specific PAVEL based on a population-derived statistical method localized VT exit sites onto a patient-specific 238-triangle LV endocardial surface. Results Ten induced VTs were analyzed and 9-exit sites were localized by PAVEL onto the patient-specific LV endocardial surface. All nine predicted VT exit sites were in the scar border zone defined by voltage mapping and spatially correlated with successful clinical lesions. There were 2.3 ± 1.9 VTs per patient in the models. All five VAAT lesions fell within regions ablated clinically. VAAT targets correlated well with 6 PAVEL-predicted VT exit sites. The distance between the center of the predicted VT-exit-site triangle and nearest corresponding VAAT ablation lesion was 10.7 ± 7.3 mm. Conclusions VAAT targets are concordant with the patient-specific PAVEL-predicted VT exit sites. These findings support investigation into combining these two complementary technologies as a noninvasive, clinical tool for targeting clinically induced VTs and regions likely to harbor potential VTs.

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited heart muscle disease characterized by progressive fibrofatty replacement of the myocardium, right ventricular enlargement, and malignant ventricular arrhythmias. Ventricular tachycardia (VT) may be seen in all stages of the disease and is associated with sudden cardiac death. In patients who failed anti-arrhythmic medical therapy, catheter ablation has become an attractive therapeutic option to reduce VT burden and implantable cardioverter-defibrillator interventions. In this article, the authors aim to address the overall concepts of epicardial catheter ablation in ARVC, focusing on substrate characterization and ablation strategies.

Aims In arrhythmogenic right ventricular cardiomyopathy (ARVC) patients, exercise worsens disease course, so exercise restriction is recommended. However, recommendations for genotype-positive ARVC family members is incompletely resolved. We aimed to provide evidence for exercise recommendations for genotype-positive ARVC family members. Methods and results Arrhythmogenic right ventricular cardiomyopathy family members inheriting a pathogenic desmosomal variant were interviewed about exercise history from age 10. Exercise was characterized by duration, intensity, and dose (duration*intensity). Associations between exercise and (i) diagnosis by 2010 Task Force Criteria and (ii) development of sustained ventricular arrhythmias were examined. The study included 101 family members (age: 40.5 ± 19.3 years, male: 41%, Plakophilin-2 variant: 81%). Forty-four individuals (44%) met diagnostic criteria and 16 (16%) experienced sustained ventricular arrhythmia. Individuals who met diagnostic criteria had significantly higher average exercise duration and dose, but not peak intensity than those who did not. Only one individual who exercised below the American Heart Association recommended minimum (650 metabolic equivalent of task-hours/year) met diagnostic criteria or experienced sustained ventricular arrhythmia as opposed to 50% of individuals who exceeded it (adjusted odds ratio = 0.03, 95% confidence interval 0.003–0.26). The difference in exercise exposure between affected and unaffected individuals was more striking in females than in males. Females who had done high-dose exercise in adolescence had the worst survival free from diagnosis (P Conclusions In phenotype-negative ARVC family members with a pathogenic desmosomal variant, athletic activities should be limited, particularly exercise dose. Exercise may play a greater role in promoting disease in female family members.

Aims Arrhythmogenic right ventricular cardiomyopathy (ARVC) is diagnosed by a complex set of clinical tests as per 2010 Task Force Criteria (TFC). Avoiding misdiagnosis is crucial to prevent sudden cardiac death as well as unnecessary implantable cardioverter-defibrillator implantations. This study aims to validate the overall performance of the TFC in a real-world cohort of patients referred for ARVC evaluation. Methods and results We included patients consecutively referred to our centres for ARVC evaluation. Patients were diagnosed by consensus of three independent clinical experts. Using this as a reference standard, diagnostic performance was measured for each individual criterion as well as the overall TFC classification. Of 407 evaluated patients (age 38 ± 17 years, 51% male), the expert panel diagnosed 66 (16%) with ARVC. The clinically observed TFC was false negative in 7/66 (11%) patients and false positive in 10/69 (14%) patients. Idiopathic outflow tract ventricular tachycardia was the most common alternative diagnosis. While the TFC performed well overall (sensitivity and specificity 92%), signal-averaged electrocardiogram (SAECG, P = 0.43), and several family history criteria (P ≥ 0.17) failed to discriminate. Eliminating these criteria reduced false positives without increasing false negatives (net reclassification improvement 4.3%, P = 0.019). Furthermore, all ARVC patients met at least one electrocardiogram (ECG) or arrhythmia criterion (sensitivity 100%). Conclusion The TFC perform well but are complex and can lead to misdiagnosis. Simplification by eliminating SAECG and several family history criteria improves diagnostic accuracy. Arrhythmogenic right ventricular cardiomyopathy can be ruled out using ECG and arrhythmia criteria alone, hence these tests may serve as a first-line screening strategy among at-risk individuals.

Background We have previously developed an intraprocedural automatic arrhythmia‐origin localization (AAOL) system to identify idiopathic ventricular arrhythmia origins in real time using a 3‐lead ECG. The objective was to assess the localization accuracy of ventricular tachycardia (VT) exit and premature ventricular contraction (PVC) origin sites in patients with structural heart disease using the AAOL system. Methods and Results In retrospective and prospective case series studies, a total of 42 patients who underwent VT/PVC ablation in the setting of structural heart disease were recruited at 2 different centers. The AAOL system combines 120‐ms QRS integrals of 3 leads (III, V2, V6) with pace mapping to predict VT exit/PVC origin site and projects that site onto the patient‐specific electroanatomic mapping surface. VT exit/PVC origin sites were clinically identified by activation mapping and/or pace mapping. The localization error of the VT exit/PVC origin site was assessed by the distance between the clinically identified site and the estimated site. In the retrospective study of 19 patients with structural heart disease, the AAOL system achieved a mean localization accuracy of 6.5±2.6 mm for 25 induced VTs. In the prospective study with 23 patients, mean localization accuracy was 5.9±2.6 mm for 26 VT exit and PVC origin sites. There was no difference in mean localization error in epicardial sites compared with endocardial sites using the AAOL system (6.0 versus 5.8 mm, P =0.895). Conclusions The AAOL system achieved accurate localization of VT exit/PVC origin sites in patients with structural heart disease; its performance is superior to current systems, and thus, it promises to have potential clinical utility.

We report a case of a woman with arrhythmogenic right ventricular cardiomyopathy (ARVC) who presented with severe right-sided heart failure and cardiac cirrhosis that mandated heart-liver transplantation. This case highlights an emerging sex-based difference in ARVC where female sex is associated with a higher risk of heart failure than in male patients with ARVC. (Level of Difficulty: Advanced.), Graphical abstract, We report a case of a woman with arrhythmogenic right ventricular cardiomyopathy (ARVC) who presented with severe right-sided heart failure…

Background Cardiac sympathetic denervation (CSD) is an important adjunctive option for patients with refractory ventricular arrhythmias (VAs). Reports of efficacy of CSD in patients with structural heart disease (SHD) and refractory VA vary widely in literature. Objective The purpose of this study was to conduct a systematic review of arrhythmic outcomes and complications in patients with SHD who underwent CSD due to recurrent VAs. Methods Electronic databases (Google Scholar and PubMed) were searched to identify reports on CSD in SHD using appropriate medical subject terms. No sample size restriction was applied. All patients with known channelopathies were excluded. Baseline demographic and surgical data, arrhythmic outcomes, and procedural complications were evaluated. Results A total of 13 studies and 173 patients were included. Of the 173 patients (121 [70%] male); pooled mean age 54.6 [95% confidence interval 52.6–56.7] years), 48 (28%) had ischemic cardiomyopathy, and 141 (82%) underwent bilateral CSD. Overall freedom from events ranged from 58% to 100%. Complications were reported in 49 patients(28%). Transient hypotension (9%), pneumothorax (5%), neuropathic pain (skin sensitivity) (4%), Horner syndrome (3%), sweating pattern changes (3%), and hemothorax (2%) were the most common complications. No procedure-related deaths were reported. Conclusion CSD reduced the number of VA events in patients with SHD, and the benefit from the intervention seemed to be independent of the underlying SHD. Although overall rate of postprocedural complications was high, most of the complications were temporary. Major postprocedural complications after CSD were infrequent.

Introduction Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited progressive cardiomyopathy characterized by frequent life-threatening arrhythmias. The diagnosis of ARVC is challenging and is on the basis of a set of major and minor criteria as described by the modified Task Force Criteria (TFC). We report our clinical experience in a series of patients who were misdiagnosed with ARVC and subsequently underwent removal of their implantable cardioverter defibrillator (ICD) after a re-evaluation at our center. Methods and results We studied 12 patients who were misdiagnosed with ARVC and had ICD implantation before our assessment. All patients had a repeat evaluation and were scored according to TFC before ICD removal. Cardiac magnetic resonance imaging (CMR) studies performed at outside institutions during the initial evaluation were reported abnormal and classified as meeting major TFC in ninety percent of patients. The most common abnormality reported was fatty infiltration of the right ventricular (RV) free wall and/or presence of focal intra-myocardial fat in six patients (50%). On re-evaluation, none of these findings fulfilled the TFC for the diagnosis. Conclusion This study demonstrated that high dependence on misinterpretation of CMR along with a misunderstanding of the TFC evaluation are the main reasons for the misdiagnosis of ARVC. Despite the updated criteria for almost a decade, this study reminds that the diagnosis of ARVC is complex and hence careful TFC evaluation and consideration of multiple cardiac test results should be the focused approach for clinicians when confronted with suspected ARVC patients.

Background The sympathetic nervous system plays an important role in arrhythmogenesis in arrhythmogenic right ventricular cardiomyopathy (ARVC). Sudden cardiac death commonly occurs during exertion, and β-blockers are associated with a reduction in arrhythmia burden. Bilateral cardiac sympathetic denervation (BCSD) has been shown to reduce implantable cardioverter-defibrillator (ICD) shocks in patients with structural heart disease and refractory ventricular tachycardia (VT); however, data in ARVC are sparse. Objective The purpose of this study was to evaluate the role of BCSD in patients with ARVC and refractory VT. Methods Consecutive patients with ARVC who underwent BCSD because of refractory VT were included. Number of ICD shocks, sustained VT episodes, VT storm, and antiarrhythmic therapy were assessed and compared before and after the intervention. VT-free survival rate, death, and heart transplantation were also evaluated. Results Eight patients with ARVC (mean age 32 ± 20 years; 3 men [38%]) underwent sympathectomy for recurrent VT. All patients failed catheter ablation, and 50% had a desmosomal mutation identified. Procedural complications included neuropathic pain, paravertebral venous plexus injury, and pneumothorax. Over a mean follow-up of 1.9 ± 0.9 years, 5 patients (63%) had no VT recurrence. BCSD significantly reduced the number of ICD shocks or sustained VT compared with 1-year pre-BCSD (mean 12.6 ± 18.2 and median 6.5 [interquartile range 4.5–10.5] pre-BCSD vs 0.9 ± 1.4 and 0 [interquartile range 0–1.5] post-BCSD; P = .011). Most of the patients (88%) were on β-blocker therapy alone at the end of follow-up. One patient underwent heart transplantation because of heart failure, and no deaths occurred. Conclusion BCSD may be an effective option for patients with ARVC and refractory ventricular arrhythmia who have failed conventional treatment modalities.

Cardiac sarcoidosis (CS) overlaps in clinical presentation with arrhythmogenic right ventricular cardiomyopathy and shares phenotypic classification, including the presence of epsilon waves. The presence of conduction disease is seen exclusively in CS, as an important phenotypic difference. We present a case of ventricular tachycardia and epsilon waves due to CS, without conduction disease. (Level of Difficulty: Intermediate.), Central Illustration

Previous studies of radiofrequency catheter ablation (RFA) of ventricular tachycardia (VT) in patients with arrhythmogenic right ventricular cardiomyopathy (ARVC), relying on limited numbers of procedures, have not reported VT-free survival in parallel for single and multiple procedures (ie, after the last procedure). Data regarding the impact of RFA on VT burden are scarce.The purpose of this study was to provide new insights on clinical outcomes based on a large series of VT ablation procedures from the current era in ARVC patients.We evaluated consecutive patients with definite ARVC who underwent RFA procedures between 2009 and 2019 at our center. We assessed VT-free survival, for single and multiple procedures, and changes in VT burden and antiarrhythmic drugs (AADs) after RFA.Among 116 patients, there were 166 RFA procedures, 106 (63.9%) of which involved epicardial ablation. Cumulative freedom from VT after a single procedure was 68.6% and 49.8% at 1 and 5 years, respectively. Cumulative VT-free survival after multiple procedures was 81.8% and 69.6% at 1 and 5 years, respectively. VT burden per RFA was reduced after vs before ablation (mean 0.7 vs 10.0 events/year; P.001). Furthermore, VT burden per patient was reduced after last ablation vs before first ablation (mean 0.5 vs 10.9 events/year; P.001). Use of AADs decreased after ablation (22.2% vs 51.9%; P.001).In ARVC patients, RFA provided good VT-free survival after a single procedure, with multiple procedures required for more sustained freedom from VT recurrence. Marked reduction in VT burden permitted discontinuation of AADs.

Background Premature ventricular contractions (PVCs) may be found in any stage of arrhythmogenic right ventricular cardiomyopathy (ARVC) and have been associated with the risk of sustained ventricular tachycardia (VT). Objective To investigate the role of PVC ablation in ARVC patients. Methods We studied consecutive ARVC patients who underwent PVC ablation due to symptomatic high PVC burden. Mean daily PVC burden and AAD use were assessed before and after the procedure. Complete long-term success was defined as > 80% reduction in PVC burden off of membrane-active AADs. Results Eight patients (37±15yo; 4 males) underwent PVC ablation. The mean daily PVC burden before ablation ranged from 5.4% to 24.8%. Seven (87.5%) patients underwent epicardial ablation. Complete acute elimination of PVCs was achieved in 4 (50%) patients (no complications). The mean daily PVC burden variation ranged from an 87% reduction to a 26% increase after the procedure. Over a median follow-up of 345 days (range: 182-3004 days), only one (12.5%) patient presented complete long-term success, and 6 (75%) patients either maintained or increased the need for Class I or Class III AADs. Two (25%) patients experienced sustained VT for the first time following the ablation procedure, requiring repeat ablation. No death or heart transplantation occurred. Conclusion PVC ablation was not associated with a consistent reduction of the PVC burden in ARVC patients with symptomatic, frequent PVCs. PVC ablation may be reserved for highly symptomatic patients who failed AADs. Additional investigation is required to improve the efficacy of PVC ablation in ARVC patients. This article is protected by copyright. All rights reserved.

Supplemental Digital Content is available in the text., Background: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is associated with ventricular arrhythmias (VA) and sudden cardiac death (SCD). A model was recently developed to predict incident sustained VA in patients with ARVC. However, since this outcome may overestimate the risk for SCD, we aimed to specifically predict life-threatening VA (LTVA) as a closer surrogate for SCD. Methods: We assembled a retrospective cohort of definite ARVC cases from 15 centers in North America and Europe. Association of 8 prespecified clinical predictors with LTVA (SCD, aborted SCD, sustained, or implantable cardioverter-defibrillator treated ventricular tachycardia >250 beats per minute) in follow-up was assessed by Cox regression with backward selection. Candidate variables included age, sex, prior sustained VA (≥30s, hemodynamically unstable, or implantable cardioverter-defibrillator treated ventricular tachycardia; or aborted SCD), syncope, 24-hour premature ventricular complexes count, the number of anterior and inferior leads with T-wave inversion, left and right ventricular ejection fraction. The resulting model was internally validated using bootstrapping. Results: A total of 864 patients with definite ARVC (40±16 years; 53% male) were included. Over 5.75 years (interquartile range, 2.77–10.58) of follow-up, 93 (10.8%) patients experienced LTVA including 15 with SCD/aborted SCD (1.7%). Of the 8 prespecified clinical predictors, only 4 (younger age, male sex, premature ventricular complex count, and number of leads with T-wave inversion) were associated with LTVA. Notably, prior sustained VA did not predict subsequent LTVA (P=0.850). A model including only these 4 predictors had an optimism-corrected C-index of 0.74 (95% CI, 0.69–0.80) and calibration slope of 0.95 (95% CI, 0.94–0.98) indicating minimal over-optimism. Conclusions: LTVA events in patients with ARVC can be predicted by a novel simple prediction model using only 4 clinical predictors. Prior sustained VA and the extent of functional heart disease are not associated with subsequent LTVA events.

Background: Infiltrating adipose tissue (inFAT) is a newly recognized proarrhythmic substrate for postinfarct ventricular tachycardias (VT) identifiable on contrast-enhanced computed tomography. This study presents novel digital-heart technology that incorporates inFAT from contrast-enhanced computed tomography to noninvasively predict VT ablation targets and assesses the capability of the technology by comparing its predictions with VT ablation procedure data from patients with ischemic cardiomyopathy. Methods: Digital-heart models reflecting patient-specific inFAT distributions were reconstructed from contrast-enhanced computed tomography. The digital-heart identification of fat-based ablation targeting (DIFAT) technology evaluated the rapid-pacing–induced VTs in each personalized inFAT-based substrate. DIFAT targets that render the inFAT substrate noninducible to VT, including VTs that arise postablation, were determined. DIFAT predictions were compared with corresponding clinical ablations to assess the capabilities of the technology. Results: DIFAT was developed and applied retrospectively to 29 ischemic cardiomyopathy patients with contrast-enhanced computed tomography. DIFAT ablation volumes were significantly less than the estimated clinical ablation volumes (1.87±0.35 versus 7.05±0.88 cm 3 , P Conclusions: DIFAT is a novel digital-heart technology for individualized VT ablation guidance designed to eliminate VT inducibility following initial ablation. DIFAT predictions colocalized well with clinical ablation locations but provided significantly smaller lesions. DIFAT also predicted VTs targeted in redo procedures years later. As DIFAT uses widely accessible computed tomography, its integration into clinical workflows may augment therapeutic precision and reduce redo procedures.

BACKGROUND: We previously developed an intraprocedural automated site of origin localization system to identify the origin of early left ventricular (LV) activation using 12-lead ECGs. However, it has limitations, as it could not identify the site of origin in the right ventricle (RV), and relied on acquiring a complete electroanatomic map (EAM). OBJECTIVE: The objective of this study was to present a new system, the Automatic Arrhythmia Origin Localization (AAOL) system, which utilized incomplete EAM for localization of idiopathic ventricular arrhythmia (IVA) origin on the patient-specific geometry of LV, RV and neighboring vessels. The accuracy of the system in localizing IVA source sites on cardiac structures where pace-mapping is challenging was assessed. METHODS: Twenty patients undergoing IVA catheter ablation had a 12-lead ECG recorded during clinical arrhythmia and during pacing at various locations identified on EAM geometries. The new system combined 3-lead (III, V2, V6) 120-ms QRS integrals and patient-specific EAM geometry with pace mapping to predict the site of earliest ventricular activation. The predicted site was projected onto EAM geometry. RESULTS: Twenty-three IVA origin sites were clinically identified by activation mapping and/or pace mapping (8 RV; 15 LV, including 8 from the posteromedial papillary muscle; 2 from the aortic root; and 1 from the distal coronary sinus). The new system achieved a mean localization accuracy of 3.6 mm for the 23 mapped IVAs. CONCLUSIONS: The new intraprocedural AAOL system achieved accurate localization of IVA origin in ventricles and neighbouring vessels, which could facilitate ablation procedures for patients with IVAs. Key Words: Idiopathic ventricular arrhythmias (IVA); Premature ventricular complexes (PVCs); idiopathic ventricular tachycardia (IVT); Pace mapping; Activation mapping; Radiofrequency (RF) Ablation; ECG.

Introduction: Few intraprocedural localization systems have been developed to predict idiopathic ventricular arrhythmia (IVA) source sites. However, an accurate and bi-ventricular patient-specific automated site of origin localization system remains elusive. To address this issue, we have developed a new automatic arrhythmia origin localization (AAOL) system that determines the sites of earliest activation in both ventricles and provides superior accuracy. Hypothesis: We hypothesized that the AAOL system can use electroanatomic mapping (EAM) geometry and accurately localize IVA source sites on patient-specific geometry of LV, RV and neighboring vessels using 3-lead ECGs. Methods: Twenty patients undergoing IVA catheter ablation had a 12-lead ECG recorded during clinical arrhythmia and during pacing at various locations identified on EAM geometries. The AAOL system combined 3-lead (III, V2, V6) 120-ms QRS integrals and patient-specific EAM geometry with intracardiac pacing to predict the site of earliest ventricular activation. The predicted site was projected onto the EAM geometry using the EAM triangular-mesh site nearest to the tip of the predicted site. Results: Twenty-three IVA source sites were clinically identified by activation mapping and/or pace mapping (8 RV, 15 LV, including 8 from the posteromedial papillary muscle; 2 from the aortic root; and 1 from the distal coronary sinus). The new system achieved a mean localization accuracy of 3.6 mm for the 23 mapped IVAs (Figure 1D), better than that achieved by previous systems. Conclusions: The new AAOL system offers highly accurate localization of IVA source sites in both ventricles and neighboring vessels, which could facilitate ablation procedures for patients with IVAs.

Introduction: With improved survival of sudden cardiac death in arrhythmogenic right ventricular cardiomyopathy (ARVC), progressive heart failure is increasingly recognized. Cardiopulmonary exercise testing (CPET) can play a role in prognostication. However, correlation of morphologic disease severity and exercise capacity is lacking. Hypothesis: Increased RV size and decreased RV function measured by two-dimensional echocardiography (2D echo) are associated with impaired exercise capacity parameters (peak oxygen consumption (pVO 2 ) and ventilator efficiency (VE/VCO 2 slope)). Methods: We retrospectively examined 21 patients who underwent 25 echocardiography and cardiopulmonary exercise testing (CPET) studies within 6 months of each other. A Spearman rank correlation was used to determine the relationship between RV dimensions and CPET parameters. Seven (28%) had submaximal effort defined as respiratory exchange ratio 2 . Results: Patients’ mean age was 44 ± 11 years and 28% were women. Despite more than half of patients (54%) being New York Heart Association Class I, mean pVO 2 was decreased at 19 ± 5 mL/kg per minute (63% ± 22% predicted for age and sex). There was a moderate negative correlation between pVO 2 and RV basal diameter ( r s = -0.46, p =0.05) and RV area in diastole ( r s = -0.55, p =0.02). There was no significant correlation between RV size or function with VE/VCO 2 slope. Conclusion: In this small study of ARVC patients, abnormalities on echo including increased RV basal diameter and RV diastolic area were associated with worse pVO 2 but not VE/VCO 2 slope. These findings suggest that RV structural abnormalities are related to reduced oxygen uptake on CPET, and larger studies are warranted to investigate the prognostic value and clinical course of ARVC patients with regards to the development of heart failure.

AIMS: Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVC) is characterized by ventricular arrhythmias (VAs) and sudden cardiac death (SCD). We aimed to develop a model for individualized prediction of incident VA/SCD in ARVC patients. METHODS AND RESULTS: Five hundred and twenty-eight patients with a definite diagnosis and no history of sustained VAs/SCD at baseline, aged 38.2 ± 15.5 years, 44.7% male, were enrolled from five registries in North America and Europe. Over 4.83 (interquartile range 2.44-9.33) years of follow-up, 146 (27.7%) experienced sustained VA, defined as SCD, aborted SCD, sustained ventricular tachycardia, or appropriate implantable cardioverter-defibrillator (ICD) therapy. A prediction model estimating annual VA risk was developed using Cox regression with internal validation. Eight potential predictors were pre-specified: age, sex, cardiac syncope in the prior 6 months, non-sustained ventricular tachycardia, number of premature ventricular complexes in 24 h, number of leads with T-wave inversion, and right and left ventricular ejection fractions (LVEFs). All except LVEF were retained in the final model. The model accurately distinguished patients with and without events, with an optimism-corrected C-index of 0.77 [95% confidence interval (CI) 0.73-0.81] and minimal over-optimism [calibration slope of 0.93 (95% CI 0.92-0.95)]. By decision curve analysis, the clinical benefit of the model was superior to a current consensus-based ICD placement algorithm with a 20.6% reduction of ICD placements with the same proportion of protected patients (P

Advances in cardiac magnetic resonance (CMR) techniques and image acquisition have made it an excellent tool in the assessment of atrial myopathy. Remolding of the left atrium is the mainstay of atrial fibrillation (AF) development and its progression. CMR can detect phasic atrial volumes, atrial function, and atrial fibrosis using cine, and contrast-enhanced or non-contrast-enhanced images. These abilities make CMR a versatile and extraordinary tool in management of patients with AF including for risk stratification, ablation prognostication and planning, and assessment of stroke risk. We review the latest advancements in utility of CMR in management of patients with AF.

Abnormalities on cardiac magnetic resonance imaging (CMR) and positron emission tomography (PET) predict ventricular arrhythmias (VA) in patients with cardiac sarcoidosis (CS). Little is known whether concurrent abnormalities on CMR and PET increases the risk of developing VA. Our aim was to compare the additive utility of CMR and PET in predicting VA in patients with CS. We included all patients treated at our institution from 2000 to 2018 who (1) had probable or definite CS and (2) had undergone both CMR and PET. The primary endpoint was VA at follow up, which was defined as sustained ventricular tachycardia, sudden cardiac death, or any appropriate device tachytherapy. Fifty patients were included, 88% of whom had a left ventricular ejection fraction >35%. During a mean follow-up 4.1 years, 7/50 (14%) patients had VA. The negative predictive value of LGE for VA was 100% and the negative predictive value of FDG for VA was 79%. Among groups, VA occurred in 4/21 (19%) subjects in the LGE+/FDG+ group, 3/14 (21%) in the LGE+/FDG- group, and 0/15 (0%) in the FDG+/LGE- group. There were no LGE-/FDG- patients. In conclusion, CMR may be the preferred initial clinical risk stratification tool in patients with CS. FDG uptake without LGE on initial imaging may not add additional prognostic information regarding VA risk.

Background Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited condition associated with ventricular arrhythmias and myocardial dysfunction; however, limited data exist on identifying patients at highest risk. The purpose of the study was to determine whether measures of right ventricular (RV) dysfunction on echocardiogram including RV strain were predictive of structural disease progression in ARVC. Methods and Results A retrospective analysis of serial echocardiograms from 40 patients fulfilling 2010 task force criteria for ARVC was performed to assess structural progression defined by an increase in proximal RV outflow tract dimensions (parasternal short or long axis) or decrease in RV fractional area change. Echocardiograms were analyzed for RV free‐wall peak longitudinal systolic strain using 2‐dimensional speckle tracking. Risk of structural progression and 5‐year change in RV outflow tract measurements were compared with baseline RV strain. Of the 40 ARVC patients, 61% had structural progression with an increase in the mean parasternal short‐axis RV outflow tract dimension from 36.2 to 38.5 mm ( P =0.022) and 68% by increase in parasternal long‐axis RV outflow tract dimension from 36.1 to 39.2 mm ( P =0.001). RV fractional area change remained stable over time. Baseline RV strain was significantly associated with the risk of structural progression and 5‐year rate of change. Patients with an RV strain more positive than −20% had a higher risk (odds ratio: 18.4; 95% CI, 2.7–125.8; P =0.003) of structural progression. Conclusions RV free wall strain is associated with the rate of structural progression in patients with ARVC. It may be a useful marker in determining which patients require closer follow‐up and treatment.

Background: Characterizing myocardial conduction velocity (CV) in patients with ischemic cardiomyopathy (ICM) and ventricular tachycardia (VT) is important for understanding the patient-specific proarrhythmic substrate of VTs and therapeutic planning. The objective of this study is to accurately assess the relation between CV and myocardial fibrosis density on late gadolinium–enhanced cardiac magnetic resonance imaging (LGE-CMR) in patients with ICM. Methods: We enrolled 6 patients with ICM undergoing VT ablation and 5 with structurally normal left ventricles (controls) undergoing premature ventricular contraction or VT ablation. All patients underwent LGE-CMR and electroanatomic mapping (EAM) in sinus rhythm (2960 electroanatomic mapping points analyzed). We estimated CV from electroanatomic mapping local activation time using the triangulation method that provides an accurate estimate of CV as it accounts for the direction of wavefront propagation. We evaluated the association between LGE-CMR intensity and CV with multilevel linear mixed models. Results: Median CV in patients with ICM and controls was 0.41 m/s and 0.65 m/s, respectively. In patients with ICM, CV in areas with no visible fibrosis was 0.81 m/s (95% CI, 0.59–1.12 m/s). For each 25% increase in normalized LGE intensity, CV decreased by 1.34-fold (95% CI, 1.25–1.43). Dense scar areas have, on average, 1.97- to 2.66-fold slower CV compared with areas without dense scar. Ablation lesions that terminated VTs were localized in areas of slow conduction on CV maps. Conclusions: CV is inversely associated with LGE-CMR fibrosis density in patients with ICM. Noninvasive derivation of CV maps from LGE-CMR is feasible. Integration of noninvasive CV maps with electroanatomic mapping during substrate mapping has the potential to improve procedural planning and outcomes. Visual Overview: A visual overview is available for this article.

Objectives This study examined the trend in growth of catheter ablation for ventricular tachycardia (VT) performed in the United States with analysis of rates and predictors of major adverse events. Background Sustained VT is a significant cause of sudden death, heart failure (HF), and recurrent shocks in implantable cardioverter-defibrillator (ICD) recipients. Catheter ablation for VT reduces arrhythmia recurrence. Limited data are available regarding the use, safety, and long-term outcomes after VT ablation. Methods Using the U.S. Medicare database linked to the Social Security Death Index, we examined the annual use of VT ablation in 21,073 patients over 12 years, with 30-day risk of mortality, nonfatal major adverse events (MAEs), 1-year risk of mortality, re-hospitalization, repeat ablation, and factors associated with adverse outcomes. Results Among 21,073 patients (age 70 ± 9 years; 77% men; 90% white), there were 1,581 (7.5%) non-fatal MAEs within 30 days. There were 963 (4.6%) vascular complications, 485 (2.3%) pericardial complications, and 201 (1%) strokes and/or transient ischemic attacks. Mechanical circulatory support use was infrequent (2.3%). The 30-day and 1-year mortality rates were 4.2% and 15.0%, respectively. The 1-year incidence of repeat ablation was 10.2 per 100 person-years and re-hospitalization for HF or VT was 15.4 per 100 person-years and 18 per 100 person-years, respectively. Patients with an ICD had increased 30-day (4.9% vs. 0.86%) and 1-year mortality (17.5% vs. 2.54% [22.9 per 100 person-years vs. 3.1 per 100 person-years]; hazard ratio [HR]: 2.93; 95% confidence interval [CI]: 2.21 to 3.88). Rates of hospitalization for HF (18 per 100 person-years vs. 1.8 per 100 person-years; HR: 4.00; 95% CI: 2.78 to 5.78) or VT recurrence (22.7 per 100 person-years vs. 2.1 per 100 person-years; HR: 5.70; 95% CI: 4.09 to 7.96) were also higher at 1 year. Between 2000 and 2012, annual VT ablation volumes increased >4-fold. Conclusions Catheter ablation for VT is frequently performed. Short-term MAEs and 1-year mortality is significant and is highest in patients with an ICD. These findings may provide greater insight of outcomes in an unselected real-world population undergoing VT ablation.

BACKGROUND Implantable defibrillators (ICD) are an important therapy for arrhythmogenic right ventricular cardiomyopathy (ARVC) patients at high risk of sudden death. Given the high appropriate ICD therapy rate, some have argued that the mere act of implanting an ICD inflates the malignant arrhythmia rate in ARVC. OBJECTIVE To report the arrhythmic course of ARVC patients without ICDs at the fulfillment of the 2010 Task Force Criteria and explore predictors of malignant ventricular arrhythmias. METHODS We included 131 definite ARVC patients (age 32 ± 15 years, male 39%, proband 50%) either without ICDs (N = 47) or receiving an ICD at least 6 months after the fulfillment of the diagnostic criteria. The primary outcome was a composite of cardiac arrest (both resuscitated successfully and unsuccessfully) and sustained ventricular tachyarrhythmias (cycle length

AIMS Prior studies identified a relationship between epicardial bipolar and endocardial unipolar voltage. Whether the relationship is valid with smaller multielectrode mapping catheters has not been reported. We explored the association of right ventricular (RV) endocardial unipolar voltage mapping with epicardial bipolar voltage mapping using a multielectrode mapping catheter. METHODS Electrograms from patients who underwent multielectrode endocardial and epicardial RV electroanatomical mapping during ablation procedures were analyzed. Each endocardial mapping point was matched to the corresponding nearest epicardial point. The correlation between unipolar endocardial voltage and epicardial bipolar voltage was determined. The optimal unipolar threshold to detect epicardial low voltage (

BACKGROUND: The clinical profile of arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) patients with late presentation is unknown.OBJECTIVE: The purpose of this study was to characterize the genotype, cardiac phenotype, and long-term outcomes of ARVC/D patients with late presentation (age ≥50 years at diagnosis).METHODS: Five hundred two patients with an ARVC/D diagnosis from Johns Hopkins and Utrecht Registries were studied and long-term clinical outcomes ascertained.RESULTS: Late presentation was seen in 104 patients (21%; 38% PKP2 carriers); 3% were ≥65 years at diagnosis. Sustained ventricular tachycardia was the major (43%) mode of presentation in patients with late presentation, whereas cardiac syncope was infrequent (P CONCLUSION: One-fifth of all ARVC/D patients present after age 50 years, often with sustained ventricular tachycardia, and are less likely to have prior syncope, ECG changes, ventricular ectopy, or identifiable pathogenic mutation. In ARVC/D, late presentation does not confer a benign prognosis and is associated with high arrhythmic risk.

BackgroundCardiac sympathetic denervation (CSD) has been shown to reduce the burden of implantable cardioverter-defibrillator (ICD) shocks in small series of patients with structural heart disease (SHD) and recurrent ventricular tachyarrhythmias (VT).ObjectivesThis study assessed the value of CSD and the characteristics associated with outcomes in this population.MethodsPatients with SHD who underwent CSD for refractory VT or VT storm at 5 international centers were analyzed by the International Cardiac Sympathetic Denervation Collaborative Group. Kaplan-Meier analysis was used to estimatefreedom from ICD shock, heart transplantation, and death. Cox proportional hazards models were used to analyze variables associated with ICD shock recurrence and mortality after CSD.ResultsBetween 2009 and 2016, 121 patients (age 55 ± 13 years, 26% female, mean ejection fraction of 30 ± 13%) underwent left or bilateral CSD. One-year freedom from sustained VT/ICD shock and ICD shock, transplant, and death were 58%and 50%, respectively. CSD reduced the burden of ICD shocks from a mean of 18 ± 30 (median 10) in the year before study entry to 2.0 ± 4.3 (median 0) at a median follow-up of 1.1 years (p< 0.01). On multivariable analysis, pre-procedure New York Heart Association functional class III and IV heart failure and longer VT cycle lengths were associated with recurrent ICD shocks, whereas advanced New York Heart Association functional class, longer VT cycle lengths, and a left-sided-only procedure predicted the combined endpoint of sustained VT/ICD shock recurrence, death, and transplantation. Of the 120 patients taking antiarrhythmic medications before CSD, 39 (32%) no longer required them at follow-up.ConclusionsCSD decreased sustained VT and ICD shock recurrence in patients with refractory VT. Characteristics independently associated with recurrence and mortality were advanced heart failure, VT cycle length, and a left-sided-only procedure.

Background Arrhythmogenic right ventricular cardiomyopathy/dysplasia ( ARVC /D) is characterized by high arrhythmic burden and progressive heart failure, which can prompt referral for heart transplantation. Cardiopulmonary exercise testing ( CPET ) has an established role in risk stratification for advanced heart failure therapies, but has not been described in ARVC /D. This study sought to determine the safety and prognostic utility of CPET in patients with ARVC /D. Methods and Results Using the Johns Hopkins ARVC /D Registry, we examined patients with ARVC /D undergoing CPET . Baseline characteristics and transplant‐free survival were compared on the basis of peak oxygen consumption (pVO2) (≤14 or >14 mL/kg per minute) and ventilatory efficiency (Ve/ VCO 2 slope ≤34 or >34). Thirty‐eight patients underwent 50 CPET s. There were no sustained arrhythmic events. Twenty‐nine patients achieved a maximal test. Patients with pVO2 ≤14 mL/kg per minute were more often men ( P =0.042) compared with patients with pVO2 >14 mL/kg per minute. Patients with Ve/ VCO 2 slope >34 tended to have more moderate/severe right ventricular dilation (7/9 [78%] versus 10/26 [38%]; P =0.060) and clinical heart failure (8/9 [89%] versus 13/26 [50%]; P =0.056) compared with patients with Ve/ VCO 2 slope ≤34. Patients who underwent heart transplantation were more likely to have clinical heart failure (10/10 [100%] versus 13/28 [46%]; P =0.003). Patients with Ve/ VCO 2 slope >34 had worse transplant‐free survival compared with patients with Ve/ VCO 2 slope ≤34 (n=35; hazard ratio, 6.57 [95% CI , 1.28–33.72]; log‐rank P =0.010), whereas transplant‐free survival was similar on the basis of pVO2 groups (n=29; hazard ratio, 3.38 [95% CI , 0.75–15.19]; log‐rank P =0.092). Conclusions CPET is safe to perform in patients with ARVC /D. Ve/ VCO 2 slope may be used for risk stratification and guide referral for heart transplantation in ARVC /D.

BACKGROUND Patients with cardiac sarcoidosis (CS) may present with arrhythmic events (AE): atrioventricular block (AVB) and/ or ventricular arrhythmias (VA). We sought to: (a) use regional analysis of cardiac magnetic resonance imaging (CMR) to describe anatomic and functional phenotypes of patients with CS and AE; (b) Assess the association of regional CMR abnormalities with the combined endpoint of death, heart transplantation (HT) and AE; and (c) use machine learning (ML) to predict the combined endpoint based on CMR features. METHODS we included 76 patients with CS and CMR. We analyzed cine images to determine regional longitudinal (LS) and radial strain (RS); and late gadolinium enhancement imaging to determine regional scar burden (%scar). RESULTS Patients with AVB (n = 7), compared with those without, had higher %scar in the anterior (21.8 ± 27.4 vs 5.1 ± 8.9; P = 0.0005) and anteroseptal (19.3 ± 24.5 vs 3.5 ± 5.5; P

Background Skeletal muscle activation has been implicated as the source of pain associated with implantable cardioverter-defibrillator shocks. We hypothesized that the skeletal muscle response to defibrillatory shocks could be attenuated with a tetanizing prepulse immediately before biphasic shock delivery. Objective The purpose of this study was to test the ability of tetanizing prepulses to reduce the skeletal muscle activation associated with defibrillation. Methods Seven adult pigs were studied. A left ventricular coil and subcutaneous dummy can in the right thorax were used to deliver either pure biphasic waveforms or test waveforms consisting of a tetanizing pulse of high-frequency alternating current (HFAC) ramped to an amplitude of 5–100 V over 0.25–1 second, immediately followed by a biphasic shock of approximately 9 J (ramped HFAC and biphasic [rHFAC+B]). We used limb acceleration and rate of force development as surrogate measures of pain. Test and control waveforms were delivered in sinus rhythm and induced ventricular fibrillation to test defibrillation efficacy. Results Defibrillation threshold energy was indistinguishable between rHFAC+B and pure biphasic shocks. Peak acceleration and rate of force development were reduced by 72% ± 7% and 71% ± 22%, respectively, with a 25-V, 1-second rHFAC+B waveform compared with pure biphasic shocks. Notably, rHFAC+B with a 9-J biphasic shock produced significantly less skeletal muscle activation than a 0.1-J pure biphasic shock. Conclusion A putative source of implantable cardioverter-defibrillator shock-related pain can be mitigated using a tetanizing prepulse followed by biphasic shock. Human studies will be required to assess true pain reduction with this approach.

We describe the novel use of bilateral cardiac sympathectomy in a woman with end-stage heart failure caused by ventricular tachycardia refractory to standard medical therapy who was under consideration for heart transplantation. Postoperatively, our patient has not experienced any symptoms of ventricular tachycardia, has returned to normal physical activity, and is no longer under consideration for transplantation as a result of the improvement in her cardiac function. Bilateral sympathectomy can be more effective than unilateral sympathectomy or percutaneous stellate ganglion blockade in patients with refractory ventricular tachycardia. Careful patient selection is necessary to identify patients who will benefit most from the procedure.

Background: Abnormalities on cardiac imaging (cardiac magnetic resonance imaging [CMR] or positron emission tomography [PET]), left ventricular ejection fraction (LVEF), and electrophysiology study (EPS) all predict ventricular arrhythmias (VA) in patients with cardiac sarcoidosis (CS). We sought to assess the utility of EPS in patients with CS and abnormal cardiac imaging, focusing on those with LVEF >35%. Methods: We identified all patients treated at our institution from 2000 to 2017 who: 1.) had probable or definite CS; 2.) had either late gadolinium enhancement (LGE) on CMR or abnormal 18-flourodeoxyglucose (FDG) uptake on PET, and 3.) had undergone EPS. The primary endpoint was VA during follow up. Results: Twenty five patients were included, of whom 10 (40%) had positive EPS. During a mean follow-up of 4.8 +/− 3.4 years, 11 (44%) patients had VA. The positive predictive value (PPV) of EPS for VA was 100% and the negative predictive value (NPV) of EPS for VA was 93%. Among 12 patients with LVEF >35% and no prior VA, the PPV of EPS for VA was 100% and the NPV of EPS for VA was 90%. Conclusion: EPS may help with risk stratification in patients with CS and abnormal imaging, especially those without conventional indications for ICD placement. Among patients with LVEF >35% and no history of prior VA, a negative EPS has good positive and negative predictive value for future VA events. Keywords: Cardiac sarcoidosis, Ventricular arrhythmia, Sudden cardiac death, Electrophysiology study, Implantable cardioverter defibrillator

This study sought to evaluate the association between contrast-enhanced multidetector computed tomography (CE-MDCT) attenuation and local epicardial conduction speed (ECS) and electrographic abnormalities in patients with arrhythmogenic right ventricular cardiomyopathy (ARVC) and ventricular tachycardia (VT).CE-MDCT is a widely available and fast imaging technology with high spatial resolution that is less prone to defibrillator generator-related safety issues and image artifacts. However, the association between hypoattenuation on MDCT and VT substrates in ARVC remains unknown.Patients with ARVC who underwent CE-MDCT followed by endocardial (n = 30) and epicardial (n = 21) electroanatomical mapping (EAM) and VT ablation were prospectively enrolled. Right ventricular (RV) mid-myocardial attenuation was calculated from 3-dimensional MDCT images and registered to EAM. Local ECS was calculated by averaging the ECS between each point and 5 adjacent points with concordant wave front direction.A total of 17,311 epicardial and 5,204 endocardial points were included. In multivariable regression analysis clustered by patient, RV myocardial attenuation was associated with epicardial bipolar voltage amplitude (2.5% decrease in amplitude per 10 HU decrease in attenuation; p 0.001), with endocardial unipolar voltage amplitude (0.9% decrease in amplitude per 10 HU decrease in attenuation; p 0.001), and with ECS (0.4% decrease in ECS per 10 HU decrease in attenuation; p = 0.001).CE-MDCT attenuation distribution is associated with regional ECS and electrographic amplitude in ARVC. Regions with low attenuation likely reflect fibro-fatty involvement in the RV and may serve as important VT substrates in patients with ARVC who are undergoing VT ablation.

Background Ventricular arrhythmias (VAs) in cardiac sarcoidosis (CS) are frequently refractory to both antiarrhythmic drug (AAD) therapy and catheter ablation (CA). Cardiac sympathetic denervation (CSD) has been shown to reduce VA burden and implantable cardioverter-defibrillator (ICD) shocks in patients with nonischemic cardiomyopathy. Objective We aimed to report our center's preliminary experience with CSD in patients with known or presumed CS and refractory VAs. Methods Patients with CS and refractory VAs who underwent CSD at our institution were included. Patient characteristics, procedural outcomes, and number of arrhythmic events including ICD shocks pre- and post-CSD are reported. Results Five patients with CS (mean age 53 ± 11 years; 2 men [40%]; mean left ventricular ejection fraction 38% ± 11%) underwent CSD for VA refractory to AAD therapy and CA. Four of 5 patients underwent bilateral CSD; 1 patient underwent right-sided sympathectomy only because of poor intraoperative visualization on the left. Procedural complications included hemothorax in 1 patient and azygous vein injury in 1 patient. The median number of ICD shocks in the 6 months pre-CSD was 5. During a median follow-up of 26 months (range 5–29 months), the median number of ICD shocks post-CSD was 0; 1 patient had sustained VA that was below the threshold for device therapy, and 1 patient had symptomatic premature ventricular contractions; both underwent repeat CA. In addition, 1 patient required cardiac transplantation for progressive heart failure. Conclusion CSD may be a feasible therapeutic adjunct for patients with CS and VA refractory to AAD therapy and CA.

PURPOSE: To compare epicardial fat in patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) with that in healthy subjects. MATERIALS AND METHODS: In this retrospective study, cardiac CT scans in 44 patients with ARVD/C (mean age, 39 years ± 12; 23 men) were compared with those in 45 control group participants between January 2008 and July 2015. Volumes of intrathoracic adipose tissue, mediastinal adipose tissue (MAT), and total epicardial adipose tissue (EAT) were quantified. EAT was subdivided into three regions—right ventricular (RV) EAT, left ventricular (LV) EAT, and peri-atrial EAT (atrial EAT)—and normalized to MAT for all regions. Logistic regression and receiver operating characteristic analysis were performed to evaluate the association between epicardial fat with the diagnosis of ARVD/C. RESULTS: Total EAT volume was higher in patients with ARVD/C than in healthy control group participants (median, 98 mL vs 76 mL, respectively; P = .04). Regionally, LV and RV EAT volumes were higher in patients with ARVD/C than in control group participants, most notably when indexed to MAT (median LV EAT index: 0.49 vs 0.15, respectively; median RV EAT index: 0.91 vs 0.52; P ˂ .0005 for both). The optimal cutoff for diagnosis of ARVD/C was an LV EAT index of 0.24, with a sensitivity and specificity of 91% and 71%, respectively. Atrial EAT volume and total intrathoracic adipose tissue volume were not different between groups. RV diameter showed a positive correlation with total EAT index and LV EAT index (r = 0.21, P = .05 and r = 0.33, P = .002, respectively). CONCLUSION: Higher amounts of right ventricular and left ventricular epicardial fat are found in hearts with arrhythmogenic right ventricular dysplasia/cardiomyopathy, particularly adjacent to the left ventricle, which correlates with disease severity and helps differentiate patients from healthy subjects. © RSNA, 2018 Online supplemental material is available for this article.

OBJECTIVES: This study sought to compare electrocardiogram (ECG) variants in athletic and arrhythmogenic right ventricular cardiomyopathy (ARVC) cohorts matched for the confounders of age, sex, and ethnicity.BACKGROUND: Anterior T-wave inversion (TWIV1-V4) is a common electrocardiographic finding in both athletes and patients with ARVC, and is a frequent conundrum in the setting of pre-participation screening. J-point elevation (JPE) has been proposed as an accurate means of identifying athletes, whereas disease markers, including premature ventricular contractions (PVCs) and low-voltage signals, have been associated with ARVC.METHODS: This study examined 200 subjects with TWI V1-V4, including 100 healthy athletes and 100 ARVC patients matched 1:1 for age, sex, and ethnicity (age: 21 ± 5 years for athletes vs. 22 ± 5 years for ARVC patients; 47% male; 97% Caucasian). The presence of TWI, JPE, PVCs, and left ventricular hypertrophy (LVH) were assessed.RESULTS: JPE was observed in 27% of athletes versus 16% of ARVC patients (p = 0.09). Thus, JPE had poor specificity (27%) and accuracy (60%) in identifying healthy athletes. In contrast, ARVC patients demonstrated a greater prevalence of precordial TWI beyond lead V3 (34% vs. 8%; p < 0.001), inferior TWI (31% vs. 3%; p < 0.001), PVCs (18% vs. 0%; p < 0.001), and lower LVH scores (SV1 + RV5; 19 ± 1 mm vs. 30 ± 1 mm; p < 0.001). These combined factors provided more reliable differentiation between health and disease (specificity 82%, accuracy 81%).CONCLUSIONS: PVCs and low QRS voltages are more prevalent among ARVC patients than athletes, whereas JPE is a relatively poor discriminator of health and disease when the confounders of age, sex, and ethnicity are considered.