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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.

Therapeutic hypothermia (TH) is one of the few proven neuroprotective modalities in clinical practice. However, current methods to achieve TH are suboptimal. We investigated a novel esophageal device that utilizes high-flow transesophageal dry air to achieve TH via evaporating cooling. Seven Yorkshire pigs (

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.

Inducing normothermia with surface cooling temperature modulating devices (TMDs) is cumbersome and often associated with significant shivering. We tested the safety and feasibility of a novel transnasal evaporative cooling device to induce and maintain normothermia in febrile patients following ischemic and hemorrhagic stroke. A single-center study utilizing the CoolStat® transnasal cooling device was used to achieve core temperature reduction in mechanically ventilated stroke patients with fever (T ≥ 38.3 C) refractory to acetaminophen by inducing an evaporative cooling energy exchange in the nasal turbinates thru a high flow of dehumidified air into the nasal cavity and out through the mouth. Continuous temperature measurements were obtained from tympanic and core (esophageal or bladder) temperature monitors. Safety assessments included continuous monitoring for hypertension, tachycardia, and raised intracranial pressure (when monitored). Otolaryngology (ENT) evaluations were monitored for any device-related nasal mucosal injury with a pre- and post-visual examination. Shivering was assessed every 30 min using the Bedside Shivering Assessment Scale (BSAS). Duration of device use was limited to 8 h, at which time patients were transitioned to routine care for temperature management. Ten subjects (median age: 54 years, BMI: 32.5 kg/m2, 60% men) were enrolled with normothermia achieved in 90% of subjects. One subject did not achieve normothermia and was later refractory to other TMDs. Median baseline temperature was 38.5 ± 0.1 C, with a reduction noted by 4 h (38.5 ± 0.1 vs 37.3 ± 0.8, P

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.

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.

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.

RationalePatients with ischemic cardiomyopathy (ICMP) are at high risk for malignant arrhythmias, largely due to electrophysiological remodeling of the non-infarcted myocardium. The electrophysiological properties of the non-infarcted myocardium of patients with ICMP remain largely unknown.ObjectivesTo assess the pro-arrhythmic behavior of non-infarcted myocardium in ICMP patients and couple computational simulations with machine learning to establish a methodology for the development of disease-specific action potential models based on clinically measured action potential duration restitution (APDR) data.Methods and ResultsWe enrolled 22 patients undergoing left-sided ablation (10 ICMP) and compared APDRs between ICMP and structurally normal left ventricles (SNLVs). APDRs were clinically assessed with a decremental pacing protocol. Using genetic algorithms (GAs), we constructed populations of action potential models that incorporate the cohort-specific APDRs. The variability in the populations of ICMP and SNLV models was captured by clustering models based on their similarity using unsupervised machine learning. The pro-arrhythmic potential of ICMP and SNLV models was assessed in cell- and tissue-level simulations. Clinical measurements established that ICMP patients have a steeper APDR slope compared to SNLV (by 38%, p < 0.01). In cell-level simulations, APD alternans were induced in ICMP models at a longer cycle length compared to SNLV models (385–400 vs 355 ms). In tissue-level simulations, ICMP models were more susceptible for sustained functional re-entry compared to SNLV models.ConclusionMyocardial remodeling in ICMP patients is manifested as a steeper APDR compared to SNLV, which underlies the greater arrhythmogenic propensity in these patients, as demonstrated by cell- and tissue-level simulations using action potential models developed by GAs from clinical measurements. The methodology presented here captures the uncertainty inherent to GAs model development and provides a blueprint for use in future studies aimed at evaluating electrophysiological remodeling resulting from other cardiac diseases.

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

BackgroundLeft ventricular (LV) fibrofatty infiltration in arrhythmogenic right ventricular (RV) dysplasia/cardiomyopathy (ARVD/C) has been reported, however, detailed cardiovascular magnetic resonance (CMR) characteristics and association with outcomes are uncertain. We aim to describe LV findings on CMR in ARVD/C patients and their relationship with arrhythmic outcomes.MethodsCMR of 73 subjects with ARVD/C according to the 2010 Task Force Criteria (TFC) were analyzed for LV involvement, defined as ≥ 1 of the following features: LV wall motion abnormality, LV late gadolinium enhancement (LGE), LV fat infiltration, or LV ejection fraction (LVEF) ResultsOf the 73 subjects, 50.7% had CMR evidence for LV involvement. Proband status and advanced RV dysfunction were independently associated with LV abnormalities. The most common pattern of LV involvement was focal fatty infiltration in the sub-epicardium of the apicolateral LV with a “bite-like” pattern. LGE in the LV was found in the same distribution and most often had a linear appearance. LV involvement was more common with non-PKP2genetic mutation variants, regardless of proband status. Only RV structural disease on CMR (HR 3.47, 95% CI 1.13–10.70) and prior arrhythmia (HR 2.85, 95% CI 1.33–6.10) were independently associated with arrhythmic events.ConclusionAmong patients with 2010 TFC for ARVD/C, CMR evidence for LV abnormalities are seen in half of patients and typically manifest as fibrofatty infiltration in the subepicardium of the apicolateral wall and are not associated with arrhythmic outcomes.

Corticosteroids are the mainstay of therapy for cardiac sarcoidosis (CS) ([1][1]); however, long-term exposure may be associated with numerous side effects. Although cardiac 18-fluorodoxyglucose position emission tomography (FDG-PET) is useful in tailoring corticosteroid therapy, the effect of

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.

Targeted temperature management (TTM) is recommended as a standard of care for postcardiac arrest patients. Current TTM methods have significant limitations to be used in an ambulatory setting. We investigated the efficacy and safety of a novel noninvasive transnasal evaporative cooling device (CoolStat™). Eleven Yorkshire pigs underwent hypothermia therapy using the CoolStat device. CoolStat induces evaporative cooling by blowing dehumidified ambient air over the nasal turbinates in a unidirectional fashion. CoolStat's efficacy and safety were assessed by applying different cooling strategies (groups A, B and C). In group A (efficacy study; n = 5, TTM for 8 hours), time to achieve brain target temperature (2°C reduction from baseline), and the percentage of time in which the temperature ranged within ±0.5°C after reaching the target temperature were investigated. In the safety assessment (groups B and C), two worst-case therapy situations were reproduced: in group B (n = 3), continuous maximum air flow (65 L/min) was applied without temperature control and, in group C (n = 3), subjects underwent 24-hour TTM (prolonged therapy). Hemodynamic and respiratory parameters, nasal mucosa integrity (endoscopic assessment), and other therapy-related adverse effects were evaluated. Efficacy study: CoolStat cooling therapy successfully induced and sustained managed hypothermia in all subjects. Brain target temperature was achieved in 0.5 ± 0.6 hours and kept within a ±0.5°C range for the therapy duration (99.9% ± 0.1%). All animals completed the safety studies. Maximum air flow (group B) and 24-hour (group C) therapies were well tolerated and no significant damage was observed on nasal mucosa for neither of the groups. CoolStat was able to efficiently induce and maintain hypothermia using unidirectional high flow of dry air into the nostrils of porcine models. CoolStat therapy was well tolerated and no damage to nasal mucosa was observed under either maximum air flow or prolonged therapy.

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

Fever is an important determinant of prognosis following acute brain injury. Current non-pharmacologic techniques to reduce fever are limited and induce a shivering response. We investigated the safety and efficacy of a novel transnasal unidirectional high flow air device in reducing core body temperature in the neurocritical care unit (NCCU) setting. This pilot study included seven consecutive patients in the NCCU who were febrile (> 37.5 °C) for > 24 h despite standard non-pharmacologic and first-line antipyretic agents. Medical grade high flow air was delivered transnasally using a standard continuous positive airway pressure machine with a positive pressure of 20 cmH2O for 2 h. Core esophageal and tympanic temperature were continuously monitored. Mean age was 40 ± 14 yo, and 72% (5/7 patients) were men. Five patients had intracerebral or intraventricular hemorrhage, one subject had transverse myelitis, and the remaining patient had anoxic brain injury due to a cardiac arrest. After 2 h of cooling, core temperature was significantly lower than the baseline pre-cooling temperature (37.3 ± 0.5 °C vs. 38.4 ± 0.6 °C; p

Early initiation of hypothermia is recommended in the setting of cardiac arrest. Current hypothermia methods are invasive and expensive and not applicable in ambulatory settings. We investigated the evaporative cooling effect of high flow transnasal dry air on core esophageal temperature in human volunteers.A total of 32 subjects (mean age 53.2 ± 9.3 yrs., mean weight 90 ± 17 kg) presenting for elective electrophysiological procedures were enrolled for the study. Half of the subjects were men. Following general anesthesia induction, high flow (30 LPM) medical grade ambient dry air with a relative humidity ∼20% was administered through a nasal mask for 60 min. Core temperature was monitored at the distal esophagus. Half of the subjects (16/32) were subject to high flow air and the remainder served as controls. Over a 1-h period, mean esophageal temperature decreased from 36.1 ± 0.3 °C to 35.5 ± 0.1 °C in the test subjects (p 0.05). No significant change in temperature was observed in the control subjects (36.3 ± 0.3 °C to 36.2 ± 0.2 °C, p = NS). No adverse events occurred.Transnasal high flow dry air through the nasopharynx reduces core body temperature. This mechanism can be harnessed to induce hypothermia in patients where clinically indicated without any deleteriouseffects in a short time exposure.

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: Pulmonary vein isolation is a common catheter ablation technique used to treat atrial fibrillation originating from the pulmonary veins. However, incomplete lesion formation, pulmonary vein reconnection, and collateral damage to the phrenic nerve and esophagus can occur. Electroporation is a new modality to ablate and has the potential for permanent pulmonary vein isolation and selective efficacy on cardiac tissue; however, strong evidence of selective myocardial injury using electroporation is lacking. Methods: Monolayers of neonatal rat ventricular cardiomyocytes, rat cortical neurons, and esophageal smooth muscle cells were stained with propidium iodide to measure shock-induced cell death. Biphasic shocks (10 ms) were delivered from line electrodes (1 mm separation). Neonatal rat ventricular cardiomyocytes were optically mapped to evaluate postelectroporation electrical conduction. Results: Conduction block occurred when 50% to 80% of the cells near the electrode were killed and required 400±50 V/cm with the electrodes in contact versus 690±70 V/cm with the electrodes 1 mm above the cells ( P Conclusions: Cell type alone yielded selective efficacy to electroporation without the confounding influences present in clinical studies, but electrode proximity to the target tissue remains important for efficacy. This exciting result suggests that electroporation may be a more selective modality for pulmonary vein isolation. Graphic Abstract: A graphic abstract is available for this article.

Background Intranasal high flow of dehumidified (dry) air results in evaporative cooling of nasal passages. In this randomized clinical trial, we investigated the effect of dry gas induced nasal cooling on migraine headaches. Methods In this single-blind study, acute migraineurs were randomized to either nasal high-flow dry oxygen, dry air, humidified oxygen or humidified air (control) at 15 L/min for 15 min. All gases were delivered at 37°C. Severity of headache and other migraine associated symptoms (International Classification for Headache Disorders, 3rd edition criteria) were recorded before and after therapy. The primary endpoint was change in pain scores, while changes in nausea, photosensitivity and sound sensitivity scores served as secondary endpoints. A linear regression model was employed to estimate the impact of individual treatment components and their individual interactions. Results Fifty-one patients (48 ± 15 years of age, 82% women) were enrolled. When compared to the control arm (humidified air), all therapeutic arms showed a significantly greater reduction in pain scores (primary endpoint) at 2 h of therapy with dry oxygen (−1.6 [95% CI −2.3, −0.9]), dry air (−1.7 [95% CI −2.6, −0.7)]), and humidified oxygen (−2.3 [95% CI −3.5, −1.1]). A significantly greater reduction in 2-h photosensitivity scores was also noted in all therapeutic arms (−1.8 [95% CI −3.2, −0.4], dry oxygen; −1.7 [95% CI −2.9, −0.4], dry air; (−2.1 [95% CI −3.6, −0.6], humidified oxygen) as compared to controls. The presence of oxygen and dryness were independently associated with significant reductions in pain and photosensitivity scores. No adverse events were reported. Conclusion Trans-nasal high-flow dry gas therapy may have a role in reducing migraine associated pain. Clinical Trial registration: NCT04129567

Sympathectomy - A “one hit wonder” for life?Fabrizio R. Assis, M.D. Harikrishna Tandri, M.D.From Division of Cardiology, Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA

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.

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.

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.

Catheter ablation has become an important element in the management of atrial fibrillation. Several technical advances allowed for better safety profiles and lower recurrence rates, leading to an increasing number of ablations worldwide. Despite that, major complications are still reported, and esophageal thermal injury remains a significant concern as atrioesophageal fistula (AEF) is often fatal. Recognition of the mechanisms involved in the process of esophageal lesion formation and the identification of the main determinants of risk have set the grounds for the development and improvement of different esophageal protective strategies. More sensitive esophageal temperature monitoring, safer ablation parameters and catheters, and different energy sources appear to collectively reduce the risk of esophageal thermal injury. Adjunctive measures such as the prophylactic use of proton-pump inhibitors, as well as esophageal cooling or deviation devices, have emerged as complementary methods with variable but promising results. Nevertheless, as a multifactorial problem, no single esophageal protective measure has proven to be sufficiently effective to eliminate the risk, and further investigation is still warranted. Early screening in the patients at risk and prompt intervention in the cases of AEF are important risk modifiers and yield better outcomes.

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.

Introduction: High transnasal airflow at ambient temperature increases evaporative cooling of the nasal passages and drives a countercurrent heat exchange between cooled venous blood draining the nasal turbinates with cephalic arterial blood. Hypothesis: High transnasal airflow is not inferior to standard surface cooling in protecting the brain in an infant swine model of asphyxic cardiac arrest. Methods: Arterial O2 saturation was decreased to ~35% for 45 min followed by 7 min airway occlusion to produce asphyxic cardiac arrest in 2-week-old anesthetized piglets (4 kg). Viable neuronal counts were assessed at 6 days of recovery in 6 groups (n=5-9): 1) sham surgery, 2) normothermic recovery, 3) surface cooling to decrease rectal temperature from 38.5 to 34C between 10-120 min 4) transnasal cooling with airflow of 32 L/min from 10-120 min, 5) surface cooling onset delayed until 120 min ROSC, and 6) transnasal cooling delayed by 120 min ROSC. In all 4 cooling groups, hypothermia was sustained at 34C with surface cooling until 20 h ROSC followed by 6-8 h of rewarming. Results: Nasal airflow of 32 L/min decreased brain temperature from 38.3±0.3°C to 33.8±0.6 within 60 min without spatial temperature gradients in these 45-g brains. Surface cooling and transnasal airflow rescued the number of viable neurons in putamen from 38±23% (% of sham viable neurons; ±SD) in the normothermic group to 67±33% and 76±36%, respectively, when initiated at 10 min ROSC, and to 72±30% and 61±25%, respectively, when initiated at 120 min. In sensorimotor cortex, surface cooling and transnasal airflow rescued neurons from 56±36% in the normothermic group to 89±37% and 89±29%, respectively, when initiated at 10 min ROSC, and to 84±19% and 81±28%, respectively, when initiated at 120 min. Conclusions: The use of a high transnasal airflow is as effective as standard surface cooling when initiated at 10 or 120 min after ROSC in protecting vulnerable putamen and sensorimotor cortex from asphyxic cardiac arrest in infant piglets. Because of its simplicity, portability, and low cost, we postulate that transnasal cooling potentially could be deployed in the field by first responders for early initiation of brain cooling prior to maintenance with standard surface cooling after pediatric cardiac arrest.

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 (