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The Long QT syndrome (LQTS) is a disorder characterized by a prolongation of the QT interval and a propensity to ventricular tachyarrhythmias, which may lead to syncope, cardiac arrest, or sudden death. Our objective was to (1) determine the incidence of v

Background Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a heritable arrhythmia syndrome entailing a high risk of sudden cardiac death. Discernment from benign arrhythmia disorders, particularly right ventricular outflow tract ventricular tachycardia (RVOT VT), may be challenging, providing an impetus to explore alternative modalities that may facilitate evaluation of patients with suspected ARVC. Objective We evaluated the role of equilibrium radionuclide angiography (ERNA) as a diagnostic tool for ARVC. Methods ERNA measures of ventricular synchrony - synchrony (S) and entropy (E) - were examined in patients with ARVC (n = 16), those with RVOT VT (n = 13), and healthy controls (n = 49). The sensitivity and specificity of ERNA parameters for ARVC diagnosis were compared with those of echocardiography (ECHO) and cardiovascular magnetic resonance (CMR). Results ERNA right ventricular synchrony parameters in patients with ARVC (S = 0.91 ± 0.07; E = 0.61 ± 0.1) differed significantly from those in patients with RVOT VT (S = 0.99 ± 0.01 [P =.0015]; E = 0.46 ± 0.05 [P

Ventricular arrhythmias in patients with ARVD/C are common. Differentiation between idiopathic VT and ARVD is of utmost importance. Baseline sinus rhythm electrocardiography as well as electrocar- diographic differences during ventricular arrhythmias (VT o

While Drs Wolff, Parkinson, and White fully described the syndrome in 1930, prior case reports had described the essentials. Over the ensuing century this syndrome has captivated the interest of anatomists, clinical cardiologists, and cardiac surgeons. Sta

The outcome of posteroseptal accessory pathway ablation by direct current (DC) shocks delivered just outside the os of the coronary sinus was studied in 21 patients. Electrocardiographic and electrophysiological parameters as well as phase image patterns of equilibrium multiple-gated blood-pool scintigrams were studied to determine their usefulness in predicting the success of ablation. A second free-wall pathway was documented by electrophysiological or surgical findings in six patients, and the value of phase images in detecting this second pathway was studied as well. Ablation was successful in 57%. The cumulative mean energy of DC shocks amounted to 524 +/- 170 joules and was not predictive of ablation outcome, neither was the mean ventriculoatrial (VA) conduction time. The predictive value of the 12-lead maximally preexcited electrocardiogram was poor in the 15 patients with a single posteroseptal bypass tract. A new method to triangulate the site of the earliest phase angle on the atrioventricular (AV) valve plane successfully localized the bypass pathway in 14 of those patients. No specific phase pattern predicted successful ablation except for a symmetrical, concentric peripheral phase progression found to be predictive of ablation success in the four patients who showed this pattern. Phase analysis was able to localize the second, nonposteroseptal pathway in four of six patients. This study showed that a concentric peripheral phase progression in the gated blood-pool scintigrams is predictive for ablation success in patients with posteroseptal pathways. A free-wall localization of the earliest phase angle is suggestive of a second bypass tract in this area.

There are no disease-specific questionnaires to measure patient sensitive outcomes in arrhythmia patients. We report the development and preliminary psychometric testing of the Patient Perception of Arrhythmia Questionnaire (PPAQ). The PPAQ was developed using formative research, exploratory factor analysis, expert review, pilot study, and regression. The PPAQ measures frequency and duration of episodes, symptoms, impact on daily activities, and restricted activity days. After preliminary content validation, the sensitivity of the PPAQ was tested in 103 arrhythmia patients. The measures showed good sensitivity and reliability. Preliminary construct validation was supported by significant differences (p < .001) among groups of arrhythmia patients consistent with clinical patterns. Preliminary evidence from patients with supraventricular arrhythmias suggests that the questionnaire has acceptable psychometrics and could be useful in future studies of arrhythmia patients. [ABSTRACT FROM AUTHOR]

Background: Atrial fibrillation (AF) may be due to an inherited trait, particularly in lone AF patients. A family history of AF in lone AF patients has not previously been compared with a family history of patients with AF and established risk factors (non-lone AF).Objective: The purpose of this study was to compare the frequency of having a first-degree relative with AF in lone and non-lone AF patients.Methods: We performed a case-control study of consecutive subjects presenting to a single electrophysiology laboratory. A convenience sample of subjects with no known arrhythmias was also enrolled.Results: Four hundred twenty-nine subjects were enrolled: 136 had AF (54 with lone AF), 84 had atrial flutter, 158 had other supraventricular arrhythmias, and 51 had no known arrhythmias. Significantly more subjects with AF reported a first-degree family history of AF compared with the remainder of the cohort (25% vs. 5%; P <.001). In multivariable analysis adjusting for potential confounders, AF patients had a 6-fold greater odds of having a family member with AF (95% confidence interval [CI] 2.93-12.7; P <.001). Lone AF patients had a first-degree family member with AF substantially more often than those with non-lone AF (41% vs. 14%; P <.001). After adjusting for potential confounders, lone AF patients remained significantly more likely than other AF patients to have a first-degree relative with AF (OR 7.2; 95% CI 2.1-24.7; P = .002).Conclusion: Lone AF patients have a first-degree family member with AF substantially more often than other AF patients. This suggests that an inherited trait may be particularly important in this subgroup of patients. [ABSTRACT FROM AUTHOR]

Look for signs of significant underlying cardiac disease in order to identify patients at high risk of sudden cardiac death. Let the latest data on drugs, devices, and other evolving treatments for ventricular arrhythmias guide your way! [ABSTRACT FROM AUTHOR]

One simple principle governs how far to take the workup when the patient has premature ventricular contractions, atrial fibrillation, or supraventricular tachycardia: Context is everything. [ABSTRACT FROM AUTHOR]

We prospectively studied the efficacy of pindolol, a beta-adrenergic blocker with intrinsic sympathomimetic activity (ISA), for the prevention of syncope recurrences in 31 patients with recurrent neurocardiogenic syncope. Pindolol proved to be an effective treatment, even in patients who had previously failed treatment with conventional beta blockers, suggesting a clinical benefit from addition of ISA to beta blockade in this setting. [ABSTRACT FROM AUTHOR]

Introduction: The decremental properties of the nodoventricular pathway (NVP) are uncertain., Methods and Results: During short RP supraventricular tachycardia, a His-refractory premature ventricular contraction (PVC) consistently terminated the tachycardia without atrial capture immediately after the PVC. Whereas a slightly earlier PVC failed to reset the subsequent His but terminated the tachycardia without atrial capture one cycle later., Conclusion: These observations are diagnostic of slow-fast atrioventricular nodal reentrant tachycardia (AVNRT) with a bystander concealed-NVP and can be explained by decremental properties in the NVP itself; greater prematurity of the PVC resulted in more decremental conduction over the NVP, causing the AVNRT termination one cycle later., (© 2024 Wiley Periodicals LLC.)

Competing Interests: Funding Support and Author Disclosures The authors have reported that they have no relationships relevant to the contents of this paper to disclose.

Competing Interests: Disclosures The authors have no conflicts of interest to disclose.

Importance: Sudden death and cardiac arrest frequently occur without explanation, even after a thorough clinical evaluation. Calcium release deficiency syndrome (CRDS), a life-threatening genetic arrhythmia syndrome, is undetectable with standard testing and leads to unexplained cardiac arrest., Objective: To explore the cardiac repolarization response on an electrocardiogram after brief tachycardia and a pause as a clinical diagnostic test for CRDS., Design, Setting, and Participants: An international, multicenter, case-control study including individual cases of CRDS, 3 patient control groups (individuals with suspected supraventricular tachycardia; survivors of unexplained cardiac arrest [UCA]; and individuals with genotype-positive catecholaminergic polymorphic ventricular tachycardia [CPVT]), and genetic mouse models (CRDS, wild type, and CPVT were used to define the cellular mechanism) conducted at 10 centers in 7 countries. Patient tracings were recorded between June 2005 and December 2023, and the analyses were performed from April 2023 to December 2023., Intervention: Brief tachycardia and a subsequent pause (either spontaneous or mediated through cardiac pacing)., Main Outcomes and Measures: Change in QT interval and change in T-wave amplitude (defined as the difference between their absolute values on the postpause sinus beat and the last beat prior to tachycardia)., Results: Among 10 case patients with CRDS, 45 control patients with suspected supraventricular tachycardia, 10 control patients who experienced UCA, and 3 control patients with genotype-positive CPVT, the median change in T-wave amplitude on the postpause sinus beat (after brief ventricular tachycardia at ≥150 beats/min) was higher in patients with CRDS (P < .001). The smallest change in T-wave amplitude was 0.250 mV for a CRDS case patient compared with the largest change in T-wave amplitude of 0.160 mV for a control patient, indicating 100% discrimination. Although the median change in QT interval was longer in CRDS cases (P = .002), an overlap between the cases and controls was present. The genetic mouse models recapitulated the findings observed in humans and suggested the repolarization response was secondary to a pathologically large systolic release of calcium from the sarcoplasmic reticulum., Conclusions and Relevance: There is a unique repolarization response on an electrocardiogram after provocation with brief tachycardia and a subsequent pause in CRDS cases and mouse models, which is absent from the controls. If these findings are confirmed in larger studies, this easy to perform maneuver may serve as an effective clinical diagnostic test for CRDS and become an important part of the evaluation of cardiac arrest.

Competing Interests: Funding Support and Author Disclosures The authors have reported that they have no relationships relevant to the contents of this paper to disclose.

Competing Interests: Disclosures None.

Competing Interests: Disclosures The author has no conflicts of interest to disclose.

Competing Interests: None relevant to the manuscript.

Competing Interests: None.

Background: Alcohol consumption is associated with a higher increased risk of atrial fibrillation (AF), but the acute effects on cardiac electrophysiology in humans remain poorly understood. The HOw ALcohol InDuces Atrial TachYarrhythmias (HOLIDAY) Trial revealed that alcohol shortened pulmonary vein atrial effective refractory periods, but more global electrophysiologic changes gleaned from the surface ECG have not yet been reported., Methods: This was a secondary analysis of the HOLIDAY Trial. During AF ablation procedures, 100 adults were randomized to intravenous alcohol titrated to 0.08% blood alcohol concentration versus a volume and osmolarity-matched, masked, placebo. Intervals measured from 12‑lead ECGs were compared between pre infusion and at infusion steady state (20 min)., Results: The average age was 60 years and 11% were female. No significant differences in the P-wave duration, PR, QRS or QT intervals, were present between alcohol and placebo arms. However, infusion of alcohol was associated with a statistically significant relative shortening of the JT interval (r: -14.73, p = 0.048) after multivariable adjustment., Conclusion: Acute exposure to alcohol was associated with a relative reduction in the JT interval, reflecting shortening of ventricular repolarization. These acute changes may reflect a more global shortening of refractoriness, suggesting immediate proarrhythmic effects pertinent to the atria and ventricles., Competing Interests: Declaration of competing interest The authors declare the following financial interests/personal relationships which may be considered as potential competing interests: Gregory M. Marcus reports financial support was provided by National Institute on Alcohol Abuse and Alcoholism Division of Intramural Clinical and Biological Research., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Competing Interests: Funding Support and Author Disclosures Drs Yagishita and Shoda belong to the same endowed department established by contributions from Medtronic Japan, Boston-Scientific, Biotronik Japan, and Abbott Medical. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose.

Competing Interests: Disclosures None.

Although there is consensus on the management of patients with Brugada Syndrome with high risk for sudden cardiac arrest, asymptomatic or intermediate-risk patients present clinical management challenges. This document explores the management opinions of experts throughout the world for patients with Brugada Syndrome who do not fit guideline recommendations. Four real-world clinical scenarios were presented with commentary from small expert groups for each case. All authors voted on case-specific questions to evaluate the level of consensus among the entire group in nuanced diagnostic and management decisions relevant to each case. Points of agreement, points of controversy, and gaps in knowledge are highlighted., Competing Interests: Disclosures Dr Lubitz is a full-time employee of Novartis Institutes of BioMedical Research as of July 18, 2022. Dr Lubitz previously received sponsored research support from Bristol Myers Squibb, Pfizer, Boehringer Ingelheim, Fitbit, Medtronic, Premier, and IBM and has consulted for Bristol Myers Squibb, Pfizer, Blackstone Life Sciences, and Invitae.

Competing Interests: Disclosures None.