ArticleEuropace : European pacing, arrhythmias, and cardiac electrophysiology : journal of the working groups on cardiac pacing, arrhythmias, and cardiac cellular electrophysiology of the European Society of Cardiology2023
Right ventricular epicardial arrhythmogenic substrate in long-QT syndrome patients at risk of sudden death.
Article in Europace : European pacing, arrhythmias, and cardiac electrophysiology : journal of the working groups on cardiac pacing, arrhythmias, and cardiac cellular electrophysiology of the European Society of Cardiology, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 22 papers.
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Who cites it
22 citing papers in PubMed, 29 citations in OpenAlex.
- Electromechanical and structural phenotypes from cardiac imaging and epicardial mapping in inherited arrhythmia syndromes.Frontiers in cardiovascular medicine · 2026Review
- Broad Electrocardiogram Syndromes Spectrum: From Common Emergencies to Particular Electrical Heart Disorders-Part II.Diagnostics (Basel, Switzerland) · 2025Review
- Congenital Long QT Syndrome: A Focus on Risk Stratification and Management.Reviews in cardiovascular medicine · 2025Review
- The contribution of a short electrocardiographic diastolic interval to diastolic dysfunction and HFpEF.BMC cardiovascular disorders · 2025Article
- Exploring epicardial arrhythmogenic substrates in long QT syndrome type III overlapping with J-wave syndrome.HeartRhythm case reports · 2025Article
- Substrate modification via epicardial ablation in a patient with long QT syndrome type II.HeartRhythm case reports · 2024Article
- From genes to clinical management: A comprehensive review of long QT syndrome pathogenesis and treatment.Heart rhythm O2 · 2024Review
- Percutaneous epicardial access for mapping and ablation of cardiac arrhythmias: a procedure in need of innovation.Europace : European pacing, arrhythmias, and cardiac electrophysiology : journal of the working groups on cardiac pacing, arrhythmias, and cardiac cellular electrophysiology of the European Society of Cardiology · 2024Article
- Catheter Ablation for Channelopathies: When Is Less More?Journal of clinical medicine · 2024Review
- Sudden Cardiac Death in the Young: State-of-the-Art Review in Molecular Autopsy.Current issues in molecular biology · 2024Review
- Ablation of the epicardial substrate in patients with long-QT syndrome at risk of sudden death.European heart journal supplements : journal of the European Society of Cardiology · 2024Article
- Management of patients with an electrical storm or clustered ventricular arrhythmias: a clinical consensus statement of the European Heart Rhythm Association of the ESC-endorsed by the Asia-Pacific Heart Rhythm Society, Heart Rhythm Society, and Latin-American Heart Rhythm Society.Europace : European pacing, arrhythmias, and cardiac electrophysiology : journal of the working groups on cardiac pacing, arrhythmias, and cardiac cellular electrophysiology of the European Society of Cardiology · 2024Article
- Contributions of Israel to the field of clinical cardiac electrophysiology and implantable devices.Heart rhythm O2 · 2024Article
- Electrocardiographic temporo-spatial assessment of depolarization and repolarization changes after epicardial arrhythmogenic substrate ablation in Brugada syndrome.European heart journal. Digital health · 2023Article
- Future direction of substrate-based catheter ablation in Brugada syndrome and other inherited primary arrhythmia syndromes: Systematic review and meta-analysis.Journal of arrhythmia · 2023Article
- Twenty-five years of catheter ablation of ventricular tachycardia: a look back and a look forward.Europace : European pacing, arrhythmias, and cardiac electrophysiology : journal of the working groups on cardiac pacing, arrhythmias, and cardiac cellular electrophysiology of the European Society of Cardiology · 2023Article
- Ablation for long QT syndrome: local or global repolarization effects?Europace : European pacing, arrhythmias, and cardiac electrophysiology : journal of the working groups on cardiac pacing, arrhythmias, and cardiac cellular electrophysiology of the European Society of Cardiology · 2023Article
- Subepicardial Cardiomyopathy: A Disease Underlying J-Wave Syndromes and Idiopathic Ventricular Fibrillation.Circulation · 2023Article
- Letter to the editor dealing with paper by Dr Pappone and coworkers.Europace : European pacing, arrhythmias, and cardiac electrophysiology : journal of the working groups on cardiac pacing, arrhythmias, and cardiac cellular electrophysiology of the European Society of Cardiology · 2023Article
- Ablation of ventricular arrhythmias in cardiogenetic diseases.European heart journal supplements : journal of the European Society of Cardiology · 2023Article
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Authors and funding
10 authors at 3 institutions in 2 countries.
Funding
Abstract
aimsThe long-QT syndrome (LQTS) represents a leading cause of sudden cardiac death (SCD). The aim of this study was to assess the presence of an underlying electroanatomical arrhythmogenic substrate in high-risk LQTS patients. METHODS AND
resultsThe present study enrolled 11 consecutive LQTS patients who had experienced frequent implantable cardioverter-defibrillator (ICD discharges triggered by ventricular fibrillation (VF). We acquired electroanatomical biventricular maps of both endo and epicardial regions for all patients and analyzed electrograms sampled from several myocardial regions. Abnormal electrical activities were targeted and eliminated by the means of radiofrequency catheter ablation. VF episodes caused a median of four ICD discharges in eleven patients (6 male, 54.5%; mean age 44.0 ± 7.8 years, range 22-53) prior to our mapping and ablation procedures. The average QTc interval was 500.0 ± 30.2 ms. Endo-epicardial biventricular maps displayed abnormally fragmented, low-voltage (0.9 ± 0.2 mV) and prolonged electrograms (89.9 ± 24.1 ms) exclusively localized in the right ventricular epicardium. We found electrical abnormalities extending over a mean epicardial area of 15.7 ± 3.1 cm2. Catheter ablation of the abnormal epicardial area completely suppressed malignant arrhythmias over a mean 12 months of follow-up (median VF episodes before vs. after ablation, 4 vs. 0; P = 0.003). After the procedure, the QTc interval measured in a 12-lead ECG analysis shortened to a mean of 461.8 ± 23.6 ms (P = 0.004).
conclusionThis study reveals that, among high-risk LQTS patients, regions localized in the epicardium of the right ventricle harbour structural electrophysiological abnormalities. Elimination of these abnormal electrical activities successfully prevented malignant ventricular arrhythmia recurrences.
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