ArticleCirculation2014
Electrophysiologic substrate in congenital Long QT syndrome: noninvasive mapping with electrocardiographic imaging (ECGI).
Article in Circulation, 2014. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 51 papers, 1 of them a synthesis that pooled it.
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Who cites it
51 citing papers in PubMed, 1 synthesis or guideline pooled it, 92 citations in OpenAlex.
- 2019 HRS/EHRA/APHRS/LAHRS expert consensus statement on catheter ablation of ventricular arrhythmias.Journal of interventional cardiac electrophysiology : an international journal of arrhythmias and pacing · 2020Pooled it
- Spatial and temporal dispersion of ventricular repolarization in pediatric patients with congenital long QT syndrome.HeartRhythm case reports · 2026Article
- Computer-aided characterization of the arrhythmogenic substrate after myocardial infarction.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 · 2026Review
- Detecting Local Myocardial Spatiotemporal Repolarization Gradients With Clinical Mapping Arrays.Circulation. Arrhythmia and electrophysiology · 2025Article
- Functional Profiling ofCirculation. Genomic and precision medicine · 2025Article
- Noninvasive Cardiac Electrical Activity Mapping Systems: Current Available Options.Reviews in cardiovascular medicine · 2025Review
- Druggability Studies of Benzene Sulfonamide Substituted Diarylamide (E3) as a Novel Diuretic.Biomedicines · 2025Article
- Interpretable manifold learning for T-wave alternans assessment with electrocardiographic imaging.Engineering applications of artificial intelligence · 2025Article
- The many faces of SCN5A pathogenic variants: from channelopathy to cardiomyopathy.Heart failure reviews · 2025Review
- Inappropriate ventricular pacing-induced ventricular fibrillation in a long QT type 3 syndrome patient with implantable cardioverter-defibrillator.HeartRhythm case reports · 2024Article
- Variant patterns of electrical activation and recovery in normal human hearts revealed by noninvasive electrocardiographic imaging.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
- High Density Body Surface Potential Mapping with Conducting Polymer-Eutectogel Electrode Arrays for ECG imaging.Advanced science (Weinheim, Baden-Wurttemberg, Germany) · 2024Article
- Electrophysiological Characterization of Subclinical and Overt Hypertrophic Cardiomyopathy by Magnetic Resonance Imaging-Guided Electrocardiography.Journal of the American College of Cardiology · 2024Article
- Mechanisms of torsades de pointes: an update.Frontiers in cardiovascular medicine · 2024Review
- Reference for Electrocardiographic Imaging-Based T-Wave Alternans Estimation.IEEE access : practical innovations, open solutions · 2024Article
- A two-step inverse solution for a single dipole cardiac source.Frontiers in physiology · 2023Article
- Electromechanical reciprocity and arrhythmogenesis in long-QT syndrome and beyond.European heart journal · 2022Review
- Assessment of time irreversibility in a time series using visibility graphs.Frontiers in network physiology · 2022Article
- Genotype-Specific ECG-Based Risk Stratification Approaches in Patients With Long-QT Syndrome.Frontiers in cardiovascular medicine · 2022Article
- Body Surface Potential Mapping: Contemporary Applications and Future Perspectives.Hearts (Basel, Switzerland) · 2021Article
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Authors and funding
10 authors at 5 institutions in 2 countries.
Funding
Abstract
backgroundCongenital Long QT syndrome (LQTS) is an arrhythmogenic disorder that causes syncope and sudden death. Although its genetic basis has become well-understood, the mechanisms whereby mutations translate to arrhythmia susceptibility in the in situ human heart have not been fully defined. We used noninvasive ECG imaging to map the cardiac electrophysiological substrate and examine whether LQTS patients display regional heterogeneities in repolarization, a substrate that promotes arrhythmogenesis. METHODS AND
resultsTwenty-five subjects (9 LQT1, 9 LQT2, 5 LQT3, and 2 LQT5) with genotype and phenotype positive LQTS underwent ECG imaging. Seven normal subjects provided control. Epicardial maps of activation, recovery times, activation-recovery intervals, and repolarization dispersion were constructed. Activation was normal in all patients. However, recovery times and activation-recovery intervals were prolonged relative to control, indicating delayed repolarization and abnormally long action potential duration (312±30 ms versus 235±21 ms in control). Activation-recovery interval prolongation was spatially heterogeneous, with repolarization gradients much steeper than control (119±19 ms/cm versus 2.0±2.0 ms/cm). There was variability in steepness and distribution of repolarization gradients between and within LQTS types. Repolarization gradients were steeper in symptomatic patients (130±27 ms/cm in 12 symptomatic patients versus 98±19 ms/cm in 13 asymptomatic patients; P<0.05).
conclusionsLQTS patients display regions with steep repolarization dispersion caused by localized action potential duration prolongation. This defines a substrate for reentrant arrhythmias, not detectable by surface ECG. Steeper dispersion in symptomatic patients suggests a possible role for ECG imaging in risk stratification.
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.