Independent validation and clinical implications of the risk prediction model for long QT syndrome (1-2-3-LQTS-Risk): comment—Authors' reply

Mazzanti, Andrea; Trancuccio, Alessandro; Kukavica, Deni; Pagan, Eleonora; Wang, Meng; Mohsin, Muhammed; Peterson, Derick R.; Bagnardi, Vincenzo; Zaręba, Wojciech; Priori, Silvia G.

doi:10.1093/europace/euac013

Cited by 5 publications

(2 citation statements)

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“… 2 Scientific understanding has increased. 3 However, despite advances in treatment, LQTS remains a lethal disease. Untreated genotyped but asymptomatic patients face a 36% chance of a non-fatal cardiac event and a 13% risk of SCD.…”

Section: Introductionmentioning

confidence: 99%

Right ventricular epicardial arrhythmogenic substrate in long-QT syndrome patients at risk of sudden death

et al. 2023

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Aims The 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 results The 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). Conclusion This 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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Section: Introductionmentioning

confidence: 99%

Right ventricular epicardial arrhythmogenic substrate in long-QT syndrome patients at risk of sudden death

et al. 2023

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“…1 In long QT syndrome (LQTS), the diagnosis usually requires the presence of QT prolongation, often associated with abnormal T-wave morphology, and the duration of the corrected QT interval (QTc) has prognostic value. [2][3][4] Outside the ICC setting, accurately and reproducibly assessing cardiac repolarisation forms part of the monitoring for other paediatric conditions, such as during certain medical therapy and cancer treatment. 5 6 KardiaMobile (AliveCor) is a mobile personal ECG device that uses a smartphone application to record a 30 s ECG, either single lead or six lead, analogous to the six limb leads (I, II, III, aVL, aVR, aVF) on a conventional 12-lead ECG.…”

Section: Introductionmentioning

confidence: 99%

Utility and acceptability of remote 6-lead electrocardiographic monitoring in children with inherited cardiac conditions

Lawley,

Luczak-Wozniak,

Chung

et al. 2024

Arch Dis Child

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ObjectiveThis pilot study sought to investigate the utility and acceptability of the KardiaMobile 6-lead ECG (KM6LECG) as a tool for remote monitoring in children with inherited cardiac conditions.DesignA single-centre prospective cohort study. Children underwent standard clinical evaluation including a 12-lead ECG and a KM6LECG in the clinic. Participants recorded KM6LECGs monthly at home for 3 months. Families completed a questionnaire on their experience.SettingGreat Ormond Street Hospital Centre for Inherited Cardiovascular Diseases.Participants64 children: 22 with hypertrophic cardiomyopathy (HCM); 22 with long QT syndrome and 20 unaffected siblings (controls).Main outcome measuresComparison of data extracted from the clinic 12-lead ECG and supervised KM6LECG, and the supervised and unsupervised KM6LECG recording.ResultsOf 64 children (35% female, mean age 12 years), 58 had a baseline 12-lead ECG and appropriate baseline KM6LECG. In children with HCM, abnormalities in ventricular depolarisation/repolarisation in the limb leads of the 12-lead ECG were reliably reproduced. From the whole cohort, there was a strong positive correlation between the corrected QT interval from the 12-lead ECG and baseline KM6LECG (intraclass correlation coefficient=0.839) and baseline KM6LECG with an unsupervised KM6LECG (intraclass correlation coefficient=0.736). Suspected ‘lead’ misplacement impacted 18% of unsupervised recordings. Overall, the acceptability of the KM6LECG to families was good.ConclusionsThe KM6LECG provides an accurate tool for assessing some ECG abnormalities associated with paediatric inherited cardiovascular disease and may provide a useful at-home adjunct to face-to-face clinical care of children requiring ECG assessment.

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