Beyond the length and look of repolarization: Defining the non-QTc electrocardiographic profiles of patients with congenital long QT syndrome

Lane, Conor M.; Bos, J. Martijn; Rohatgi, Ram K.; Ackerman, Michael J.

doi:10.1016/j.hrthm.2018.04.033

Cited by 19 publications

(25 citation statements)

References 30 publications

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“…The clinical and genetic epidemiology of congenital LQTS has been described in detail in Western populations. For example, differences in electrocardiographic variables have been observed between LQTS types 1, 2, and 3 (1). Bradycardia is a common feature regardless of subtype (1) and early-onset atrial fibrillation may be present (2).…”

Section: Introductionmentioning

confidence: 99%

RETRACTED: Territory-Wide Chinese Cohort of Long QT Syndrome: Random Survival Forest and Cox Analyses

Tse

Lee²,

Zhou

et al. 2021

Front. Cardiovasc. Med.

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Introduction: Congenital long QT syndrome (LQTS) is a cardiac ion channelopathy that predisposes affected individuals to spontaneous ventricular tachycardia/fibrillation (VT/VF) and sudden cardiac death (SCD). The main aims of the study were to: (1) provide a description of the local epidemiology of LQTS, (2) identify significant risk factors of ventricular arrhythmias in this cohort, and (3) compare the performance of traditional Cox regression with that of random survival forests.Methods: This was a territory-wide retrospective cohort study of patients diagnosed with congenital LQTS between 1997 and 2019. The primary outcome was spontaneous VT/VF.Results: This study included 121 patients [median age of initial presentation: 20 (interquartile range: 8–44) years, 62% female] with a median follow-up of 88 (51–143) months. Genetic analysis identified novel mutations in KCNQ1, KCNH2, SCN5A, ANK2, CACNA1C, CAV3, and AKAP9. During follow-up, 23 patients developed VT/VF. Univariate Cox regression analysis revealed that age [hazard ratio (HR): 1.02 (1.01–1.04), P = 0.007; optimum cut-off: 19 years], presentation with syncope [HR: 3.86 (1.43–10.42), P = 0.008] or VT/VF [HR: 3.68 (1.62–8.37), P = 0.002] and the presence of PVCs [HR: 2.89 (1.22–6.83), P = 0.015] were significant predictors of spontaneous VT/VF. Only initial presentation with syncope remained significant after multivariate adjustment [HR: 3.58 (1.32–9.71), P = 0.011]. Random survival forest (RSF) model provided significant improvement in prediction performance over Cox regression (precision: 0.80 vs. 0.69; recall: 0.79 vs. 0.68; AUC: 0.77 vs. 0.68; c-statistic: 0.79 vs. 0.67). Decision rules were generated by RSF model to predict VT/VF post-diagnosis.Conclusions: Effective risk stratification in congenital LQTS can be achieved by clinical history, electrocardiographic indices, and different investigation results, irrespective of underlying genetic defects. A machine learning approach using RSF can improve risk prediction over traditional Cox regression models.

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Section: Introductionmentioning

confidence: 99%

RETRACTED: Territory-Wide Chinese Cohort of Long QT Syndrome: Random Survival Forest and Cox Analyses

Tse

Lee²,

Zhou

et al. 2021

Front. Cardiovasc. Med.

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“…Maximum HR during exercise may also be reduced in LQTS [see ( 8 , 11 ), and primary references cited therein]. Thus, LQTS may have a direct impact on HR ( 17 ), but this is not a consistent finding ( 18 , 19 ). One may argue that this is because effects on HR differ between types of LQTS and between specific mutations.…”

Section: Introductionmentioning

confidence: 99%

Long QT Syndrome and Sinus Bradycardia–A Mini Review

Wilders

Verkerk

2018

Front. Cardiovasc. Med.

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Congenital long-QT syndrome (LQTS) is an inherited cardiac disorder characterized by the prolongation of ventricular repolarization, susceptibility to Torsades de Pointes (TdP), and a risk for sudden death. Various types of congenital LQTS exist, all due to specific defects in ion channel-related genes. Interestingly, almost all of the ion channels affected by the various types of LQTS gene mutations are also expressed in the human sinoatrial node (SAN). It is therefore not surprising that LQTS is frequently associated with a change in basal heart rate (HR). However, current data on how the LQTS-associated ion channel defects result in impaired human SAN pacemaker activity are limited. In this mini-review, we provide an overview of known LQTS mutations with effects on HR and the underlying changes in expression and kinetics of ion channels. Sinus bradycardia has been reported in relation to a large number of LQTS mutations. However, the occurrence of both QT prolongation and sinus bradycardia on a family basis is almost completely limited to LQTS types 3 and 4 (LQT3 and Ankyrin-B syndrome, respectively). Furthermore, a clear causative role of this sinus bradycardia in cardiac events seems reserved to mutations underlying LQT3.

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“…Although there is disagreement between U.S. (who recommend only anamnesis and physical examination) and European authorities (who recommend adding 12-lead ECG to anamnesis and clinical examination), 314 the Brazilian Society of Cardiology considers 12-lead ECG as mandatory at the first cardiological examination. 315 Resting ECG can diagnose numerous heart diseases that can lead to SD, including long QT syndrome, 316 Brugada syndrome, 193 Wolf-Parkinson-White syndrome 317 and HCM. 318 The European protocol, which includes anamnesis, physical examination and ECG, is currently used by the International Olympic Committee, the Italian Olympic Committee, FIFA and the Union of European Football Associations.…”

Section: Basic Life Support For Athletesmentioning

confidence: 99%

The Brazilian Society of Cardiology and Brazilian Society of Exercise and Sports Medicine Updated Guidelines for Sports and Exercise Cardiology - 2019

Ghorayeb¹,

Stein²,

Daher³

et al. 2019

Arquivos Brasileiros De Cardiologia

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Beyond the length and look of repolarization: Defining the non-QTc electrocardiographic profiles of patients with congenital long QT syndrome

Cited by 19 publications

References 30 publications

RETRACTED: Territory-Wide Chinese Cohort of Long QT Syndrome: Random Survival Forest and Cox Analyses

RETRACTED: Territory-Wide Chinese Cohort of Long QT Syndrome: Random Survival Forest and Cox Analyses

Long QT Syndrome and Sinus Bradycardia–A Mini Review

The Brazilian Society of Cardiology and Brazilian Society of Exercise and Sports Medicine Updated Guidelines for Sports and Exercise Cardiology - 2019

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