Dynamic QT response to cold‐water face immersion in long‐QT syndrome type 3

Takahashi, Kazuhiro; Shimizu, Wataru; Makita, Naomasa; Nakayashiro, Mami

doi:10.1111/ped.14319

Cited by 7 publications

(12 citation statements)

References 42 publications

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“… 160 The fact that cardiac events are more often associated with sympathetic stimulation (physical or emotional stress) forms the basis of different provocative tests to uncover concealed LQTS. 161 , 162 , 163 , 164 , 165 , 166 , 167 , 168 , 169 , 170 , 171 Therefore, in order to further improve identification of LQTS and to augment the diagnostic sensitivity to further unmask concealed LQTS, different solutions have been proposed: heart rate dependent of QT interval, 161 mental stress test, 162 standing response, 163 sympathetic stimulation, 164 , 165 , 166 , 171 and exercise test. 167 , 168 , 169 , 170 Additionally, the use of provocative drug challenges with epinephrine can be used to facilitate diagnosis.…”

Section: Molecular Underpinning Of Lqtsmentioning

confidence: 99%

“…[161][162][163][164][165][166][167][168][169][170][171] Therefore, in order to further improve identification of LQTS and to augment the diagnostic sensitivity to further unmask concealed LQTS, different solutions have been proposed: heart rate dependent of QT interval, 161 mental stress test, 162 standing response, 163 sympathetic stimulation, [164][165][166]171 and exercise test. [167][168][169][170] Additionally, the use of provocative drug challenges with epinephrine can be used to facilitate diagnosis.…”

Section: Lqts Diagnosismentioning

confidence: 99%

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Long QT syndrome – Bench to bedside

Ponce-Balbuena

Deschênes

2021

Heart Rhythm O2

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Long QT syndrome (LQTS) is a cardiovascular disorder characterized by an abnormality in cardiac repolarization leading to a prolonged QT interval and T-wave irregularities on the surface electrocardiogram. It is commonly associated with syncope, seizures, susceptibility to torsades de pointes, and risk for sudden death. LQTS is a rare genetic disorder and a major preventable cause of sudden cardiac death in the young. The availability of therapy for this lethal disease emphasizes the importance of early and accurate diagnosis. Additionally, understanding of the molecular mechanisms underlying LQTS could help to optimize genotype-specific treatments to prevent deaths in LQTS patients. In this review, we briefly summarize current knowledge regarding molecular underpinning of LQTS, in particular focusing on LQT1, LQT2, and LQT3, and discuss novel strategies to study ion channel dysfunction and drug-specific therapies in LQT1, LQT2, and LQT3 syndromes.

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Section: Molecular Underpinning Of Lqtsmentioning

confidence: 99%

Section: Lqts Diagnosismentioning

confidence: 99%

Long QT syndrome – Bench to bedside

Ponce-Balbuena

Deschênes

2021

Heart Rhythm O2

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“…It is noticeable that a majority (60.5%) of IVF patients suffered the index arrhythmic event during rest. LQTS type 3 is thought to be affected by parasympathetic tone, and abnormal QT dynamics during parasympathetic provocation test has also been observed 12 . It is conceivable to think parasympathetic tone also play a role in IVF.…”

Section: Discussionmentioning

confidence: 99%

“…However, the overall diagnostic work-up of all patients is comparable to similar studies. 12 It is conceivable to think parasympathetic tone also play a role in IVF. We observed a low incidence of subsequently diagnosed patients (3.6%) compared to similar studies.…”

Section: Clinical Assessmentmentioning

confidence: 99%

Clinical characteristics and risk factors of arrhythmia during follow‐up of patients with idiopathic ventricular fibrillation

Stampe

Jespersen

Glinge

et al. 2020

Cardiovasc electrophysiol

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Aims: The current knowledge of idiopathic ventricular fibrillation (IVF) is limited. We aimed to investigate the nature of IVF, including clinical assessment and later diagnosis, and risk factors of implantable cardioverter defibrillator (ICD) therapy in the follow-up period. Methods: Between 2007 and 2019 we systematically identified all patients from Rigshospitalet, Denmark, with a resuscitated sudden cardiac arrest (SCA) with no identifiable cause. All patients were followed routinely in the ICD outpatient clinic and the majority also in an inherited heart disease outpatient clinic. Outcomes were analysed with Cox regressions models and cumulative incidence curves. Results: We identified 84 patients with an initial diagnosis of IVF; of these, three (3.6%) patients were later diagnosed with a cardiac disease. The remaining IVF patients (n = 81, median age 45 years; men 71.6%) were followed a median follow-up of 5.2 years (interquartile range, 2.0-7.6). A total of 24 (29.6%) patients had appropriate ICD therapy and 12 (14.8%) patients had inappropriate ICD therapy. No predominant type of ventricular arrhythmia at first appropriate ICD therapy was observed. Early repolarization at baseline was not associated with an increased risk of appropriate ICD therapy (P = .842). Repeated cardiac arrest at index SCA increased the risk of appropriate ICD therapy (hazard ratio, 2.63 [95% CI, 1.08-6.40; P = .033]). Conclusion: Most patients remained idiopathic throughout the follow-up period and the overall long-term prognosis of IVF was good. Repeated cardiac arrest at index SCA was a risk factor of appropriate ICD therapy and early repolarization was not associated with an increased risk of appropriate ICD therapy.

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“…Provocative testing, including exercise stress testing (treadmill test, 5 , 6 , 7 , 8 , 9 , 10 , 11 , 12 , 13 bicycle test 14 , 15 , 16 , 17 , 18 ), abrupt stand testing, 5 , 6 , 8 , 19 , 20 , 21 , 22 and epinephrine QT stress testing, 23 , 24 , 25 , 26 has been explored as a means of unmasking the characterization of patients with LQTS. QTc interval changes in provocative testing have been proposed as meaningful parameters to facilitate diagnosis and genotyping of LQTS but without uniform standards between studies.…”

mentioning

confidence: 99%

Utility of Provocative Testing in the Diagnosis and Genotyping of Congenital Long QT Syndrome: A Systematic Review and Meta‐Analysis

Yang

et al. 2022

JAHA

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Background Diagnosis is particularly challenging in concealed or asymptomatic long QT syndrome (LQTS). Provocative testing, unmasking the characterization of LQTS, is a promising alternative method for the diagnosis of LQTS, but without uniform standards. Methods and Results A comprehensive search was conducted in PubMed, Embase, and the Cochrane Library through October 14, 2021. The fixed effects model was used to assess the effect of the provocative testing on QTc interval. A total of 22 studies with 1137 patients with LQTS were included. At baseline, QTc interval was 40 ms longer in patients with LQTS than in controls (mean difference [MD], 40.54 [95% CI, 37.43–43.65]; P <0.001). Compared with the control group, patients with LQTS had 28 ms longer ΔQTc upon standing (MD, 28.82 [95% CI, 23.05–34.58]; P <0.001), nearly 30 ms longer both at peak exercise (MD, 27.31 [95% CI, 21.51–33.11]; P <0.001) and recovery 4 to 5 minutes (MD, 29.85 [95% CI, 24.36–35.35]; P <0.001). With epinephrine infusion, QTc interval was prolonged both in controls and patients with QTS, most obviously in LQT1 (MD, 68.26 [95% CI, 58.91–77.60]; P <0.001) and LQT2 (MD, 60.17 [95% CI, 50.18–70.16]; P <0.001). Subgroup analysis showed QTc interval response to abrupt stand testing and exercise testing varied between LQT1, LQT2, and LQT3, named Type Ⅰ, Type Ⅱ, and Type Ⅲ. Conclusions QTc trend Type Ⅰ and Type Ⅲ during abrupt stand testing and exercise testing can be used to propose a prospective evaluation of LQT1 and LQT3, respectively. Type Ⅱ QTc trend combined epinephrine infusion testing could distinguish LQT2 from control. A preliminary diagnostic workflow was proposed but deserves further evaluation.

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Dynamic QT response to cold‐water face immersion in long‐QT syndrome type 3

Cited by 7 publications

References 42 publications

Long QT syndrome – Bench to bedside

Long QT syndrome – Bench to bedside

Clinical characteristics and risk factors of arrhythmia during follow‐up of patients with idiopathic ventricular fibrillation

Utility of Provocative Testing in the Diagnosis and Genotyping of Congenital Long QT Syndrome: A Systematic Review and Meta‐Analysis

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