Congenital long QT syndrome: Diagnosis and management in pediatric patients

Bar‐Cohen, Yaniv; Silka, Michael J.

doi:10.1007/s11936-006-0043-5

Cited by 11 publications

(3 citation statements)

References 40 publications

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“…We also found that propranolol, a b-receptor blocker, decreases susceptibility to VF, relating to its suppression of antioxidant activity (Anderson et al 1996) and other pro-inflammatory factors that lead to abnormal expression of ERG protein in the myocardium. This is consistent with previous reports that propranolol can be used to treat LQTS (Bar-Cohen & Silka 2006;Hobbs et al 2006).…”

Section: Over-expression Of Erg Proteinsupporting

confidence: 93%

Inflammatory factors that contribute to upregulation of ERG and cardiac arrhythmias are suppressed by CPU86017, a class III antiarrhythmic agent

Dai

et al. 2008

Journal of Pharmacy and Pharmacology

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The aim of this study was to verify whether exaggerated arrhythmogenesis is attributed to inflammatory factors actively involving an excess of reactive oxygen species (ROS), transforming growth factor (TGF)-b and endothelin (ET). We hypothesized that CPU86017, derived from berberine, which possesses multi-channel blocking activity, could suppress inflammatory factors, resulting in inhibition of over-expression of ether-a-go-go (ERG) and an augmented incidence of ventricular fibrillation (VF) in ischaemia/reperfusion (I/R). Rats with cardiomyopathy (CMP) induced by thyroxine (0.2 mg −1 kg −1 s.c. daily for 10 days) were treated with propranolol (10 mgkg −1 p.o.) or CPU86017(80 mgkg −1 p.o.) on days 6-10. On the 11th day, arrhythmogenesis of the CMP was evaluated by I/R.In the CMP control group, an increase in VF incidence was found with the I/R episode, accompanied by increased ROS, which manifested as an increased level of malondialdehyde and decreased activities of SOD, glutathione peroxidase and catalase in the myocardium. Levels of inducible nitric oxide synthase and TGF-b mRNA were increased in association with upregulation of preproET-1 and ETconverting enzyme. We found increased levels of ERG, which correlated well with arrhythmogenesis. Treatment with CPU86017 or propranolol reversed these changes. These experiments verified our hypothesis that the inflammatory factors ROS, iNOS, TGF-b and ET-1 are actively involved in upregulation of ERG and arrhythmogenesis. CPU86017 and propranolol reduced VF by suppressing these inflammatory factors in the myocardium.

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Section: Over-expression Of Erg Proteinsupporting

confidence: 93%

Inflammatory factors that contribute to upregulation of ERG and cardiac arrhythmias are suppressed by CPU86017, a class III antiarrhythmic agent

Dai

et al. 2008

Journal of Pharmacy and Pharmacology

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“…Congenital coronary artery anomalies Absence of specific findings or ischaemic findings 22,24 Kawasaki disease QRS amplitude changes: increased S wave depth in V1, increased R wave height in V6, increased Q wave depth in V6; Increased QT dispersion 174 Hypertrophic cardiomyopathy Pathological Q waves and repolarisation abnormalities; Increased left ventricular voltage 30 Arrhythmogenic right ventricular dysplasia T-wave inversion in V3; QRS ⩾ 110 ms in V1-V2-V3; Right bundle branch block; epsilon wave (arrow); frequent premature ventricular complexes with left bundle branch block morphology 175,176 LQT syndrome QTc ⩾480 ms; biphasic contour or prominent notch in the T wave; T-wave morphologic alterations; alternating T-wave morphology. [177][178][179] LQT1: broad T wave; LQT2: lowamplitude T wave; LQT3: long isoelectric ST segment Short QT syndrome QTc < 320 ms; short or absent ST segment; tall, narrow, and peaked T-waves 75,77,180 Catecholaminergic polymorphic ventricular tachycardia…”

Section: Conditionmentioning

confidence: 99%

Sudden cardiac death in the young: the bogeyman

et al. 2014

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Sudden cardiac death in the young is a relatively uncommon but marked event usually related to congenital diseases or anomalies. Despite the prevalence of each condition being variable, most common causes include primary myocardial diseases and arrhythmic disorder, frequently with inheritance pattern. Sudden cardiac death is usually preceded by symptoms, thus making personal and family history fundamental for its prevention. Nevertheless, in more than 50% of cases, sudden cardiac death is the first manifestation of the disease. In this review, we describe the different causes of sudden cardiac death, their incidence, and currently used preventive strategies.

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“…[3][4][5][6] Whether these events are due to inherent limitations of ␤-blocker or avoidable factors is unknown. Implantable cardioverter-defibrillators (ICDs) are being used increasingly in LQTS patients, and these "failures" are used to justify implantation in patients without a cardiac arrest and even as initial therapy, including in children and teenagers in whom ICDs produce many complications [7][8][9][10] and impair quality of life without always preventing sudden death. 8,11,12 …”

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confidence: 99%

High Efficacy of β-Blockers in Long-QT Syndrome Type 1

et al. 2009

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Background-␤-Blocker efficacy in long-QT syndrome type 1 is good but variably reported, and the causes of cardiac events despite ␤-blocker therapy have not been ascertained. Methods and Results-This was a retrospective study of the details surrounding cardiac events in 216 genotyped long-QT syndrome type 1 patients treated with ␤-blocker and followed up for a median time of 10 years. Before ␤-blocker, cardiac events occurred in 157 patients (73%) at a median age of 9 years, with cardiac arrest (CA) in 26 (12%). QT-prolonging drugs were used by 17 patients; 9 of 17 (53%) had CA compared with 17 of 199 nonusers (8.5%; odds ratio, 12.0; 95% confidence interval, 4.1 to 35.3; PϽ0.001). After ␤-blocker, 75% were asymptomatic, and cardiac events were significantly reduced (PϽ0.001), with a median event count (quartile 1 to 3) per person of 0 (0 to 1). Twelve patients (5.5%) suffered CA/sudden death, but 11 of 12 (92%) were noncompliant (nϭ8), were on a QT-prolonging drug (nϭ2), or both (nϭ1) at the time of the event. The risk for CA/sudden death in compliant patients not taking QT-prolonging drugs was dramatically less compared with noncompliant patients on QT-prolonging drugs (odds ratio, 0.03; 95% confidence interval, 0.003 to 0.22; Pϭ0.001). None of the 26 patients with CA before ␤-blocker had CA/sudden death on ␤-blockers. Conclusions-␤-Blockers

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Congenital long QT syndrome: Diagnosis and management in pediatric patients

Cited by 11 publications

References 40 publications

Inflammatory factors that contribute to upregulation of ERG and cardiac arrhythmias are suppressed by CPU86017, a class III antiarrhythmic agent

Inflammatory factors that contribute to upregulation of ERG and cardiac arrhythmias are suppressed by CPU86017, a class III antiarrhythmic agent

Sudden cardiac death in the young: the bogeyman

High Efficacy of β-Blockers in Long-QT Syndrome Type 1

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