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144 results

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Page 1
Long QT Syndrome Management during and after Pregnancy.
Marcinkeviciene A, Rinkuniene D, Puodziukynas A. Marcinkeviciene A, et al. Medicina (Kaunas). 2022 Nov 21;58(11):1694. doi: 10.3390/medicina58111694. Medicina (Kaunas). 2022. PMID: 36422233 Free PMC article. Review.
Long QT syndrome (LQTS) is majorly an autosomal dominantly inherited electrical dysfunction, but there are exceptions (Jervell and Lange-Nielsen syndrome is inherited in an autosomal recessive pattern). ...
Long QT syndrome (LQTS) is majorly an autosomal dominantly inherited electrical dysfunction, but there are exceptions ( …
From Genotype to Phenotype.
Mackley M, McGuire K, Taylor J, Watkins H, Ormondroyd E. Mackley M, et al. Circ Genom Precis Med. 2018 Oct;11(10):e002316. doi: 10.1161/CIRCGEN.118.002316. Circ Genom Precis Med. 2018. PMID: 30354302 Free PMC article. Review.
Here we illustrate some diagnostic and psychosocial complexities of inherited cardiac condition secondary findings, exemplified by disclosure of a pathogenic variant in KCNQ1, associated with long QT syndrome, to a healthy male enrolled in diagnostic genome s …
Here we illustrate some diagnostic and psychosocial complexities of inherited cardiac condition secondary findings, exemplified by disclosur …
Provocation testing in congenital long QT syndrome: A practical guide.
Abrahams T, Davies B, Laksman Z, Sy RW, Postema PG, Wilde AAM, Krahn AD, Han HC. Abrahams T, et al. Heart Rhythm. 2023 Nov;20(11):1570-1582. doi: 10.1016/j.hrthm.2023.07.059. Epub 2023 Jul 20. Heart Rhythm. 2023. PMID: 37481219 Review.
Congenital long QT syndrome (LQTS) is a hereditary cardiac channelopathy with an estimated prevalence of 1 in 2500. A prolonged resting QT interval corrected for heart rate (QTc interval) remains a key diagnostic component; however, the QTc value may b …
Congenital long QT syndrome (LQTS) is a hereditary cardiac channelopathy with an estimated prevalence of 1 in 2500. A p …
Short QT syndrome.
Brugada R, Hong K, Cordeiro JM, Dumaine R. Brugada R, et al. CMAJ. 2005 Nov 22;173(11):1349-54. doi: 10.1503/cmaj.050596. CMAJ. 2005. PMID: 16301704 Free PMC article. Review.
It has long been appreciated that a long QT interval predisposes patients to life-threatening ventricular arrhythmia. Short QT syndrome is a newly described disease characterized by a shortened QT interval and by episodes of syncope, paro …
It has long been appreciated that a long QT interval predisposes patients to life-threatening ventricular arrhythmia. S …
Celebrities in the heart, strangers in the pancreatic beta cell: Voltage-gated potassium channels K(v) 7.1 and K(v) 11.1 bridge long QT syndrome with hyperinsulinaemia as well as type 2 diabetes.
Lubberding AF, Juhl CR, Skovhøj EZ, Kanters JK, Mandrup-Poulsen T, Torekov SS. Lubberding AF, et al. Acta Physiol (Oxf). 2022 Mar;234(3):e13781. doi: 10.1111/apha.13781. Epub 2022 Jan 22. Acta Physiol (Oxf). 2022. PMID: 34990074 Free PMC article. Review.
Recently, individuals carrying loss-of-function (LoF) mutations in KCNQ1, encoding K(v) 7.1, and KCNH2 (hERG), encoding K(v) 11.1, were found to exhibit post-prandial hyperinsulinaemia and episodes of hypoglycaemia. These LoF mutations also cause the cardiac disorder lo …
Recently, individuals carrying loss-of-function (LoF) mutations in KCNQ1, encoding K(v) 7.1, and KCNH2 (hERG), encoding K(v) 11.1, we …
Utility of Provocative Testing in the Diagnosis and Genotyping of Congenital Long QT Syndrome: A Systematic Review and Meta-Analysis.
Yang Y, Lv TT, Li SY, Liu P, Gao QG, Zhang P. Yang Y, et al. J Am Heart Assoc. 2022 Jul 19;11(14):e025246. doi: 10.1161/JAHA.122.025246. Epub 2022 Jul 8. J Am Heart Assoc. 2022. PMID: 35861842 Free PMC article. Review.
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. ... …
Background Diagnosis is particularly challenging in concealed or asymptomatic long QT syndrome (LQTS). Provocative test …
Long QT Syndrome.
Goldenberg I, Zareba W, Moss AJ. Goldenberg I, et al. Curr Probl Cardiol. 2008 Nov;33(11):629-94. doi: 10.1016/j.cpcardiol.2008.07.002. Curr Probl Cardiol. 2008. PMID: 18835466 Review.
The hereditary Long QT syndrome (LQTS) is a genetic channelopathy with variable penetrance that is associated with increased propensity for polymorphic ventricular tachyarrhythmias and sudden cardiac death in young individuals with normal cardiac morphology. …
The hereditary Long QT syndrome (LQTS) is a genetic channelopathy with variable penetrance that is associated with incr …
Drug-induced Brugada syndrome.
Yap YG, Behr ER, Camm AJ. Yap YG, et al. Europace. 2009 Aug;11(8):989-94. doi: 10.1093/europace/eup114. Epub 2009 May 29. Europace. 2009. PMID: 19482855 Review.
Patients with Brugada syndrome or suspected mutation carriers can have normal ECG recordings at other times. ...As published reports of the drug-induced Brugada sign have become increasingly prevalent, there is growing interest in the mechanisms responsible for this acquir …
Patients with Brugada syndrome or suspected mutation carriers can have normal ECG recordings at other times. ...As published reports …
Risk stratification of sudden cardiac death: a review.
Tfelt-Hansen J, Garcia R, Albert C, Merino J, Krahn A, Marijon E, Basso C, Wilde AAM, Haugaa KH. Tfelt-Hansen J, et al. Europace. 2023 Aug 25;25(8):euad203. doi: 10.1093/europace/euad203. Europace. 2023. PMID: 37622576 Free PMC article. Review.
Treatment strategies include treatment of the underlying disease with lifestyle advice and drugs and decisions to implant a primary prevention implantable cardioverter-defibrillator (ICD) and perform ablation of the ventricles and novel treatment modalities such as left cardiac s …
Treatment strategies include treatment of the underlying disease with lifestyle advice and drugs and decisions to implant a primary preventi …
Sodium channel blockers in the management of long QT syndrome types 3 and 2: A system review and meta-analysis.
Yang Y, Lv TT, Li SY, Zhang P. Yang Y, et al. J Cardiovasc Electrophysiol. 2021 Nov;32(11):3057-3067. doi: 10.1111/jce.15223. Epub 2021 Sep 1. J Cardiovasc Electrophysiol. 2021. PMID: 34427958 Review.
BACKGROUND: beta-Blockers are first-line therapy in patients with long QT syndrome (LQTS). However, beta-blockers had genotype dependent efficacy (LQT1>LQT2>LQT3). ...RESULTS: Pooled analysis of 14 studies with 213 LQTS (9 LQT1 + 63 LQT2 + 135 LQT3 + 6 …
BACKGROUND: beta-Blockers are first-line therapy in patients with long QT syndrome (LQTS). However, beta-blockers had g …
144 results