Management of patients with long QT syndrome

Publication date: Available online 31 July 2017 Source:Journal of Arrhythmia Author(s): Serkan Cay, Ozcan Ozeke, Firat Ozcan, Serhat Koca, Aysenur Pac, Dursun Aras, Serkan Topaloglu
Source: Journal of Arrhythmia - Category: Cardiology Source Type: research

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In conclusion, we identified a novel hERG channel activator HW-0168 that can be used for studying the physiological role of hERG in cardiac myocytes and may be beneficial for treating long QT syndrome.
Source: Journal of Pharmacological Sciences - Category: Drugs & Pharmacology Source Type: research
This article summarizes the diseases that we have learned about, such as the long QT syndrome, Brugada syndrome, and catecholaminergic polymorphic ventricular tachycardia. The article examines the diagnosis, genetic screening of patients and their relatives, management, and referral to a specialist for further therapy. PMID: 31378327 [PubMed - in process]
Source: The Medical Clinics of North America - Category: General Medicine Authors: Tags: Med Clin North Am Source Type: research
This article summarizes the diseases that we have learned about, such as the long QT syndrome, Brugada syndrome, and catecholaminergic polymorphic ventricular tachycardia. The article examines the diagnosis, genetic screening of patients and their relatives, management, and referral to a specialist for further therapy.
Source: Medical Clinics of North America - Category: Primary Care Authors: Source Type: research
Contributors : Malou van den Boogaard ; Jan H van Weerd ; Harmen J van de Werken ; Vincent M ChristoffelsSeries Type : OtherOrganism : Mus musculusThe human ether-a-go-go-related gene KCNH2 encodes the voltage-gated potassium channel underlying IKr, a current critical for the repolarization phase of the cardiac action potential. Mutations in KCNH2 that cause a reduction of the repolarizing current can result in cardiac arrhythmias associated with long QT syndrome. Here, we investigated the regulation of this critical cardiac gene and identified multiple active enhancers in the Kcnh2 locus. An enhancer ~85 kbp downstream of...
Source: GEO: Gene Expression Omnibus - Category: Genetics & Stem Cells Tags: Other Mus musculus Source Type: research
CONGENITAL LONG QT syndrome (LQTS) is the most common of genetic arrhythmia syndromes associated with lethal ventricular arrhythmias and sudden cardiac death in an otherwise healthy young population.1 Hundreds of mutations in genes linked to LQTS have been identified. Although at least 17 genotypes of congenital LQTS have been reported to date, types 1, 2, and 3 LQTS (LQT1, LQT2, and LQT3, respectively) amount to approximately 90% of all genotype-confirmed cases.2,3 LQT1 is caused by loss-of-function mutations in KCNQ1 gene encoding α-subunit of cardiac slowly activating delayed rectifier K+ channel (IKs channel).
Source: Journal of Cardiothoracic and Vascular Anesthesia - Category: Anesthesiology Authors: Tags: Case Report Source Type: research
In this study, the non-integrational Sendai reprogramming method was used to express four Yamanaka factors and to generate induced pluripotent stem cell (iPSC) lines carrying the KCNQ1 c.1697C>A (p.S566Y) mutation from familial LQT1 patients. The patient-specific iPSC lines harbouring the c.1697C>A mutation expressed pluripotency markers and had the capacity to differentiate into three germ layers.
Source: Stem Cell Research - Category: Stem Cells Source Type: research
Authors: Kawai H, Watanabe E, Ohno S, Horie M, Ozaki Y Abstract A 14-year-old boy collapsed suddenly after a basketball game and was transported to our hospital after recovering from ventricular fibrillation by an automated external defibrillator. He had experienced loss of consciousness twice and has been examined for suspected long-QT syndrome at another hospital. The 12-lead electrocardiogram on admission revealed a prolonged QTc interval of 480 milliseconds. After the patient recovered without any sequelae, computed tomography revealed an anomalous left coronary artery arising from the opposite sinus of Valsalv...
Source: International Heart Journal - Category: Cardiology Tags: Int Heart J Source Type: research
Conclusions: AED reliably identifies the underlying lethal ventricular arrhythmias in addition to aborting SCD.What is Known:•Although infrequent in children, sudden cardiac death (SCD) is often an unexpected and tragic event. The etiology is diverse and sometimes remains unknown despite extensive investigations.•Automated external defibrillator (AED) is both therapeutic in aborting SCD and diagnostic in identifying the underlying lethal ventricular arrhythmias. However, the diagnostic aspect of AED is under-acknowledged by most medical providers.What is New:•Four of 25 patients (16%) were initially managed ...
Source: European Journal of Pediatrics - Category: Pediatrics Source Type: research
Authors: Blich M, Khoury A, Suleiman M, Lorber A, Gepstein L, Boulous M Abstract Congenital long QT syndrome (LQTS) is a cardiac channelopathy that leads to the prolongation of the QT interval. This prolongation can lead to ventricular tachyarrhythmia, syncope, and sudden cardiac death. There are various types of LQTS. Treatment of LQT1 and LQT2 is mainly based on antiadrenergic therapy. LQT3, on the other hand, is a result of a mutation of the SCN5A gene, which encodes the sodium channels. In this type, patients are sensitive to vagal stimuli and episodes tend to occur at rest. Sodium channel blocking compounds, s...
Source: International Heart Journal - Category: Cardiology Tags: Int Heart J Source Type: research
Background: The prevalence of atrial fibrillation (AF) has not been well described in young patients with inherited heart diseases such as the inherited cardiomyopathies: hypertrophic cardiomyopathy (HCM), familial dilated cardiomyopathy (DCM), arrhythmogenic right ventricular cardiomyopathy (ARVC), left ventricular non-compaction (LVNC); or inherited arrhythmia syndromes including long QT syndrome (LQTS), Brugada syndrome (BrS) or catecholaminergic polymorphic ventricular tachycardia (CPVT).
Source: Heart, Lung and Circulation - Category: Cardiology Authors: Tags: 455 Source Type: research
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