Therapeutic approaches for Long QT syndrome type 3: an update

This editorial refers to ‘Long-term flecainide therapy in type 3 long QT syndrome’ by Chorinet al., on pages 370 –376.
Source: Europace - Category: Cardiology Source Type: research

Related Links:

lini C, Shimamoto K, Tadros R, Cadrin-Tourigny J, Duff HJ, Simpson CS, Roston TM, Wijeyeratne YD, El Hajjaji I, Yousif MD, Gula LJ, Leong-Sit P, Chavali N, Landstrom AP, Marcus GM, Dittmann S, Wilde AAM, Behr ER, Tfelt-Hansen J, Scheinman MM, Perez MV, Kaski JP, Gow RM, Drago F, Aziz PF, Abrams DJ, Gollob MH, Skinner JR, Shimizu W, Kaufman ES, Roden DM, Zareba W, Schwartz PJ, Schulze-Bahr E, Etheridge SP, Priori SG, Ackerman MJ Abstract Background: Insight into type 5 long QT syndrome (LQT5) has been limited to case reports and small family series. Improved understanding of the clinical phenotype and genetic featu...
Source: Circulation - Category: Cardiology Authors: Tags: Circulation Source Type: research
We examined frequency, clinical characteristics and AF‐related management and outcomes amongst this patient population.MethodsWe retrospectively studied consecutive probands with inherited cardiomyopathy (n=962) and inherited arrhythmia syndromes (n=195) evaluated between 2002 ‐2018.ResultsAF was observed in 5 ‐31% of patients, with the highest frequency in HCM. Age of AF onset was 45.8 ± 21.9 years in the inherited arrhythmia syndromes compared to 53.3 ± 15.3 years in the inherited cardiomyopathies, with 4 CPVT patients developing AF at median age of 20 years. Overall, 11% of patients with AF had a t r...
Source: Journal of Cardiovascular Electrophysiology - Category: Cardiology Authors: Tags: ORIGINAL ARTICLE Source Type: research
In this study, dynamical mechanisms of EAD formation in human ventricular myocytes (HVMs) were investigated using the mathematical model developed by ten Tusscher and Panfilov (Am J Physiol Heart Circ Physiol 291, 2006). We explored how the rapid (IKr) and slow (IKs) components of delayed-rectifier K+ channel currents, L-type Ca2+ channel current (ICaL), Na+/Ca2+ exchanger current (INCX), and intracellular Ca2+ handling via the sarcoplasmic reticulum (SR) contribute to initiation, termination and modulation of phase-2 EADs during pacing in relation to bifurcation phenomena in non-paced model cells. Parameter-dependent dyna...
Source: Frontiers in Physiology - Category: Physiology Source Type: research
Diagnosing long-QT syndrome (LQTS) remains challenging due to a considerable overlap in QT-interval between LQTS and healthy subjects. Characterizing T-wave morphology might improve LQTS diagnosis.
Source: Heart Rhythm - Category: Cardiology Authors: Source Type: research
ConclusionBoth mutations, KV7.1 A150T and L374H, led to loss of channel function. The degree of loss ‐of‐function may mirror the disease phenotype observed in the patients.This article is protected by copyright. All rights reserved
Source: Pacing and Clinical Electrophysiology : PACE - Category: Cardiology Authors: Tags: ELECTROPHYSIOLOGY Source Type: research
The genetics underlying familial long QT syndrome (LQTS) are among the best characterised of all of the inherited heart conditions. Cohort and registry studies have demonstrated important genotype-phenotype correlations that are now essential in guiding clinical practice of patients with the most common three genotypes; KCNQ1 (LQT type 1), KCNH2 (LQT type 2) and SCN5A (LQT type 3). However, the growing number of genes —now more than 16—is confusing, and there is much doubt as to whether many actually cause LQTS at all.
Source: Heart, Lung and Circulation - Category: Cardiology Authors: Source Type: research
Authors: Liu P, Wang L, Han D, Sun C, Xue X, Li G Abstract Cardiovascular disease (CVD) is the leading cause of morbidity and mortality in chronic kidney disease (CKD) patients. QT interval prolongation is a congenital or acquired condition that is associated with an increased risk of torsade de pointes (TdP), sudden cardiac death (SCD), and all-cause mortality in the general population. The prevalence of acquired long QT syndrome (aLQTS) is high, and various acquired conditions contribute to the prolonged QT interval in patients with CKD. More notably, the prolonged QT interval in CKD is an independent risk factor...
Source: Renal Failure - Category: Urology & Nephrology Tags: Ren Fail Source Type: research
PMID: 31884420 [PubMed - as supplied by publisher]
Source: Aging - Category: Biomedical Science Authors: Tags: Aging (Albany NY) Source Type: research
This state-of-the art review discusses sudden cardiac death (SCD) risk stratification and prevention using implantable cardioverter defibrillator (ICD) therapy and the place of catheter ablation in the major inherited cardiomyopathies and primary arrhythmic syndromes. ICD therapy protects against SCD in many inherited cardiac conditions, particularly the cardiomyopathies in advanced stages, such as hypertrophic cardiomyopathy (HCM) and arrhythmogenic right ventricular cardiomyopathy (ARVC). However, they are not usually indicated in most patients with cardiac ion channelopathies, particularly long QT syndrome, since medica...
Source: Heart, Lung and Circulation - Category: Cardiology Authors: Tags: Review Source Type: research
Abstract The cardiac potassium IKs current is carried by a channel complex formed from a-subunits encoded by KCNQ1 and b-subunits encoded by KCNE1. Deleterious mutations in either gene are associated with hereditary long QT syndrome. Interactions between the transmembrane domains of the a- and b-subunits determine the activation kinetics of IKs. A physical and functional interaction between C-termini of the proteins has also been identified that impacts deactivation rate and voltage-dependence of activation. We sought to explore the specific physical interactions between the C-termini of the subunits that confer s...
Source: American Journal of Physiology. Heart and Circulatory Physiology - Category: Physiology Authors: Tags: Am J Physiol Heart Circ Physiol Source Type: research
More News: Long QT Syndrome