Results 1 to 10 of about 7,572,448 (234)

Congenital Long QT Syndrome.

JACC: Clinical Electrophysiology, 2022
Congenital long QT syndrome (LQTS) encompasses a group of heritable conditions that are associated with cardiac repolarization dysfunction. Since its initial description in 1957, our understanding of LQTS has increased dramatically.
A. Krahn, Z. Laksman, Raymond W. Sy, P. Postema, M. Ackerman, A. Wilde, Hui Han   +6 more
semanticscholar   +3 more sources

An International, Multicentered, Evidence-Based Reappraisal of Genes Reported to Cause Congenital Long QT Syndrome

Circulation, 2020
Supplemental Digital Content is available in the text. Background: Long QT syndrome (LQTS) is the first described and most common inherited arrhythmia.
John Garcia, Arnon Adler, Emanuela Abiusi   +2 more
exaly   +2 more sources

Long QT syndrome – Bench to bedside

Heart Rhythm O2, 2021
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,
Daniela Ponce-Balbuena, PhD, Isabelle Deschênes, PhD   +1 more
doaj   +2 more sources

Prevalence of the Congenital Long-QT Syndrome

Circulation, 2009
Background— The prevalence of genetic arrhythmogenic diseases is unknown. For the long-QT syndrome (LQTS), figures ranging from 1:20 000 to 1:5000 were published, but none was based on actual data.
Marco Stramba-Badiale, Fabio Mosca, Lia Crotti   +2 more
exaly   +2 more sources

Case Report: Two cases of recurrent syncope caused by KCNH2 gene mutation in congenital long QT syndrome [PDF]

Frontiers in Cardiovascular Medicine
This study presents two cases of congenital long QT syndrome caused by KCNH2 gene mutations. It highlights the critical role of genetic testing in its diagnosis and underscores the importance of early detection and personalized treatment strategies to ...
Aihua Xing, Lifang Liu, Lifang Liu, Wenjuan Pei, Wenjuan Pei, Xiaoyan Zhang   +5 more
doaj   +2 more sources

Cryo-EM Structure of a KCNQ1/CaM Complex Reveals Insights into Congenital Long QT Syndrome

Cell, 2017
Roderick Mackinnon, Ji Sun
exaly   +2 more sources

Diagnosis, management and therapeutic strategies for congenital long QT syndrome

Heart, 2021
Congenital long QT syndrome (LQTS) is characterised by heart rate corrected QT interval prolongation and life-threatening arrhythmias, leading to syncope and sudden death.
A. Wilde, A. Amin, P. Postema
semanticscholar   +1 more source

Use of Artificial Intelligence and Deep Neural Networks in Evaluation of Patients With Electrocardiographically Concealed Long QT Syndrome From the Surface 12-Lead Electrocardiogram.

JAMA cardiology, 2021
Importance Long QT syndrome (LQTS) is characterized by prolongation of the QT interval and is associated with an increased risk of sudden cardiac death.
J. Bos, Z. Attia, D. Albert, P. Noseworthy, P. Friedman, M. Ackerman   +5 more
semanticscholar   +1 more source

In Vitro Drug Screening Using iPSC-Derived Cardiomyocytes of a Long QT-Syndrome Patient Carrying KCNQ1 & TRPM4 Dual Mutation: An Experimental Personalized Treatment

Cells, 2022
Congenital long QT syndrome is a type of inherited cardiovascular disorder characterized by prolonged QT interval. Patient often suffer from syncopal episodes, electrocardiographic abnormalities and life-threatening arrhythmia.
Feifei Wang, Yafan Han, Wanyue Sang, Lu Wang, Xiaoyan Liang, Liang Wang, Qiang Xing, Yankai Guo, Jianghua Zhang, Ling Zhang, Tuerhong Zukela, Jiasuoer Xiaokereti, Yanmei Lu, Xianhui Zhou, Baopeng Tang, Yaodong Li   +15 more
doaj   +1 more source

Suppression-Replacement KCNQ1 Gene Therapy for Type 1 Long QT Syndrome.

Circulation, 2021
Background: Type 1 long QT syndrome (LQT1) is caused by loss-of-function variants in the KCNQ1-encoded Kv7.1 potassium channel α-subunit which is essential for cardiac repolarization, providing the slow delayed rectifier current (IKs).
Steven M. Dotzler, C. S. John Kim, William A. C. Gendron, Wei Zhou, D. Ye, J. Bos, David J. Tester, Michael A. Barry, M. Ackerman   +8 more
semanticscholar   +1 more source