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Long QT Syndrome: Genetics and Future Perspective [PDF]

Pediatric Cardiology, 2019
Long QT syndrome (LQTS) is an inherited primary arrhythmia syndrome that may present with malignant arrhythmia and, rarely, risk of sudden death. The clinical symptoms include palpitations, syncope, and anoxic seizures secondary to ventricular arrhythmia,
Eimear Wallace, Linda Howard, Min Liu, Timothy O’Brien, Deirdre Ward, Sanbing Shen, Terence Prendiville   +6 more
openalex   +2 more sources

Impact of Drug Induced Long QT Syndrome: A Systematic Review

Journal of Clinical Medicine Research, 2018
Background Drug induced long QT syndrome is quite common in daily clinical practice but its impact is unknown. Methods PubMed and EMBASE databases (until May 2, 2017) were searched to identify studies reporting drug induced long QT syndrome and followed ...
Karuppiah Arunachalam, Seetha Lakshmanan, Abhishek Maan, Narendra Kumar, Paari Dominic   +4 more
openalex   +3 more sources

Current and future precision therapy approaches in the long QT syndrome. [PDF]

Med Genet
Nimani S, Barbieri M, Rieder M, Odening KE.   +3 more
europepmc   +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

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).
BA Steven M. Dotzler, Cs, PhD John Kim, BS William A.C. Gendron, MD Wei Zhou, MD Dan Ye, M. P. J. Martijn Bos, BS David J. Tester, PhD Michael A. Barry, M. P. Michael J. Ackerman, Ph.D Mayo Clinic, Windland   +11 more
semanticscholar   +1 more source

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.
A. Adler, V. Novelli, A. Amin, E. Abiusi, M. Care, E. Nannenberg, H. Feilotter, S. Amenta, D. Mazzà, H. Bikker, A. Sturm, John Garcia, M. Ackerman, R. E. Hershberger, M. Perez, W. Zareba, J. Ware, A. Wilde, M. Gollob   +18 more
semanticscholar   +1 more source

Transethnic Genome-Wide Association Study Provides Insights in the Genetic Architecture and Heritability of Long QT Syndrome

Circulation, 2020
Supplemental Digital Content is available in the text. Background: Long QT syndrome (LQTS) is a rare genetic disorder and a major preventable cause of sudden cardiac death in the young.
N. Lahrouchi, R. Tadros, L. Crotti, Y. Mizusawa, P. Postema, L. Beekman, R. Walsh, K. Hasegawa, J. Barc, Mark Ernsting, Kari L. Turkowski, A. Mazzanti, B. Beckmann, K. Shimamoto, Ulla-Britt Diamant, Y. Wijeyeratne, Yu Kucho, T. Robyns, T. Ishikawa, E. Arbelo, Michael Christiansen, A. Winbo, R. Jabbari, S. Lubitz, J. Steinfurt, B. Rudic, B. Loeys, M. Shoemaker, P. Weeke, R. Pfeiffer, B. Davies, A. Andorin, N. Hofman, F. Dagradi, M. Pedrazzini, D. Tester, J. Bos, G. Sarquella-Brugada, Ó. Campuzano, P. Platonov, B. Stallmeyer, S. Zumhagen, E. Nannenberg, J. Veldink, Leonard H van den Berg, A. Al-Chalabi, C. Shaw, P. Shaw, K. Morrison, P. M. Andersen, M. Müller-Nurasyid, D. Cusi, C. Barlassina, P. Galan, M. Lathrop, M. Munter, T. Werge, M. Ribasés, T. Aung, C. Khor, M. Ozaki, P. Lichtner, T. Meitinger, J. P. van Tintelen, Y. Hoedemaekers, I. Denjoy, A. Leenhardt, C. Napolitano, W. Shimizu, J. Schott, J. Gourraud, T. Makiyama, S. Ohno, H. Itoh, A. Krahn, Charles Antzelevitch, D. Roden, J. Saenen, M. Borggrefe, K. Odening, P. Ellinor, J. Tfelt-Hansen, J. Skinner, Maarten P. van den Berg, M. Olesen, J. Brugada, Ramon Brugada, N. Makita, J. Breckpot, M. Yoshinaga, E. Behr, A. Rydberg, T. Aiba, S. Kääb, S. Priori, P. Guicheney, H. Tan, C. Newton‐Cheh, M. Ackerman, P. Schwartz, E. Schulze-Bahr, V. Probst, M. Horie, A. Wilde, M. Tanck, C. Bezzina   +105 more
semanticscholar   +1 more source

QT correction using Bazett’s formula remains preferable in long QT syndrome type 1 and 2

Annals of Noninvasive Electrocardiology, 2020
The heart rate (HR) corrected QT interval (QTc) is crucial for diagnosis and risk stratification in the long QT syndrome (LQTS). Although its use has been questioned in some contexts, Bazett's formula has been applied in most diagnostic and prognostic ...
P. Dahlberg, Ulla-Britt Diamant, T. Gilljam, A. Rydberg, L. Bergfeldt   +4 more
semanticscholar   +1 more source

Reclassification of genetic variants in children with long QT syndrome

The thoracic and cardiovascular surgeon, 2020
Genes encoding cardiac ion channels or regulating proteins have been associated with the inherited form of long QT syndrome (LQTS). Complex pathophysiology and missing functional studies, however, often bedevil variant interpretation and classification ...
D. Westphal, T. Burkard, A. Moscu-Gregor, R. Gebauer, G. Hessling, C. Wolf   +5 more
semanticscholar   +1 more source