Preclinical short QT syndrome models: studying the phenotype and drug-screening.

Europace : European Pacing, Arrhythmias, and Cardiac Electrophysiology : Journal of the Working Groups on Cardiac Pacing, Arrhythmias, and Cardiac Cellular Electrophysiology of the European Society of Cardiology
Xuehui FanIbrahim El-Battrawy

Abstract

Cardiovascular diseases are the main cause of sudden cardiac death (SCD) in developed and developing countries. Inherited cardiac channelopathies are linked to 5-10% of SCDs, mainly in the young. Short QT syndrome (SQTS) is a rare inherited channelopathy, which leads to both atrial and ventricular tachyarrhythmias, syncope, and even SCD. International European Society of Cardiology guidelines include as diagnostic criteria: (i) QTc ≤ 340 ms on electrocardiogram, (ii) QTc ≤ 360 ms plus one of the follwing, an affected short QT syndrome pathogenic gene mutation, or family history of SQTS, or aborted cardiac arrest, or family history of cardiac arrest in the young. However, further evaluation of the QTc ranges seems to be required, which might be possible by assembling large short QT cohorts and considering genetic screening of the newly described pathogenic mutations. Since the mechanisms underlying the arrhythmogenesis of SQTS is unclear, optimal therapy for SQTS is still lacking. The disease is rare, unclear genotype-phenotype correlations exist in a bevy of cases and the absence of an international short QT registry limit studies on the pathophysiological mechanisms of arrhythmogenesis and therapy of SQTS. This leads to the ne...Continue Reading

References

Feb 15, 2001·Cardiology·I GussakP Bjerregaard
Dec 17, 2003·Circulation·Ramon BrugadaCharles Antzelevitch
Dec 18, 2003·Journal of Cardiovascular Electrophysiology·Rainer SchimpfMartin Borggrefe
Apr 20, 2004·Journal of the American College of Cardiology·Fiorenzo GaitaChristian Wolpert
May 26, 2004·Circulation·Chloé BellocqArthur A M Wilde
Mar 12, 2005·Circulation Research·Silvia G PrioriJosé Jalife
Jun 1, 2005·Biochemical and Biophysical Research Communications·Min XiaYihan Chen
Jul 14, 2005·Biochemical and Biophysical Research Communications·Mark J McPateJules C Hancox
Aug 20, 2005·Cardiovascular Research·Kui HongRamon Brugada
Mar 28, 2006·Circulation Journal : Official Journal of the Japanese Circulation Society·Hideki ItohKeiji Imoto
May 23, 2006·Biochemical and Biophysical Research Communications·Yasutaka TanabeYoshifusa Aizawa
Jul 18, 2006·Journal of Molecular and Cellular Cardiology·Mark J McPateJules C Hancox
Aug 12, 2008·International Journal of Cardiology·Hideki ItohMinoru Horie
Dec 18, 2008·Cellular Physiology and Biochemistry : International Journal of Experimental Cellular Physiology, Biochemistry, and Pharmacology·Morten GrunnetSoren-Peter Olesen
Mar 17, 2009·Journal of Molecular and Cellular Cardiology·Aziza El HarchiJules C Hancox
Apr 3, 2009·The Canadian Journal of Cardiology·Calum J RedpathMichael H Gollob
Aug 28, 2009·Cellular Physiology and Biochemistry : International Journal of Experimental Cellular Physiology, Biochemistry, and Pharmacology·Angelica Lopez-IzquierdoJose A Sanchez-Chapula
Mar 27, 2010·Nature Reviews. Drug Discovery·Allison D Ebert, Clive N Svendsen
Apr 20, 2010·American Journal of Physiology. Heart and Circulatory Physiology·R H KeldermannA V Panfilov
Jul 28, 2010·The New England Journal of Medicine·Alessandra MorettiKarl-Ludwig Laugwitz
Dec 7, 2010·Journal of Molecular and Cellular Cardiology·Yaxun SunGan-Xin Yan
Jan 18, 2011·Nature·Ilanit ItzhakiLior Gepstein
Feb 12, 2011·Journal of the American College of Cardiology·Michael H GollobJason D Roberts
Jul 30, 2011·Journal of the American College of Cardiology·Carla GiustettoFiorenzo Gaita
Oct 13, 2011·Journal of Molecular and Cellular Cardiology·Aziza El HarchiJules C Hancox
Feb 7, 2012·Cardiovascular Research·Ismail AdeniranHenggui Zhang
Mar 14, 2012·Pediatric Cardiology·Ralf J DirschingerDaniel Sinnecker
Apr 12, 2012·European Journal of Human Genetics : EJHG·Kui HongRamon Brugada
Apr 21, 2012·Science Translational Medicine·Ning SunJoseph C Wu
May 23, 2012·Cellular Physiology and Biochemistry : International Journal of Experimental Cellular Physiology, Biochemistry, and Pharmacology·Ulrike HenrionGuiscard Seebohm
Jul 4, 2012·Journal of the American College of Cardiology·Ilanit ItzhakiLior Gepstein
Nov 22, 2012·Nature Reviews. Molecular Cell Biology·J Keith Joung, Jeffry D Sander
Feb 27, 2013·Proceedings of the National Academy of Sciences of the United States of America·Makarand DeoSilvia G Priori
Apr 30, 2013·Cardiovascular Research·Hiroki TakanariMarcel A G van der Heyden

❮ Previous
Next ❯

Related Concepts

Related Feeds

Arrhythmia

Arrhythmias are abnormalities in heart rhythms, which can be either too fast or too slow. They can result from abnormalities of the initiation of an impulse or impulse conduction or a combination of both. Here is the latest research on arrhythmias.

Blood And Marrow Transplantation

The use of hematopoietic stem cell transplantation or blood and marrow transplantation (bmt) is on the increase worldwide. BMT is used to replace damaged or destroyed bone marrow with healthy bone marrow stem cells. Here is the latest research on bone and marrow transplantation.

Anemia

Anemia develops when your blood lacks enough healthy red blood cells. Anemia of inflammation (AI, also called anemia of chronic disease) is a common, typically normocytic, normochromic anemia that is caused by an underlying inflammatory disease. Here is the latest research on anemia.

Related Papers

The Journal of Innovations in Cardiac Rhythm Management
Srikanth Perike, Mark D McCAULEY
The Canadian Journal of Cardiology
Calum J RedpathMichael H Gollob
Pediatric Cardiology
Juan VillafanePeter S Fischbach
Journal of the American College of Cardiology
Michael H GollobJason D Roberts
Arrhythmia & Electrophysiology Review
Boris RudicMartin Borggrefe
© 2021 Meta ULC. All rights reserved