Inherited cardiac arrhythmias.

Nature Reviews. Disease Primers
Peter J SchwartzArthur A M Wilde

Abstract

The main inherited cardiac arrhythmias are long QT syndrome, short QT syndrome, catecholaminergic polymorphic ventricular tachycardia and Brugada syndrome. These rare diseases are often the underlying cause of sudden cardiac death in young individuals and result from mutations in several genes encoding ion channels or proteins involved in their regulation. The genetic defects lead to alterations in the ionic currents that determine the morphology and duration of the cardiac action potential, and individuals with these disorders often present with syncope or a life-threatening arrhythmic episode. The diagnosis is based on clinical presentation and history, the characteristics of the electrocardiographic recording at rest and during exercise and genetic analyses. Management relies on pharmacological therapy, mostly β-adrenergic receptor blockers (specifically, propranolol and nadolol) and sodium and transient outward current blockers (such as quinidine), or surgical interventions, including left cardiac sympathetic denervation and implantation of a cardioverter-defibrillator. All these arrhythmias are potentially life-threatening and have substantial negative effects on the quality of life of patients. Future research should focu...Continue Reading

References

Jun 1, 1976·The American Journal of Cardiology·P J SchwartzA M Brown
Jan 1, 1988·Circulation Research·S H Litovsky, C Antzelevitch
Oct 14, 1971·The New England Journal of Medicine·A J Moss, J McDonald
Apr 1, 1982·The American Journal of Cardiology·P J Schwartz, H L Stone
Aug 1, 1993·Circulation·P J SchwartzR S Crampton
Jan 15, 1996·Circulation·G X Yan, C Antzelevitch
Nov 14, 1997·Nature Genetics·E Schulze-BahrH Funke
Nov 15, 1997·Journal of the American College of Cardiology·J J de Vreede-SwagemakersH J Wellens
Jun 12, 1998·The New England Journal of Medicine·P J SchwartzP Salice
Nov 24, 1999·Mayo Clinic Proceedings·M J AckermanC J Porter
Jul 27, 2000·The New England Journal of Medicine·P J SchwartzR Bloise
Oct 31, 2001·Lancet·P J SchwartzJ Nastoli
Jan 16, 2002·Current Opinion in Cardiology·Robert Dumaine, Charles Antzelevitch
Jan 16, 2002·Current Opinion in Cardiology·Charles Antzelevitch, Wataru Shimizu
Jan 17, 2002·The New England Journal of Medicine·H V HuikuriR J Myerburg
Sep 25, 2002·European Heart Journal·P J SchwartzUNKNOWN European Society of Cardiology
Oct 24, 2002·Journal of the American College of Cardiology·Hiroshi MoritaTohru Ohe
Nov 27, 2002·European Heart Journal·A A M WildeUNKNOWN Study Group on the Molecular Basis of Arrhythmias of the European Society of Cardiology
May 9, 2003·The New England Journal of Medicine·Silvia G PrioriDonata Cappelletti
Jul 1, 1963·American Heart Journal·S Z WALSH
Jan 1, 1964·Circulation Research·J HAN, G K MOE
Oct 3, 2003·Journal of Cardiovascular Electrophysiology·Peter J SchwartzCarlo Napolitano
Dec 17, 2003·Circulation·Ramon BrugadaCharles Antzelevitch
Mar 5, 2004·The New England Journal of Medicine·Dan M Roden

❮ Previous
Next ❯

Citations

Nov 18, 2020·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·Malene BrohusPeter J Schwartz
Mar 10, 2021·European Heart Journal·Massimiliano GnecchiPeter J Schwartz
Feb 20, 2021·JACC. Clinical Electrophysiology·Anna SarconMelvin Scheinman
Apr 4, 2021·International Journal of Molecular Sciences·Aleksandra NijakMaaike Alaerts
Apr 4, 2021·International Journal of Molecular Sciences·Steffan Noe ChristiansenNiels Morling
Apr 30, 2021·Circulation·Joyce C K ManVincent M Christoffels
Apr 21, 2021·Molecular Genetics and Genomics : MGG·Maliheh Najari BeidokhtiSaquib A Lakhani
May 16, 2021·Proceedings of the National Academy of Sciences of the United States of America·Yangyang LinJianmin Cui
Jul 14, 2021·Trends in Cardiovascular Medicine·Charles Antzelevitch, Jose M Di Diego
Aug 11, 2021·Proceedings of the National Academy of Sciences of the United States of America·Iacopo GalleanoStephan Alexander Pless
Aug 8, 2021·Biochemical and Biophysical Research Communications·Elisa GiacomelliMilena Bellin
Aug 17, 2021·Journal of the American Heart Association·Laura KasakMaris Laan
Aug 17, 2021·Frontiers in Pediatrics·Georgia Sarquella-BrugadaOscar Campuzano
Sep 22, 2021·Human Genetics·Georgia Sarquella-BrugadaOscar Campuzano
Oct 13, 2021·The Journal of General Physiology·Jodene EldstromDavid Fedida
Nov 2, 2021·Frontiers in Pharmacology·Anastasia K ZaytsevaBoris S Zhorov
Oct 30, 2021·European Journal of Clinical Investigation·Amedeo TirandiLuca Liberale

❮ Previous
Next ❯

Methods Mentioned

BETA
genotyping
transgenic

Software Mentioned

CM
ClinGen

Related Concepts

Related Feeds

Anti-Arrhythmic Drug Therapies

Anti-arrhythmic drugs are used to prevent abnormal heart rhythms. These medications are used in conditions including, ventricular tachycardia, ventricular fibrillation and atrial fibrillation. Discover the latest research on anti-arrhythmic drug therapies here.

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.

Atrial Fibrillation

Atrial fibrillation is a common arrhythmia that is associated with substantial morbidity and mortality, particularly due to stroke and thromboembolism. Here is the latest research.

Related Papers

The Medical Clinics of North America
Jessica Kline, Otto Costantini
Italian Heart Journal : Official Journal of the Italian Federation of Cardiology
S Priori, Marina Cerrone
International Journal of Molecular Sciences
Anna Garcia-Elias, Begoña Benito
© 2021 Meta ULC. All rights reserved