Quinidine in Short QT Syndrome: An Old Drug for a New Disease

  title={Quinidine in Short QT Syndrome: An Old Drug for a New Disease},
  author={Elizabeth S. Kaufman},
  journal={Journal of Cardiovascular Electrophysiology},
  • E. Kaufman
  • Published 1 June 2007
  • Medicine, Biology
  • Journal of Cardiovascular Electrophysiology
Congenital short QT syndrome (SQTS) is a recently described genetically heterogeneous disorder characterized by a very short QT interval and by susceptibility to atrial and ventricular fibrillation.1 Despite rapid advances in understanding the genetic bases of SQTS, much less is known about the spectrum of clinical outcomes and the most appropriate treatments for patients with this disease. Because of the high risk of sudden cardiac death in SQTS, use of the implantable cardioverter… 
Quinidine, a life-saving medication for Brugada syndrome, is inaccessible in many countries.
Modelling the effects of quinidine, disopyramide, and E-4031 on short QT syndrome variant 3 in the human ventricles.
This study substantiates a causal link between quinidine and QT interval prolongation in SQT3 Kir2.1 mutations and highlights possible pharmacological agent qu inidine for treating SQT 3 patients.
Neuromodulation of Cardiac Repolarization and Arrhythmogenesis
Increasing evidence shows that non-pharmacologic modulation of the ANS that has initially been underutilized is now one of the main therapeutic strategies in the treatment of cardiac arrhythmias.
Pro-arrhythmogenic Effects of the V141M KCNQ1 Mutation in Short QT Syndrome and Its Potential Therapeutic Targets: Insights from Modeling
V141M KCNQ1 mutation shortens ventricular APs and enhances transmural APD heterogeneity under β-adrenergic stimulation, which identified IK1 blockers as a potential antiarrhythmic drug of choice for SQTS.
Basic Cardiac Electrophysiology and Common Drug-induced Arrhythmias.
Quinidine: a valuable medication joins the list of 'endangered species'.
  • S. Viskin, C. Antzelevitch, M. Márquez, B. Belhassen
  • Medicine
    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
  • 2007
In May 2006, AstraZeneca decided to stop the production of quinidine sulfate, and the demand for the product declined considerably due to the availability of newer, more effective alternatives, whereas the implantable cardioverter defibrillator (ICD) replaced practically all antiarrhythmic drugs in the treatment of ventricular arrhythmias associated with atrial fibrillation.
Quinidine Depresses the Transmural Electrical Heterogeneity of Transient Outward Potassium Current of the Right Ventricular Outflow Tract Free Wall
There exists a robust Ito transmural electrical heterogeneity in RVOT free wall and quinidine in clinical therapeutic concentration can depress this kind of heterogeneity effectively.
Modelling conduction through the Purkinje ventricular junction and the short-QT syndrome associated with HERG mutation in the rabbit ventricles
A family of electrophysiologically detailed computer models for rabbit epicardial, midmyocardial and endocardial ventricular myocytes, as well as the rabbit Purkinje fibre cells, are developed to simulate a realistic APD dispersion during conduction through the PVJ under normal conditions and under pathological conditions of the short QT syndrome.


Short QT Syndrome
  • P. Bjerregaard, I. Gussak
  • Medicine
    Annals of noninvasive electrocardiology : the official journal of the International Society for Holter and Noninvasive Electrocardiology, Inc
  • 2005
The best form of treatment is still not known, but prevention of atrial fibrillation has been accomplished by propafenone, and an implantable cardioverter defibrillator is recommended for prevention of SCD.
Short QT syndrome: pharmacological treatment.
Sudden Death Associated With Short-QT Syndrome Linked to Mutations in HERG
The genetic basis for a new clinical entity characterized by sudden death and short-QT intervals in the ECG is described and a novel genetic and biophysical mechanism responsible for sudden death in infants, children, and young adults caused by mutations in KCNH2 is demonstrated.
Short QT Syndrome: A Familial Cause of Sudden Death
The short QT syndrome is characterized by familial sudden death, short refractory periods, and inducible ventricular fibrillation, which is related to a high risk of sudden death in young, otherwise healthy subjects.
Mutation in the KCNQ1 Gene Leading to the Short QT-Interval Syndrome
It is demonstrated that the electrocardiographic short QT-interval syndrome is genetically heterogeneous and can also be caused by mutation in the KCNQ1 gene, which is linked to gain-of-function mutation in KCNH2.
Idiopathic Short QT Interval:A New Clinical Syndrome?
This report describes three members of one family demonstrating this ECG phenomenon, associated in the 17-year-old with several episodes of paroxysmal atrial fibrillation requiring electrical cardioversion, and considers the possible arrhythmogenic potential of the short QTI.
Short QT Syndrome and Atrial Fibrillation Caused by Mutation in KCNH2
A genetic basis has been identified linking the disease to mutations in KCNH2 in the familial forms and a mutation in KCNQ1 in a sporadic form of the disease.