Short Qt Syndrome

A cardiac CHANNELOPATHY and arrhythmia associated with a predisposition to ATRIAL FIBRILLATION and SUDDEN CARDIAC DEATH. Patients have a structurally normal heart, but electrocardiography (ECG) exhibits abbreviated QTc (Bazett's corrected QT) intervals of less than 360 ms. May be caused by autosomal dominant mutations in the KCNH2 gene. OMIM: 609620

Networked: 131 relevant articles (7 outcomes, 12 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Long QT Syndrome
2.	Brugada Syndrome
3.	Sudden Death
4.	Ventricular Tachycardia
5.	Ventricular Fibrillation

Experts

1.	Borggrefe, Martin: 17 articles (01/2021 - 12/2003)
2.	Schimpf, Rainer: 13 articles (10/2019 - 12/2003)
3.	Wolpert, Christian: 13 articles (10/2019 - 12/2003)
4.	Zhang, Henggui: 11 articles (07/2019 - 11/2009)
5.	Giustetto, Carla: 11 articles (10/2015 - 12/2003)
6.	Hancox, Jules C: 10 articles (01/2018 - 09/2006)
7.	Gaita, Fiorenzo: 10 articles (10/2015 - 04/2004)
8.	Akin, Ibrahim: 8 articles (04/2022 - 01/2018)
9.	El-Battrawy, Ibrahim: 8 articles (04/2022 - 01/2018)
10.	Cyganek, Lukas: 6 articles (04/2022 - 01/2018)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Short Qt Syndrome:

1.	Quinidine (Chinidin)FDA LinkGeneric 01/01/2005 - "The principal aim of this study was to assess the efficacy of quinidine in suppressing IKr in vitro and in modulating the rate dependence of the QT interval in the "SQT1" form of the short QT syndrome. " 07/01/2019 - "Pharmacotherapeutic Effects of Quinidine on Short QT Syndrome by Using Purkinje-Ventricle Model: A Simulation Study." 08/01/2019 - "Electrical storm in an acquired short QT syndrome successfully treated with quinidine." 06/01/2007 - "Quinidine in short QT syndrome: an old drug for a new disease." 06/01/2007 - "Reduction of dispersion of repolarization and prolongation of postrepolarization refractoriness explain the antiarrhythmic effects of quinidine in a model of short QT syndrome."
2.	Anti-Arrhythmia AgentsIBA 04/21/2004 - "The purpose of this study was to evaluate the efficacy of various antiarrhythmic drugs at prolonging the QT interval into the normal range and preventing ventricular arrhythmias in patients with short QT syndrome. " 01/01/2021 - "Effects of Antiarrhythmic Drugs on hERG Gating in Human-Induced Pluripotent Stem Cell-Derived Cardiomyocytes From a Patient With Short QT Syndrome Type 1." 01/01/2019 - "Impact of Antiarrhythmic Drugs on the Outcome of Short QT Syndrome." 11/01/2008 - "Pharmacology of the short QT syndrome N588K-hERG K+ channel mutation: differential impact on selected class I and class III antiarrhythmic drugs." 03/01/2018 - "In this study we established 3 different models to assess the potential anti-arrhythmic effects of TMCC on type 2 short QT syndrome (SQT2). "
3.	RanolazineFDA Link 09/01/2014 - "The N588K mutation is responsible for the SQT1 variant of short QT syndrome and our data suggest that ranolazine is unlikely to be effective against IKr/hERG in SQT1 patients. " 10/01/2016 - "Considering Ranolazine as a Potential Treatment for K+ Channel Linked Short QT Syndrome." 10/01/2016 - "Considering Ranolazine as a Potential Treatment for K+ Channel Linked Short QT Syndrome." 10/01/2016 - "The aim of this study was to assess the electrophysiologic effects of ranolazine and to compare its effects to vernakalant in an experimental whole-heart model of short-QT syndrome. " 10/01/2016 - "Ranolazine and Vernakalant Prevent Ventricular Arrhythmias in an Experimental Whole-Heart Model of Short QT Syndrome."
4.	IvabradineIBA 01/01/2017 - "Could ivabradine be a new treatment for the short QT syndrome?" 09/01/2017 - "In the present study ivabradine reversed the electrophysiologic effects of pharmacologically simulated short-QT syndrome. " 09/01/2017 - "The aim of the present study was to assess the electrophysiologic effects of ivabradine in an experimental model of short-QT-syndrome. " 09/01/2017 - "Antiarrhythmic properties of ivabradine in an experimental model of Short-QT- Syndrome."
5.	Disopyramide (Norpace)FDA LinkGeneric 01/01/2020 - "Ionic Mechanisms of Disopyramide Prolonging Action Potential Duration in Human-Induced Pluripotent Stem Cell-Derived Cardiomyocytes From a Patient With Short QT Syndrome Type 1." 09/01/2006 - "Disopyramide is an effective inhibitor of mutant HERG K+ channels involved in variant 1 short QT syndrome." 09/01/2006 - "Disopyramide: although potentially life-threatening in the setting of long QT, could it be life-saving in short QT syndrome?" 01/01/2017 - "Computational Analysis of the Mode of Action of Disopyramide and Quinidine on hERG-Linked Short QT Syndrome in Human Ventricles." 09/01/2008 - "Nifekalant and disopyramide in a patient with short QT syndrome: evaluation of pharmacological effects and electrophysiological properties."
6.	Ion Channels (Ion Channel)IBA 10/01/2013 - "β-blockers in congenital short-QT syndrome as ion channel blockers." 07/01/2008 - "Short-QT syndrome has been recently recognized as a genetic ion channel dysfunction leading to an abbreviation of action potential and a potential substrate for arrhythmias. " 05/01/2007 - "To date, different mutations in genes encoding for cardiac ion channels (KCNH2, KCNQ1, and KCNJ2) have been identified to cause the short QT syndrome. " 10/01/2006 - "The short QT syndrome has been recently recognised as a genetic ion channel dysfunction. " 07/01/2005 - "To date, three different mutations in genes encoding cardiac ion channels (KCNH2, KCNQ1 and KCNJ2) have been identified as causing short QT syndrome. "
7.	Carnitine (L-Carnitine)FDA LinkGeneric 01/01/2016 - "The purpose of this study was to confirm a relationship between a short QT syndrome and carnitine deficiency. " 11/10/2023 - "The whole QTc was prolonged, and no patient reached the criterion of short QT syndrome with carnitine supplementation. " 01/01/2016 - "To confirm the relationship between carnitine, short QT syndrome, and arrhythmias, we used a mouse model of carnitine deficiency induced by long-term subcutaneous perfusion of MET88. " 01/01/2016 - "Carnitine deficiency induces a short QT syndrome." 01/01/2019 - "Her brother was subsequently diagnosed clinically with short QT syndrome, received an implantable defibrillator, and was then found to carry the same pathogenic homozygous mutation and critically low levels of carnitine. "
8.	Amiodarone (Amiodarona)FDA LinkGeneric 06/07/2017 - "Effects of amiodarone on short QT syndrome variant 3 in human ventricles: a simulation study." 07/01/2017 - "Modeling the effects of amiodarone on short QT syndrome variant 2 in the human ventricles."
9.	vernakalantIBA 04/09/2022 - "Antiarrhythmic Effects of Vernakalant in Human-Induced Pluripotent Stem Cell-Derived Cardiomyocytes from a Patient with Short QT Syndrome Type 1." 10/01/2016 - "The aim of this study was to assess the electrophysiologic effects of ranolazine and to compare its effects to vernakalant in an experimental whole-heart model of short-QT syndrome. " 10/01/2016 - "Ranolazine and Vernakalant Prevent Ventricular Arrhythmias in an Experimental Whole-Heart Model of Short QT Syndrome."
10.	Doxepin (Mareen)FDA LinkGeneric 08/01/2007 - "Notably, this study also establishes doxepin as an effective inhibitor of mutant (N588K) HERG channels responsible for variant 1 of the short QT syndrome."

Therapies and Procedures

1.	Implantable Defibrillators (Implantable Cardioverter-Defibrillator) 10/01/2019 - "Long-term follow-up of implantable cardioverter-defibrillators in Short QT syndrome." 01/01/2019 - "We report implantable cardioverter defibrillator (ICD) generator replacement guided by T wave safety margin (TWSM) in a short QT syndrome patient with T wave oversensing (TWOS). " 01/01/2019 - "Implantable cardioverter defibrillator replacement guided by T wave safety margin in a short QT syndrome patient." 12/01/2003 - "Inappropriate implantable cardioverter defibrillator therapy in the short QT syndrome: old problem in a new disease." 01/31/2007 - "As of today, automatic implantable defibrillator is the only effective treatment for the prevention of sudden death in the short QT syndrome."
2.	Therapeutics 01/01/2016 - "Short QT syndrome carries the risk for inappropriate therapies using transvenous ICD because of overdetection of the ample T wave. " 06/01/2006 - "Targeted therapy for short QT syndrome." 08/15/2005 - "However, ICD therapy in patients with a short QT syndrome has an increased risk for inappropriate shock therapies due to possible T wave oversensing. " 12/01/2003 - "Inappropriate implantable cardioverter defibrillator therapy in the short QT syndrome: old problem in a new disease." 10/01/2006 - "Clinical presentation, occurrence of sudden infant death, and results of the available therapies in the largest group of patients with short QT syndrome (SQTS), studied so far, are reported. "
3.	Catheter Ablation 05/01/2019 - "Successful radiofrequency catheter ablation of a premature ventricular contraction triggering ventricular fibrillation in a patient with short QT syndrome."
4.	Cardiopulmonary Resuscitation (CPR) 10/08/2016 - "Bystander Cardio Pulmonary Resuscitation Saves Life in a Patient with Short QT Syndrome."
5.	Electric Countershock (Cardioversion) 01/01/2009 - "[Pronounced shortening of QT-interval (acquired short QT syndrome) after transthoracic cardioversion in a patient with acute coronary syndrome hemodynamically unstable ventricular tachycardia]."