Pharmacological approach to the treatment of long and short QT syndromes.

[1]  Martin Borggrefe,et al.  In vivo Effects of Mutant HERG K+ Channel Inhibition by Disopyramide in Patients with a Short QT‐1 Syndrome: A Pilot Study , 2007, Journal of cardiovascular electrophysiology.

[2]  R. Bai,et al.  Increasing Gap Junction Coupling Reduces Transmural Dispersion of Repolarization and Prevents Torsade de Pointes in Rabbit LQT3 Model , 2007, Journal of cardiovascular electrophysiology.

[3]  Michael J Ackerman,et al.  SCN4B-Encoded Sodium Channel &bgr;4 Subunit in Congenital Long-QT Syndrome , 2007, Circulation.

[4]  C. Antzelevitch,et al.  Cellular basis for the electrocardiographic and arrhythmic manifestations of Timothy syndrome: effects of ranolazine. , 2007, Heart rhythm.

[5]  Michel Haïssaguerre,et al.  Loss-of-Function Mutations in the Cardiac Calcium Channel Underlie a New Clinical Entity Characterized by ST-Segment Elevation, Short QT Intervals, and Sudden Cardiac Death , 2007, Circulation.

[6]  Derick R. Peterson,et al.  Long QT syndrome in adults. , 2007, Journal of the American College of Cardiology.

[7]  Michael J Ackerman,et al.  Postmortem long QT syndrome genetic testing for sudden unexplained death in the young. , 2007, Journal of the American College of Cardiology.

[8]  A. Moss,et al.  Genotype-Specific Onset of Arrhythmias in Congenital Long-QT Syndrome: Possible Therapy Implications , 2006, Circulation.

[9]  Michael J Ackerman,et al.  Mutant Caveolin-3 Induces Persistent Late Sodium Current and Is Associated With Long-QT Syndrome , 2006, Circulation.

[10]  M. Borggrefe,et al.  Short QT syndrome: clinical findings and diagnostic-therapeutic implications. , 2006, European heart journal.

[11]  Wei Zhou,et al.  Short QT syndrome: a case report and review of literature. , 2006, Resuscitation.

[12]  Michael J Ackerman,et al.  Risk of aborted cardiac arrest or sudden cardiac death during adolescence in the long-QT syndrome. , 2006, JAMA.

[13]  Jules C Hancox,et al.  Disopyramide is an effective inhibitor of mutant HERG K+ channels involved in variant 1 short QT syndrome. , 2006, Journal of molecular and cellular cardiology.

[14]  B. Knight,et al.  Verapamil decreases ventricular tachyarrhythmias in a patient with Timothy syndrome (LQT8). , 2006, Heart rhythm.

[15]  C. January,et al.  Most LQT2 Mutations Reduce Kv11.1 (hERG) Current by a Class 2 (Trafficking-Deficient) Mechanism , 2006, Circulation.

[16]  I. Gussak,et al.  Short QT syndrome--5 years of progress. , 2005, Journal of electrocardiology.

[17]  I. Gussak,et al.  Short QT Syndrome , 2005, Annals of noninvasive electrocardiology : the official journal of the International Society for Holter and Noninvasive Electrocardiology, Inc.

[18]  Jun Zhou,et al.  Novel Potent Human Ether-à-Go-Go-Related Gene (hERG) Potassium Channel Enhancers and Their in Vitro Antiarrhythmic Activity , 2005, Molecular Pharmacology.

[19]  Jonathan M. Cordeiro,et al.  Modulation of IKr inactivation by mutation N588K in KCNH2: A link to arrhythmogenesis in short QT syndrome , 2005 .

[20]  S. Priori From Genes to Cell Therapy: Molecular Medicine Meets Clinical EP , 2005, Journal of cardiovascular electrophysiology.

[21]  S. Priori,et al.  A Novel Form of Short QT Syndrome (SQT3) Is Caused by a Mutation in the KCNJ2 Gene , 2005, Circulation research.

[22]  F. Gaita,et al.  Short QT syndrome: successful prevention of sudden cardiac death in an adolescent by implantable cardioverter-defibrillator treatment for primary prophylaxis. , 2005, Heart rhythm.

[23]  Ramon Brugada,et al.  Short QT Syndrome and Atrial Fibrillation Caused by Mutation in KCNH2 , 2005, Journal of cardiovascular electrophysiology.

[24]  Jiesheng Kang,et al.  Discovery of a Small Molecule Activator of the Human Ether-a-go-go-Related Gene (HERG) Cardiac K+ Channel , 2005, Molecular Pharmacology.

[25]  I. Gussak,et al.  Short QT syndrome: mechanisms, diagnosis and treatment , 2005, Nature Clinical Practice Cardiovascular Medicine.

[26]  K. Sunagawa,et al.  Cellular and ionic mechanism for drug-induced long QT syndrome and effectiveness of verapamil. , 2005, Journal of the American College of Cardiology.

[27]  R. Brugada,et al.  Further Insights into the Effect of Quinidine in Short QT Syndrome Caused by a Mutation in HERG , 2005, Journal of cardiovascular electrophysiology.

[28]  S. Priori,et al.  Association of long QT syndrome loci and cardiac events among patients treated with beta-blockers. , 2004, JAMA.

[29]  S. Priori,et al.  CaV1.2 Calcium Channel Dysfunction Causes a Multisystem Disorder Including Arrhythmia and Autism , 2004, Cell.

[30]  S. Priori,et al.  Association of Long QT Syndrome Loci and Cardiac Events Among Patients Treated With β-Blockers , 2004 .

[31]  B. Balkau,et al.  New KCNQ1 mutations leading to haploinsufficiency in a general population; Defective trafficking of a KvLQT1 mutant. , 2004, Cardiovascular research.

[32]  R. Myerburg,et al.  Recurrent Third-Trimester Fetal Loss and Maternal Mosaicism for Long-QT Syndrome , 2004, Circulation.

[33]  A. V. van Ginneken,et al.  Mutation in the KCNQ1 Gene Leading to the Short QT-Interval Syndrome , 2004, Circulation.

[34]  D. Tester,et al.  Identification of a common genetic substrate underlying postpartum cardiac events in congenital long QT syndrome. , 2004, Heart rhythm.

[35]  Michel Haissaguerre,et al.  Short QT syndrome: pharmacological treatment. , 2004, Journal of the American College of Cardiology.

[36]  S. Priori,et al.  Left Cardiac Sympathetic Denervation in the Management of High-Risk Patients Affected by the Long-QT Syndrome , 2004, Circulation.

[37]  J. Brugada,et al.  Sudden Death Associated With Short-QT Syndrome Linked to Mutations in HERG , 2003, Circulation.

[38]  Martin Borggrefe,et al.  Congenital Short QT Syndrome and Implantable Cardioverter Defibrillator Treatment: , 2003, Journal of cardiovascular electrophysiology.

[39]  J. Mason,et al.  A new oral therapy for long QT syndrome: long-term oral potassium improves repolarization in patients with HERG mutations. , 2003, Journal of the American College of Cardiology.

[40]  C. January,et al.  Thapsigargin Selectively Rescues the Trafficking Defective LQT2 Channels G601S and F805C* , 2003, Journal of Biological Chemistry.

[41]  Calum A MacRae,et al.  Risk stratification in the long-QT syndrome. , 2003, The New England journal of medicine.

[42]  Martin Borggrefe,et al.  Short QT Syndrome: A Familial Cause of Sudden Death , 2003, Circulation.

[43]  S. Viskin Implantable Cardioverter Defibrillator in High‐Risk Long QT Syndrome Patients , 2003, Journal of cardiovascular electrophysiology.

[44]  C. Antzelevitch,et al.  Short QT Interval: ECG Phenomenon and Clinical Syndrome , 2003 .

[45]  S. Priori,et al.  Epinephrine unmasks latent mutation carriers with LQT1 form of congenital long-QT syndrome. , 2003, Journal of the American College of Cardiology.

[46]  W. Shimizu,et al.  Exercise Stress Test Amplifies Genotype-Phenotype Correlation in the LQT1 and LQT2 Forms of the Long-QT Syndrome , 2003, Circulation.

[47]  A. Gramolini,et al.  Ankyrin-B mutation causes type 4 long-QT cardiac arrhythmia and sudden cardiac death , 2003, Nature.

[48]  S. Chow,et al.  Prenatal diagnosis and treatment of fetal long QT syndrome: a case report , 2002, Prenatal diagnosis.

[49]  P. Kowey,et al.  Increasing I(Ks) corrects abnormal repolarization in rabbit models of acquired LQT2 and ventricular hypertrophy. , 2002, American journal of physiology. Heart and circulatory physiology.

[50]  S. Priori,et al.  Differential effects of beta-blockade on dispersion of repolarization in the absence and presence of sympathetic stimulation between the LQT1 and LQT2 forms of congenital long QT syndrome. , 2002, Journal of the American College of Cardiology.

[51]  C. January,et al.  Pharmacological Rescue of Human K+ Channel Long-QT2 Mutations: Human Ether-a-Go-Go-Related Gene Rescue Without Block , 2002, Circulation.

[52]  S. Priori,et al.  Gene-specific response of dynamic ventricular repolarization to sympathetic stimulation in LQT1, LQT2 and LQT3 forms of congenital long QT syndrome. , 2002, European heart journal.

[53]  W. Shen,et al.  Epinephrine-induced QT interval prolongation: a gene-specific paradoxical response in congenital long QT syndrome. , 2002, Mayo Clinic proceedings.

[54]  C Antzelevitch,et al.  Larger late sodium conductance in M cells contributes to electrical heterogeneity in canine ventricle. , 2001, American journal of physiology. Heart and circulatory physiology.

[55]  S. Subramony,et al.  Mutations in Kir2.1 Cause the Developmental and Episodic Electrical Phenotypes of Andersen's Syndrome , 2001, Cell.

[56]  S. Priori,et al.  Sympathetic stimulation produces a greater increase in both transmural and spatial dispersion of repolarization in LQT1 than LQT2 forms of congenital long QT syndrome. , 2001, Journal of the American College of Cardiology.

[57]  R. Gow Effectiveness and limitations of beta-blocker therapy in congenital long-QT syndrome. , 2001, Circulation.

[58]  J. Brugada,et al.  Idiopathic Short QT Interval:A New Clinical Syndrome? , 2001, Cardiology.

[59]  Wojciech Zareba,et al.  Spectrum of ST-T–Wave Patterns and Repolarization Parameters in Congenital Long-QT Syndrome: ECG Findings Identify Genotypes , 2000, Circulation.

[60]  S. Viskin,et al.  Rate smoothing with cardiac pacing for preventing torsade de pointes. , 2000, The American journal of cardiology.

[61]  A J Moss,et al.  Spectrum of Mutations in Long-QT Syndrome Genes: KVLQT1, HERG, SCN5A, KCNE1, and KCNE2 , 2000, Circulation.

[62]  S. Priori,et al.  The Elusive Link Between LQT3 and Brugada Syndrome: The Role of Flecainide Challenge , 2000, Circulation.

[63]  C. Antzelevitch,et al.  Effects of a K(+) channel opener to reduce transmural dispersion of repolarization and prevent torsade de pointes in LQT1, LQT2, and LQT3 models of the long-QT syndrome. , 2000, Circulation.

[64]  B. Kerem,et al.  Effects of flecainide in patients with new SCN5A mutation: mutation-specific therapy for long-QT syndrome? , 2000, Circulation.

[65]  Y Rudy,et al.  Cellular arrhythmogenic effects of congenital and acquired long-QT syndrome in the heterogeneous myocardium. , 2000, Circulation.

[66]  C. Antzelevitch,et al.  Differential effects of beta-adrenergic agonists and antagonists in LQT1, LQT2 and LQT3 models of the long QT syndrome. , 2000, Journal of the American College of Cardiology.

[67]  W. Allan,et al.  The long-QT syndrome. , 2000, The New England journal of medicine.

[68]  S. Priori,et al.  Effectiveness and limitations of beta-blocker therapy in congenital long-QT syndrome. , 2000, Circulation.

[69]  D. Tester,et al.  Swimming, a gene-specific arrhythmogenic trigger for inherited long QT syndrome. , 1999, Mayo Clinic proceedings.

[70]  C. January,et al.  Correction of Defective Protein Trafficking of a Mutant HERG Potassium Channel in Human Long QT Syndrome , 1999, The Journal of Biological Chemistry.

[71]  S. Priori,et al.  Comparison of clinical and genetic variables of cardiac events associated with loud noise versus swimming among subjects with the long QT syndrome. , 1999, The American journal of cardiology.

[72]  M. Viitasalo,et al.  Sinus node function and ventricular repolarization during exercise stress test in long QT syndrome patients with KvLQT1 and HERG potassium channel defects. , 1999, Journal of the American College of Cardiology.

[73]  A. Jervell,et al.  CONGENITAL DEAF‐MUTISM, FUNCTIONAL HEART DISEASE WITH PROLONGATION OF THE Q‐T INTERVAL, AND SUDDEN DEATH , 1999, American heart journal.

[74]  M. Keating,et al.  MiRP1 Forms IKr Potassium Channels with HERG and Is Associated with Cardiac Arrhythmia , 1999, Cell.

[75]  C. Antzelevitch,et al.  Cellular and ionic basis for T-wave alternans under long-QT conditions. , 1999, Circulation.

[76]  CharlesAntzelevitch,et al.  Cellular and Ionic Basis for T-Wave Alternans Under Long-QT Conditions , 1999 .

[77]  R. Hauer,et al.  Auditory stimuli as a trigger for arrhythmic events differentiate HERG-related (LQTS2) patients from KVLQT1-related patients (LQTS1). , 1999, Journal of the American College of Cardiology.

[78]  M. Rosen,et al.  The Controversial M Cell , 1999, Journal of cardiovascular electrophysiology.

[79]  C Antzelevitch,et al.  Cellular basis for the ECG features of the LQT1 form of the long-QT syndrome: effects of beta-adrenergic agonists and antagonists and sodium channel blockers on transmural dispersion of repolarization and torsade de pointes. , 1998, Circulation.

[80]  C Antzelevitch,et al.  Cellular basis for the normal T wave and the electrocardiographic manifestations of the long-QT syndrome. , 1998, Circulation.

[81]  E. Rosenthal,et al.  Prolongation of the QT interval and the sudden infant death syndrome. , 1998, The New England journal of medicine.

[82]  S. Priori,et al.  Influence of the genotype on the clinical course of the long-QT syndrome. International Long-QT Syndrome Registry Research Group. , 1998, The New England journal of medicine.

[83]  C. January,et al.  HERG Channel Dysfunction in Human Long QT Syndrome , 1998, The Journal of Biological Chemistry.

[84]  J. Towbin,et al.  Improvement of repolarization abnormalities by a K+ channel opener in the LQT1 form of congenital long-QT syndrome. , 1998, Circulation.

[85]  S. Viskin,et al.  Prevention of torsade de pointes in the congenital long QT syndrome: use of a pause prevention pacing algorithm , 1998, Heart.

[86]  CharlesAntzelevitch,et al.  Sodium Channel Block With Mexiletine Is Effective in Reducing Dispersion of Repolarization and Preventing Torsade de Pointes in LQT2 and LQT3 Models of the Long-QT Syndrome , 1997 .

[87]  Jacques Barhanin,et al.  KvLQT1 and IsK (minK) proteins associate to form the IKS cardiac potassium current , 1996, Nature.

[88]  M. Sanguinetti,et al.  Coassembly of KVLQT1 and minK (IsK) proteins to form cardiac IKS potassium channel , 1996, Nature.

[89]  H. Hirao,et al.  Frequency-dependent electrophysiologic properties of ventricular repolarization in patients with congenital long QT syndrome. , 1996, Journal of the American College of Cardiology.

[90]  R Lazzara,et al.  Multiple mechanisms in the long-QT syndrome. Current knowledge, gaps, and future directions. The SADS Foundation Task Force on LQTS. , 1996, Circulation.

[91]  J. Mason,et al.  Genetically defined therapy of inherited long-QT syndrome. Correction of abnormal repolarization by potassium. , 1996, Circulation.

[92]  S. Priori,et al.  Long QT syndrome patients with mutations of the SCN5A and HERG genes have differential responses to Na+ channel blockade and to increases in heart rate. Implications for gene-specific therapy. , 1995, Circulation.

[93]  A. Moss,et al.  ECG T-wave patterns in genetically distinct forms of the hereditary long QT syndrome. , 1995, Circulation.

[94]  K Shimomura,et al.  Effects of verapamil and propranolol on early afterdepolarizations and ventricular arrhythmias induced by epinephrine in congenital long QT syndrome. , 1995, Journal of the American College of Cardiology.

[95]  J E Saffitz,et al.  The Molecular Basis of Anisotropy: Role of Gap Junctions , 1995, Journal of cardiovascular electrophysiology.

[96]  M. Sanguinetti,et al.  A mechanistic link between an inherited and an acquird cardiac arrthytmia: HERG encodes the IKr potassium channel , 1995, Cell.

[97]  Arthur J Moss,et al.  SCN5A mutations associated with an inherited cardiac arrhythmia, long QT syndrome , 1995, Cell.

[98]  Rabi Tawil,et al.  Andersen's syndrome: Potassium‐sensitive periodic paralysis, ventricular ectopy, and dysmorphic features , 1994, Annals of neurology.

[99]  G. V. Van Hare,et al.  Combined use of beta-adrenergic blocking agents and long-term cardiac pacing for patients with the long QT syndrome. , 1992, Journal of the American College of Cardiology.

[100]  D. Zipes The long QT interval syndrome. A Rosetta stone for sympathetic related ventricular tachyarrhythmias. , 1991, Circulation.

[101]  A. Malliani,et al.  The long Q-T syndrome. , 1975, American heart journal.

[102]  P. Krasilnikoff,et al.  INTERMITTENT MUSCULAR WEAKNESS, EXTRASYSTOLES, AND MULTIPLE DEVELOPMENTAL ANOMALIES , 1971, Acta paediatrica Scandinavica.

[103]  A. Jervell,et al.  Congenital deaf-mutism, functional heart disease with prolongation of the Q-T interval, and sudden death , 1957 .

[104]  M. Bitner-Glindzicz,et al.  The Jervell and Lange-Nielsen syndrome: natural history, molecular basis, and clinical outcome. , 2006, Circulation.

[105]  I. Gussak Cardiac repolarization : bridging basic and clinical science , 2003 .

[106]  A. A. Armoundas,et al.  Electrical and Structural Remodeling of the Ventricular Myocardium in Disease , 2003 .

[107]  C. Antzelevitch,et al.  Cellular basis for long QT, transmural dispersion of repolarization, and torsade de pointes in the long QT syndrome. , 1999, Journal of electrocardiology.

[108]  A. Moss,et al.  The long QT syndrome: a prospective international study. , 1985, Circulation.

[109]  G. Breithardt,et al.  Life-threatening Arrhythmias Genotype-phenotype Correlation in the Long-qt Syndrome : Gene-specific Triggers for Genotype-phenotype Correlation in the Long-qt Syndrome Gene-specific Triggers for Life-threatening Arrhythmias , 2022 .