The congenital long QT syndrome and implications for young athletes.

The congenital long QT syndrome (LQTS) is caused by cardiac ion channel mutations, which predispose young individuals to sudden cardiac death often related to exercise. The issue of LQTS and sports participation has received significant publicity due to reports of sudden death in young competitive athletes. This article reviews the pathophysiology, clinical characteristics, and management of LQTS in the physically active and athletic population.

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

[2]  B. Maron,et al.  Task Force 1: preparticipation screening and diagnosis of cardiovascular disease in athletes. , 2005, Journal of the American College of Cardiology.

[3]  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 .

[4]  Carlo Napolitano,et al.  Risk stratification in the long-QT syndrome. , 2003, The New England journal of medicine.

[5]  M. Sanguinetti,et al.  Compound Mutations: A Common Cause of Severe Long-QT Syndrome , 2004, Circulation.

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

[7]  Carlo Napolitano,et al.  How Really Rare Are Rare Diseases?: , 2003, Journal of cardiovascular electrophysiology.

[8]  W. Haskell,et al.  Task Force 8: classification of sports. , 2005, Journal of the American College of Cardiology.

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

[10]  A. Camm,et al.  Relation between QT and RR intervals is highly individual among healthy subjects: implications for heart rate correction of the QT interval , 2002, Heart.

[11]  B. Maron How should we screen competitive athletes for cardiovascular disease? , 2005, European heart journal.

[12]  Derick R. Peterson,et al.  Increased Risk of Arrhythmic Events in Long-QT Syndrome With Mutations in the Pore Region of the Human Ether-a-go-go–Related Gene Potassium Channel , 2002, Circulation.

[13]  R. Hauer,et al.  Genetic and molecular basis of cardiac arrhythmias: impact on clinical management part III. , 1999, Circulation.

[14]  W. Roberts,et al.  Sudden Death in Young Athletes , 1980, Circulation.

[15]  Michael J Ackerman,et al.  Recommendations for physical activity and recreational sports participation for young patients with genetic cardiovascular diseases. , 2004, Circulation.

[16]  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.

[17]  M. Eldar,et al.  Long-term follow-up of patients with long-QT syndrome treated with beta-blockers and continuous pacing. , 1999, Circulation.

[18]  Peter J. Schwartz,et al.  Diagnostic Criteria for the Long QT Syndrome An Update , 1993, Circulation.

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

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

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

[22]  E. Kaufman,et al.  Location of Mutation in the KCNQ1 and Phenotypic Presentation of Long QT Syndrome , 2003, Journal of cardiovascular electrophysiology.

[23]  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.

[24]  S. Priori,et al.  Low penetrance in the long-QT syndrome: clinical impact. , 1999, Circulation.

[25]  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.

[26]  A. Moss,et al.  The Long QT Syndrome: Prospective Longitudinal Study of 328 Families , 1991, Circulation.

[27]  R. Hauer,et al.  Genetic and Molecular Basis of Cardiac Arrhythmias: Impact on Clinical Management , 2022 .

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

[29]  R. Fagard Athlete’s heart , 2003, Cardiology clinics.

[30]  D. Corrado,et al.  Screening for hypertrophic cardiomyopathy in young athletes. , 1998, The New England journal of medicine.

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

[32]  U Ravens,et al.  Task Force on Sudden Cardiac Death of the European Society of Cardiology. , 2001, European heart journal.

[33]  S. Priori,et al.  Clinical Implications for Affected Parents and Siblings of Probands With Long-QT Syndrome , 2001, Circulation.

[34]  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.

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

[36]  C. Wren Cardiovascular pre-participation screening of young competitive athletes for prevention of sudden death: proposal for a common European protocol. , 2005, European heart journal.

[37]  D. Tester,et al.  Spectrum and Frequency of Cardiac Channel Defects in Swimming-Triggered Arrhythmia Syndromes , 2004, Circulation.

[38]  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.

[39]  M. Leppert,et al.  The spectrum of symptoms and QT intervals in carriers of the gene for the long-QT syndrome. , 1992, The New England journal of medicine.

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

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

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