[New perspectives in long QT syndrome].
Long QT Syndrome (LQTS) is a cardiac channelopathy characterized by prolonged ventricular repolarization and increased risk to sudden death secondary to ventricular dysrrhythmias. Was the first cardiac channelopathy described and is probably the best understood. After a decade of the sentinel identification of ion channel mutation in LQTS, genotype-phenotype correlations have been developed along with important improvement in risk stratification and genetic guided-treatment. Genetic screening has shown that LQTS is more frequent than expected and interestingly, ethnic specific polymorphism conferring increased susceptibility to drug induced QT prolongation and torsades de pointes have been identified. A better understanding of ventricular arrhythmias as an adverse effect of ion channel binding drugs, allow the development of more safety formulas and better control of this public health problem. Progress in understanding the molecular basis of LQTS has been remarkable; eight different genes have been identified, however still 25% of patients remain genotype-negative. This article is an overview of the main LQTS knowledge developed during the last years.
Medeiros-Domingo
Argelia
A
Unidad de Biología Molecular y Medicina Genómica, Instituto de Investigaciones Biomédicas. argeliamed@yahoo.com
Iturralde-Torres
Pedro
P
Canizales-Quinteros
Samuel
S
Hernández-Cruz
Arturo
A
Tusié-Luna
M Teresa
MT
D001919:Bradycardia / Q000175:diagnosis / Q000196:embryology / Q000235:genetics; D002317:Cardiovascular Agents / Q000627:therapeutic use; D016757:Death, Sudden, Cardiac / Q000209:etiology; D004554:Electric Countershock; D005315:Fetal Diseases / Q000175:diagnosis / Q000235:genetics; D005318:Fetal Heart / Q000503:physiopathology; D015171:Ganglionectomy; D005838:Genotype; D006801:Humans; D017136:Ion Transport / Q000235:genetics; D008133:Long QT Syndrome / Q000139:chemically induced / Q000145:classification / Q000150:complications / Q000175:diagnosis / Q000196:embryology / Q000453:epidemiology / Q000235:genetics / Q000628:therapy; D010138:Pacemaker, Artificial; D010641:Phenotype; D015221:Potassium Channels / Q000235:genetics / Q000502:physiology; D011296:Prenatal Diagnosis; D015222:Sodium Channels / Q000235:genetics / Q000502:physiology; D013233:Stellate Ganglion / Q000601:surgery; D017180:Tachycardia, Ventricular / Q000209:etiology; D016171:Torsades de Pointes / Q000209:etiology
D002317:Cardiovascular Agents; D015221:Potassium Channels; D015222:Sodium Channels