title: [Congenital long QT syndrome].
abstract: Long QT syndrome (LQTS) is a clinically and genetically heterogenous syndrome characterized by a lengthening of the QT interval on the surface ECG and a propensity to severe ventricular arrhythmias such as torsades de pointes and ventricular fibrillation, leading eventually to syncope and sudden death. This rare syndrome with a mendelian inheritance occurs in subjects with otherwise normal cardiac morphological examination. The potentially severe prognosis justifies a presymptomatic diagnosis. The genetic nature of the disease has been confirmed with the identification of at least six loci and five genes. This syndrome is a perfect illustration of an adrenergic-induced ventricular arrhythmia. The first-line treatment is a beta-blocking agent for all symptomatic patients. In addition, a number of drugs known to lengthen ventricular repolarization must be prohibited. In case of suspicion of LQTS, all family members should be tested both clinically with a surface ECG and genetically in order to diagnose presymptomatic patients.
authors.affiliation: Service de cardiologie infantile, hôpital Robert-Debré, 48, boulevard Sérurier, 75019 Paris, France. jean-marc.lupoglazof@rdb.ap-hop-paris.fr
authors.forename: J M
authors.initials: JM
authors.lastname: Lupoglazoff
authors.forename: I
authors.initials: I
authors.lastname: Denjoy
authors.forename: P
authors.initials: P
authors.lastname: Guicheney
authors.forename: A
authors.initials: A
authors.lastname: Casasoprana
authors.forename: P
authors.initials: P
authors.lastname: Coumel
mesh_terms: D000319:Adrenergic beta-Antagonists / Q000627:therapeutic use; D016757:Death, Sudden, Cardiac; D004562:Electrocardiography; D005192:Family Health; D006801:Humans; D008133:Long QT Syndrome / Q000175:diagnosis / Q000235:genetics / Q000473:pathology*; D011379:Prognosis; D016171:Torsades de Pointes / Q000209:etiology; D014693:Ventricular Fibrillation / Q000209:etiology*
chemical_list: D000319:Adrenergic beta-Antagonists