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[Congenital long QT syndrome]
- Source :
- Archives de pediatrie : organe officiel de la Societe francaise de pediatrie. 8(5)
- Publication Year :
- 2001
-
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.
Details
- Language :
- French
- ISSN :
- 0929693X
- Volume :
- 8
- Issue :
- 5
- Database :
- OpenAIRE
- Journal :
- Archives de pediatrie : organe officiel de la Societe francaise de pediatrie
- Accession number :
- edsair.pmid..........9eba51463911dd6e34a01c7895bc00cf