| Long QT syndrome in neonates: conduction disorders associated with HERG mutations and sinus bradycardia with KCNQ1 mutations. | |
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MedLine Citation:
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PMID: 14998624 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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OBJECTIVES: We hypothesized that neonatal long QT syndrome (LQTS) with 2:1 atrioventricular block (AVB) could be related to HERG mutations. BACKGROUND: Early onset of LQTS is rare but carries a high risk of life-threatening events such as ventricular arrhythmias and conduction disorders. There are no data on possible gene specificity. METHODS: We analyzed the characteristics and outcomes of 23 neonate probands from our LQTS population. Samples of DNA were available in 18 cases. RESULTS: Long QT syndrome was diagnosed because of corrected QT interval (QTc) prolongation (mean QTc of 558 +/- 62 ms) and neonatal bradycardia attributable to sinus bradycardia (n = 8) or 2:1 AVB (n = 15). Symptoms included syncope (n = 2), torsades de pointes (n = 7), and hemodynamic failure (n = 6). Three infants with 2:1 AVB died during the first month of life. During the neonatal period, all living patients received beta-blockers (BB) and 13 had a combination of BB and permanent cardiac pacing. Under treatment, patients remained asymptomatic, with a mean follow-up of seven years. Mutations were identified in HERG (n = 8) and KCNQ1 (n = 8), and one child had three mutations (HERG, KCNQ1, and SCN5A). Conduction disorders were associated with LQT2, whereas sinus bradycardia was associated with LQT1. CONCLUSIONS: Two-to-one AVB seems preferentially associated with HERG mutations, either isolated or combined. Long QT syndrome with relative bradycardia attributable to 2:1 AVB has a poor prognosis during the first month of life. In contrast, sinus bradycardia seems to be associated with KCNQ1 mutations, with a good short-term prognosis under BB therapy. |
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Authors:
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Jean-Marc Lupoglazoff; Isabelle Denjoy; Elisabeth Villain; Véronique Fressart; Françoise Simon; André Bozio; Myriam Berthet; Nawal Benammar; Bernard Hainque; Pascale Guicheney |
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Publication Detail:
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Type: Journal Article; Research Support, Non-U.S. Gov't |
Journal Detail:
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Title: Journal of the American College of Cardiology Volume: 43 ISSN: 0735-1097 ISO Abbreviation: J. Am. Coll. Cardiol. Publication Date: 2004 Mar |
Date Detail:
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Created Date: 2004-03-04 Completed Date: 2004-04-30 Revised Date: 2006-11-15 |
Medline Journal Info:
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Nlm Unique ID: 8301365 Medline TA: J Am Coll Cardiol Country: United States |
Other Details:
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Languages: eng Pagination: 826-30 Citation Subset: AIM; IM |
Affiliation:
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Pediatric Cardiology, Necker-Enfants-Malades (AP/HP), Paris, France. jean=marc.lupoglazo@rdb.ap-hop-paris.fr |
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| MeSH Terms | |
Descriptor/Qualifier:
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Bradycardia
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genetics* Cation Transport Proteins / genetics* Ether-A-Go-Go Potassium Channels Female Follow-Up Studies Humans Infant, Newborn KCNQ Potassium Channels KCNQ1 Potassium Channel Long QT Syndrome / genetics* Male Mutation* Potassium Channels / genetics* Potassium Channels, Voltage-Gated* |
| Chemical | |
Reg. No./Substance:
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0/Cation Transport Proteins; 0/Ether-A-Go-Go Potassium Channels; 0/KCNH6 protein, human; 0/KCNQ Potassium Channels; 0/KCNQ1 Potassium Channel; 0/KCNQ1 protein, human; 0/Potassium Channels; 0/Potassium Channels, Voltage-Gated |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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