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Titolo:
Effects of flecainide in patients with new SCN5A mutation - Mutation-specific therapy for long-QT syndrome?
Autore:
Benhorin, J; Taub, R; Goldmit, M; Kerem, B; Kass, RS; Windman, I; Medina, A;
Indirizzi:
Bikur Cholim Hosp, Heiden Dept Cardiol, IL-91004 Jerusalem, Israel Bikur Cholim Hosp Jerusalem Israel IL-91004 , IL-91004 Jerusalem, Israel Hebrew Univ Jerusalem, Dept Genet, IL-91904 Jerusalem, Israel Hebrew Univ Jerusalem Jerusalem Israel IL-91904 -91904 Jerusalem, Israel Columbia Univ, Coll Phys & Surg, Dept Pharmacol, New York, NY USA ColumbiaUniv New York NY USA s & Surg, Dept Pharmacol, New York, NY USA
Titolo Testata:
CIRCULATION
fascicolo: 14, volume: 101, anno: 2000,
pagine: 1698 - 1706
SICI:
0009-7322(20000411)101:14<1698:EOFIPW>2.0.ZU;2-G
Fonte:
ISI
Lingua:
ENG
Soggetto:
INHERITED CARDIAC-ARRHYTHMIA; CANINE VENTRICULAR EPICARDIUM; SODIUM-CHANNEL; GENETIC-HETEROGENEITY; MULTIPLE MECHANISMS; ALPHA-SUBUNIT; FAMILIES; LINKAGE; BLOCK;
Keywords:
long-QT syndrome; genetics; sodium (ion) channels;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Life Sciences
Citazioni:
35
Recensione:
Indirizzi per estratti:
Indirizzo: Benhorin, J Bikur Cholim Hosp, Heiden Dept Cardiol, POB 492, IL-91004 Jerusalem, Israel Bikur Cholim Hosp POB 492 Jerusalem Israel IL-91004 m, Israel
Citazione:
J. Benhorin et al., "Effects of flecainide in patients with new SCN5A mutation - Mutation-specific therapy for long-QT syndrome?", CIRCULATION, 101(14), 2000, pp. 1698-1706

Abstract

Background-Mutations in the cardiac sodium channel gene (SCN5A) can cause one variant of the congenital long-QT syndrome, The effects of some of these mutations on the alpha-subunit channel properties can be blocked by type Ib antiarrhythmic drugs. Recently, we have described a new SCN5A mutation (D1790G) that affects the channel properties in a manner suggesting that sodium blockers of the Ib type will be ineffective in carriers of this mutation. Hence, the ECG effects of flecainide-acetate, a type Ic sodium blocker, were evaluated in carriers of this mutation. Methods and Results-Eight asymptomatic mutation carriers and 5 control subjects were studied. Intravenous lidocaine was tested first in only 2 mutation carriers and had no significant effect on any ECG parameter. Flecainide significantly shortened all heart rate-corrected repolarization duration parameters only in carriers and not in control subjects: QT, shortened by 9.5% (from 517+/-45 to 468+/-36 ms, P=0.011), and the S-offset to T-onset interval shortened by 63.7% (ft om 187+/-88 to 66+/-50 ms, P=0.0092), Flecainide also normalized the marked baseline repolarization dispersion in most mutation carriers. These effects among carriers were maintained during long-term (9 to 17 months) outpatient flecainide therapy with no adverse effects. Conclusions-This report is the first to describe SCN5A mutation carriers who significantly responded to flecainide therapy yet did not respond to lidocaine, These results have important implications for long-QT allele-specific therapeutic strategies.

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Documento generato il 06/04/20 alle ore 21:42:22