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Titolo:
Cellular and ionic mechanisms responsible for the Brugada syndrome
Autore:
Antzelevitch, C; Yan, GX;
Indirizzi:
Mason Med Res Lab, Utica, NY 13501 USA Mason Med Res Lab Utica NY USA 13501 son Med Res Lab, Utica, NY 13501 USA
Titolo Testata:
JOURNAL OF ELECTROCARDIOLOGY
, volume: 33, anno: 2000, supplemento:, S
pagine: 33 - 39
SICI:
0022-0736(2000)33:<33:CAIMRF>2.0.ZU;2-V
Fonte:
ISI
Lingua:
ENG
Soggetto:
ST-SEGMENT ELEVATION; IDIOPATHIC VENTRICULAR-FIBRILLATION; BUNDLE-BRANCH BLOCK; SUDDEN CARDIAC DEATH; PHASE-2 REENTRY; HEART-DISEASE; ELECTROPHYSIOLOGICAL RESPONSE; ELECTRICAL HETEROGENEITY; EXTRASYSTOLIC ACTIVITY; EPICARDIUM;
Keywords:
arrhythmias; inherited disease; reentry; heterogeneity; electrophysiology; quinidine; SCN5A;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Citazioni:
36
Recensione:
Indirizzi per estratti:
Indirizzo: Antzelevitch, C Mason Med Res Lab, 2150 Bleecker St, Utica, NY 13501 USA Mason Med Res Lab 2150 Bleecker St Utica NY USA 13501 USA
Citazione:
C. Antzelevitch e G.X. Yan, "Cellular and ionic mechanisms responsible for the Brugada syndrome", J ELCARDIOL, 33, 2000, pp. 33-39

Abstract

The Brugada syndrome is characterized by ST-segment elevation in the rightprecordial leads, V-1-V-3 (unrelated to ischemia or structural disease), normal QT intervals. RBBB pattern, and sudden cardiac death, particularly inmen of Asian origin. An autosomal dominant mode of inheritance with variable penetrance is generally observed. The only gene mutations thus far linked to the Brugada Syndrome appear in the alpha subunit of the gene that encodes for the cardiac sodium channel, SCN5A. An outward shift in the balance of currents contributing to phase 1 of the right ventricular action potential is thought to underline to electrocardiographic manifestation of the syndrome. Strong sodium channel block, among other modalities, can accentuate the action potential notch in right ventricular epicardial cells, eventually leading to loss of the action potential dome. This results in the development of a large dispersion of repolarization within epicardium as well as between epicardium and endocardium. providing the substrate for the development of phase 2 and cirus movement reentry, which underline VT/VF. Therapy is directed at restoring the balance of current via inhibition of the transient outward current, I-to, and/or stimulation of inward calcium using beta adrenergic agonists, among several strategies.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 01/06/20 alle ore 01:52:06