Prolonged repolarization in long QT3 syndrome: unusual electrocardiographic findings

Raviña, Tomás; Lapuerta, J. A. Reinaldo; Brugada, Josep

doi:10.1016/s0167-5273(01)00589-7

Cited by 4 publications

(5 citation statements)

References 7 publications

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“…Gender might actually be a determinant factor in the development of lethal ventricular rhythms in BS patients as has been reported in animal and clinical studies [9,10]. The surface ECG, as previously reported, may be useful in recognising congenitally determined forms of Long-QT syndromes [11,12]; the dynamic behavior of repolarization in response to Na+- Fig. 2.…”

mentioning

confidence: 66%

Localized and dynamic repolarization alternans in Ajmaline accentuated Brugada syndrome

Raviña

Lapuerta

2006

International Journal of Cardiology

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mentioning

confidence: 66%

Localized and dynamic repolarization alternans in Ajmaline accentuated Brugada syndrome

Raviña

Lapuerta

2006

International Journal of Cardiology

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“…7 On the other hand, of six forms of long QT syndrome (long QT-1 to long QT-6) caused by multiple genetic defects, five of those genes have been identified, and four (KCNQ1, HERG, KCNE1, KCNE2) of these are related to potassium ion channels and one (SCN5A) to the sodium ion channel. [8][9][10][11][12][13] As mutations in SCN5A gene cause long QT-3 syndrome, both Brugada syndrome and the long QT-3 syndrome are two allelic diseases caused by different mutations in the SCN5A gene.…”

Section: Etiology and Geneticsmentioning

confidence: 99%

“…There is no conduction disturbance in long QT-3 syndrome, although a repolarization-dependent atrioventricular block may develop if the QT c interval is markedly prolonged. 8 A feature unique to long QT-3 syndrome is the presence of sinus bradycardia and pauses, described in about half of the patients with long QT-3 syndrome.…”

Section: Pathophysiologymentioning

confidence: 99%

Brugada and Long QT‐3 Syndromes: Two Phenotypes of the Sodium Channel Disease

Khan

Nair

2004

Noninvasive Electrocardiol

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Brugada and long QT-3 syndromes are two allelic diseases caused by different mutations in SCN5A gene inherited by an autosomal dominant pattern with variable penetrance. Both of these syndromes are ion channel diseases of the heart manifest on surface electrocardiogram by ST-segment elevation in the right precordial leads and prolonged QT(c) interval, respectively, with predilection for polymorphic ventricular tachycardia and sudden death, which may be the first manifestation of the disease. Brugada syndrome usually manifests during adulthood with male preponderance, whereas long QT3 syndrome usually manifests in teenage years, although it can also manifest in adulthood. Class IA and IC antiarrhythmic drugs increase ST-segment elevation and predilection for polymorphic ventricular tachycardia and ventricular fibrillation in Brugada syndrome, whereas these agents shorten the repolarization and QT(c) interval, and thus may be beneficial in long QT-3 syndrome. Beta-blockade also increases the ST-segment elevation in Brugada syndrome but decreases the dispersion of repolarization in long QT-3 syndrome. Mexiletine, a class IB sodium channel blocker decreases QT(c) interval as well as dispersion of repolarization in long QT-3 syndrome but has no effect on Brugada syndrome. The only effective treatment available at this time for Brugada syndrome is implantable cardioverter defibrillator, although repeated episodes of polymorphic ventricular tachycardia can be treated with isoproterenol. In symptomatic patients of long QT-3 syndrome in whom the torsade de pointes is bradycardia-dependent or pause-dependent, a pacemaker could be used to avoid bradycardia and pauses and an implantable cardioverter defibrillator is indicated where arrhythmia is not controlled with pacemaker and beta-blockade. However, the combination of new devices with pacemaker and cardioverter-defibrillator capabilities appear promising in these patients warranting further study.

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“…In Na + channel variant syndromes (LQTS3, manifest or drug-accentuated Brugada Syndrome, Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia? ), the "electrical participation" of the RV (or of its outflow tract), as observed in surface transmural ECGs, is remarkable [13,14]. The factors so far presented, specially if acting in concert, could lead to a decrease of I Ks expression in RV, outweighing, (if present in the human heart), the larger content in I K s [2], and prolonging APD and repolarization time in that area.…”

mentioning

confidence: 99%

Acquired long QT syndrome: Unequal regression of Amiodarone-induced repolarization lengthening

Raviña¹,

Raviña²,

Gutiérrez³

2008

International Journal of Cardiology

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Prolonged repolarization in long QT3 syndrome: unusual electrocardiographic findings

Cited by 4 publications

References 7 publications

Localized and dynamic repolarization alternans in Ajmaline accentuated Brugada syndrome

Localized and dynamic repolarization alternans in Ajmaline accentuated Brugada syndrome

Brugada and Long QT‐3 Syndromes: Two Phenotypes of the Sodium Channel Disease

Acquired long QT syndrome: Unequal regression of Amiodarone-induced repolarization lengthening

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