Female gender: risk factor for congenital long QT-related arrhythmias

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Female gender: risk factor for congenital long QT-related arrhythmias.

Congenital long-QT syndrome (LQTS) is a genetic cardiac channelopathy in which patients exhibit delayed ventricular repolarization that appears as a prolongation of the corrected QT (QTc) interval on the electrocardiogram. More than 200 mutations have been found in 13 different genes encoding ion channels, accessory beta subunits, and regulatory proteins.Most cases of congenital LQTS are the co...

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Congenital long QT syndrome

Congenital long QT syndrome (LQTS) is a hereditary cardiac disease characterized by a prolongation of the QT interval at basal ECG and by a high risk of life-threatening arrhythmias. Disease prevalence is estimated at close to 1 in 2,500 live births. The two cardinal manifestations of LQTS are syncopal episodes, that may lead to cardiac arrest and sudden cardiac death, and electrocardiographic ...

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Efficient genotyping for congenital long QT syndrome.

WITHIN THE PAST DECADE, INVESTIGATORS HAVE uncovered the genetic basis for multiple long QT syndromes (LQTSs). These include LQT1, in which mutations in the gene KCNQ1 lead to abnormal IKs, a slow delayed-rectifier potassium current; LQT2, in which mutations in the gene KCNH2 lead to abnormal IKr, a rapid delayed-rectifier potassium current; and LQT3, in which mutations in the gene SCN5A lead t...

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Electrocardiographic risk stratification in families with congenital long QT syndrome.

AIMS The QT interval in the surface ECG is one of the most often used risk stratifiers in families with congenital long QT syndrome (LQTS). The best ECG lead for clinical management of LQTS families remains unclear. METHODS AND RESULTS The predictive power of the QTc interval in all ECG leads was studied in 200 consecutive genotyped LQTS family members to identify mutation carriers (n = 103; ...

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Assessment for congenital long QT syndrome.

A 29-year-old male presented for an evaluation of his risk for having congenital long QT syndrome. Despite being asymptomatic and having a normal QTc interval on the resting ECG, a suggestive family history was an indication for a thorough cardiac evaluation. A geneticist reviewed this workup and recommended against genetic testing. While up to 10% of affected carriers of a congenital long QT s...

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ژورنال

عنوان ژورنال: Cardiovascular Research

سال: 2012

ISSN: 0008-6363

DOI: 10.1093/cvr/cvs203