he advent of genetic testing and the implantable ardioverter-defibrillator (ICD) have so revolutionized the utlook for patients with long QT that the cardiac commuity needs to revise its thinking about long QT syndrome LQTS). This is the underlying message of a report by theridge et al. (1) in this issue of the Journal. Etheridge et al. (1) studied 128 children with LQTS. bout one-half of their...

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 neo...

Infective endocarditis in hypertrophic obstructive cardiomyopathy is uncommon. We present a case of extensive infective endocarditis in a female patient with mild hypertrophic obstructive cardiomyopathy and long QT syndrome. Upon a definite diagnosis, an urgent operation was performed successfully, and the patient had an uneventful postoperative course. The clinical features and the possible me...

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The short and long QT syndromes are inherited diseases associated with an increased risk for life-threatening arrhythmias. The first case of long QT syndrome (LQTS) was reported more than 150 years ago, and the study of this disease led to crucial advancement of our understanding of channelopathies and associated ventricular arrhythmias. Ten years ago, Gussak et al. reported four cases of idiop...

diograms were normal. There have been 2 reported cases of infants who experienced an apparent life-threatening event attributed to long QT syndrome, and there is 1 reported case of an infant with Brugada syndrome who experienced an apparent life-threatening event associated with ventricular fibrillation, but his QT interval was normal. Hence, considering the rarity of apparent life-threatening ...