2547 Gene Mutations in AV Block-Induced TdP effects in congenital LQTS of adult patients
with complete AV block complicated by QT prolongation> 600 ms. In this issue of the …

Background: Atrioventricular block (AVB) sometimes complicates QT prolongation and
torsades de pointes (TdP). Methods and Results: The clinical and genetic background of 14 …

OBJECTIVES We studied the clinical characteristics and molecular background underlying a
severe phenotype of long QT syndrome (LQTS). BACKGROUND Mutations of cardiac ion …

Since the early description of its phenotypes, 1–3 congenital long QT syndrome (LQTS) has
been recognized as a disease entity showing prolonged QT intervals on electrocardiogram …

Reappraisal of LQTS-causing genes Page 1 www.nature.com/nrcardio long QT syndrome (lQTS)
is the most common inherited cardiac arrhy thmia (affecting 1 in 2,000 individuals) and has …

Background—It is still currently held that most patients affected by the long-QT syndrome
(LQTS) show QT interval prolongation or clinical symptoms. This is reflected by the …

The QT interval on the electrocardiogram reflects the duration of ventricular repolarization
which results from the concerted opening and closing of ion channels in the cell membrane …

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 …

In the last two decades, genetic investigations have identified many inherited arrhythmia
syndromes without the presence of structural heart disease (so-called primary electrical …

Congenital long QT syndrome (LQTS) is a group of ion channel disorders of ventricular
myocytes due to genetic mutations. The main symptoms of LQTS are pre-syncopal or …