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Genetics of long-QT syndrome

Journal of Human Genetics, 2015
Congenital long QT syndrome (LQTS) is an inherited arrhythmia syndrome characterized by a prolonged QT interval in the 12-lead ECG, torsades de pointes and not negligible prevalence of sudden cardiac death. The genetic testing plays an important role in the diagnosis of LQTS. A total of 15 genes have been reported for autosomal-dominant forms of Romano-
Yukiko, Nakano, Wataru, Shimizu
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Long QT syndromes

Current Treatment Options in Cardiovascular Medicine, 2000
The clinical phenotype of the long QT syndrome (LQTS) is quite variable, with the frequency and type of life-threatening arrhythmias influenced by the specific genotype and a spectrum of genetic and environmental factors that are not well characterized.
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The Long QT Syndrome

Heart, Lung and Circulation, 2007
The long QT syndrome (LQTS) is a genetically transmitted cardiac arrhythmia due to ion channel protein abnormalities, which affects the transport of potassium and sodium ions across the cell membrane. Patients with LQTS may present with syncope, seizures or aborted cardiac arrest.
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Congenital Long QT Syndrome

Cardiac Electrophysiology Review, 1997
Many important advances have been made in the last several years regarding the molecular genetics and physiology of the congenital long QT syndrome. These findings have significantly changed our perceptions of the clinical manifestations of this disorder, and added new diagnostic and therapeutic strategies.
G Michael, Vincent   +2 more
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Seizures and the Long-QT Syndrome

Annals of Emergency Medicine, 1996
We describe a case of idiopathic long-QT syndrome in a 4-year-old Hispanic girl. She had been seen previously at an outside hospital for possible new-onset seizure disorder but was brought to our emergency department after sustaining an unwitnessed fall. Her ECG was significant for changes consistent with long-QT syndrome.
M R, Bell, R J, Kozak
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Management of long QT syndrome

Nature Clinical Practice Cardiovascular Medicine, 2005
Congenital long QT syndrome (LQTS) is a genetic disorder characterized by prolongation of the QT interval on the electrocardiogram and by life-threatening cardiac arrhythmias, occurring especially during conditions of increased sympathetic activity. Existing therapies are very effective, but mortality is high among untreated, symptomatic individuals ...
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Domperidone and Long QT Syndrome

Current Drug Safety, 2010
Domperidone is a prokinetic agent widely prescribed in adults and children with gastrointestinal disorders. Recently several Regulatory Agencies published safety information on the risk of long QT syndrome associated with the use of domperidone.
Marco, Rossi, Giorgio, Giorgi
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Long and short QT syndrome

Herzschrittmachertherapie & Elektrophysiologie, 2006
In the past decade molecular genetic analysis has greatly expanded our knowledge about inherited arrhythmogenic syndromes. The congenital long QT syndrome (LQTS) and the recently described short QT syndrome (SQTS), with the defining characteristic of abnormal prolongation or shortening of the QTc interval on the surface electrocardiogram, are caused by
B, Borchert, T, Lawrenz, C, Stellbrink
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Significance of QT dispersion in the long QT syndrome

Progress in Cardiovascular Diseases, 2000
The long QT syndrome (LQTS) has often been considered as a model to study the abnormalities of cardiac repolarization in humans because it represents a pure electrical disease with no evidence of cardiac structural abnormalities. The arrhythmogenic potential of prolonged ventricular repolarization has been extensively studied both in experimental ...
Napolitano C   +2 more
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The beginnings of long QT syndrome

Current Opinion in Cardiology, 2015
The purpose of this study is to update the perinatal cardiologist and obstetrical care provider on the presentation and management of the fetus with long QT syndrome (LQTS).LQTS is a known cause of sudden death in childhood, adolescence and young adulthood that presents during fetal life, but is often not recognized.
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