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Europace : European Pacing,... Apr 2023
Topics: Humans; Long QT Syndrome; Death, Sudden, Cardiac; Risk Assessment; ERG1 Potassium Channel
PubMed: 36857538
DOI: 10.1093/europace/euad035 -
Der Anaesthesist Aug 2015The long QT syndrome is caused by a change in cardiac repolarization due to functional ion channel defects. A differentiation is made between a congenital (cLQTS) and an... (Review)
Review
The long QT syndrome is caused by a change in cardiac repolarization due to functional ion channel defects. A differentiation is made between a congenital (cLQTS) and an acquired (aLQTS) form of the disease. The disease results in the name-giving prolongation of the QT interval in the electrocardiogram and represents a predisposition for cardiac arrhythmia and sudden cardiac death. This article summarizes the current knowledge on the history, pathophysiology, clinical symptoms and therapy of cLQTS and aLQTS. This knowledge of pathophysiological features of the symptoms allows the underlying anesthesiological approach for individualized perioperative concepts for patients suffering from LQTS to be derived.
Topics: Arrhythmias, Cardiac; Death, Sudden, Cardiac; Electrocardiography; History, 20th Century; History, 21st Century; Humans; Long QT Syndrome; Perioperative Care
PubMed: 26250931
DOI: 10.1007/s00101-015-0068-9 -
Anesthesiology Jan 2005Long QT syndrome is a malfunction of cardiac ion channels resulting in impaired ventricular repolarization that can lead to a characteristic polymorphic ventricular... (Review)
Review
Long QT syndrome is a malfunction of cardiac ion channels resulting in impaired ventricular repolarization that can lead to a characteristic polymorphic ventricular tachycardia known as torsades de pointes. Stressors, by increasing sympathetic tone, and drugs can provoke torsade de pointes, leading to syncope, seizures, or sudden cardiac death in these patients. Beta blockade, implantation of cardioverter defibrillators, and left cardiac sympathetic denervation are used in the treatment of these patients. However, these treatment modalities do not guarantee the prevention of sudden cardiac death. Certain drugs, including anesthetic agents, are known to contribute to QT prolongation. After reviewing the literature the authors give recommendations for the anesthetic management of these patients in the perioperative period.
Topics: Anesthesia; Anesthetics; Humans; Long QT Syndrome
PubMed: 15618804
DOI: 10.1097/00000542-200501000-00029 -
The Journal of Emergency Medicine 1999This article reviews the clinical course of a 10-year-old child with a lifelong history of seizures and congenital deafness who presented after an episode of sudden... (Review)
Review
This article reviews the clinical course of a 10-year-old child with a lifelong history of seizures and congenital deafness who presented after an episode of sudden cardiac arrest secondary to long-QT syndrome-induced torsade de pointes. Jervell-Lange-Nielsen syndrome is a rare cardioauditory syndrome in which affected subjects are susceptible to recurrent syncope and sudden death from ventricular dysrhythmias, usually before the second decade of life. Careful evaluation of suspected subjects is important because of the variability of the QTc interval. Recent research has identified specific gene sequences that encode ion channels responsible for both prolonged QTc interval and deafness. Treatment of symptomatic cardiac disease with beta-blockers in combination with pacemakers and automated internal cardioverter defibrillators can markedly improve quality of life and suppress ventricular dysrhythmias even in the most severely affected subjects. The recent identification of gene sequences identifying some congenital long-QT syndromes may improve screening methods for affected patients and lead to potential therapeutic intervention.
Topics: Child; Deafness; Emergencies; Heart Arrest; Humans; Long QT Syndrome; Male
PubMed: 10499703
DOI: 10.1016/s0736-4679(99)00096-7 -
Vojnosanitetski Pregled Apr 2008
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Kardiologia Polska 2022
Topics: Death, Sudden, Cardiac; Defibrillators; Defibrillators, Implantable; Electric Countershock; Humans; Long QT Syndrome; Wearable Electronic Devices
PubMed: 35677993
DOI: 10.33963/KP.a2022.0142 -
Heart Rhythm Feb 2024
Topics: Humans; Long QT Syndrome; Electrocardiography
PubMed: 38296457
DOI: 10.1016/j.hrthm.2023.10.010 -
The American Journal of Medicine Jan 2002The long QT syndrome is characterized by prolongation of the corrected QT (QTc) interval on the surface electrocardiogram. It is associated with precipitation of a... (Review)
Review
The long QT syndrome is characterized by prolongation of the corrected QT (QTc) interval on the surface electrocardiogram. It is associated with precipitation of a polymorphic ventricular tachycardia, torsade de pointes, which may cause sudden death. The syndrome is a disorder of cardiac repolarization caused by the alterations in the transmembrane potassium and sodium currents. Six genetic loci for the congenital forms of the syndrome have been identified; sporadic cases occur because of spontaneous mutations. Acquired causes of the long QT syndrome include drugs, electrolyte imbalance, toxins, marked bradycardia, subarachnoid hemorrhage, stroke, myocardial ischemia, protein-sparing fasting, autonomic neuropathy, and human immunodeficiency virus disease. Clinical symptoms are the result of the precipitation of torsade de pointes and range from such minor symptoms as dizziness to syncope and sudden death. Short-term treatment is aimed at preventing the recurrences of torsade de pointes and includes intravenous magnesium and potassium administration, temporary cardiac pacing, and correction of electrolyte imbalance; rarely, intravenous isoproterenol is indicated. Long-term management includes use of beta-blockers, permanent pacemaker placement, and cardioverter-defibrillator implantation. Asymptomatic patients are treated if under the age of 40 years at the time of diagnosis.
Topics: Humans; Long QT Syndrome
PubMed: 11812408
DOI: 10.1016/s0002-9343(01)01011-7 -
Nature Clinical Practice.... Jul 2005Congenital long QT syndrome (LQTS) is a genetic disorder characterized by prolongation of the QT interval on the electrocardiogram and by life-threatening cardiac... (Review)
Review
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. The identification of several of the genes responsible for LQTS and the realization that they all encode cardiac ion-channels has represented a landmark finding. This advance has fostered novel genotype-phenotype studies that are providing unique insight into how close the relationship can be between molecular biology and clinical cardiology. LQTS represents a paradigm for sudden cardiac death. Indeed, the growing knowledge developed for LQTS is likely to provide the key to understanding the genetic propensity to sudden death in patients with more-common cardiovascular diseases. The data presented here illustrate how the treatment of LQTS is rapidly evolving toward a highly individually tailored approach on the basis of patient-specific genetic information.
Topics: Electrocardiography; Humans; Long QT Syndrome
PubMed: 16265560
DOI: 10.1038/ncpcardio0239 -
The Journal of Adolescent Health :... Aug 2015Adolescent athletes undergo preparticipation screening for long QT syndrome. Medications that are associated with QT interval prolongation are increasingly being...
PURPOSE
Adolescent athletes undergo preparticipation screening for long QT syndrome. Medications that are associated with QT interval prolongation are increasingly being prescribed to precollege students. The side effect profile of these medications during exercise is unknown. The aims of this study were to (1) measure the prevalence of medications known to be associated with QT interval prolongation taken by adolescents participating in athletics and (2) to determine the association between the use of these medications and QT interval prolongation in adolescents participating in athletics.
METHODS
Sports preparticipation exams were performed on 484 students at an urban high school. This consisted of a physical examination, 12-Lead electrocardiogram, and health history including current prescription and over-the-counter medications. Electrocardiograms were interpreted by a pediatric cardiologist using Bazett's correction formula. Descriptive statistics were used to characterize the cohort. The participants' parents consented to the preparticipation examination before history and physicals.
RESULTS
No athletes exhibited a corrected QT interval >500 ms. Borderline long QT was identified in 6.65% of the subjects. In this study, 7.64% of the subjects reported that they were taking medications associated with QT prolongation. After controlling for age and gender, medication usage was not significantly related to the presence a borderline long QT interval.
CONCLUSIONS
Although adolescent athletes are taking medications associated with QT interval prolongation, their side effect profile during exercise is unknown. Although there was no significant association between medications and borderline long QT, these data were taken from adolescent athletes at rest. The prevalence of adolescents participating in athletics and taking medications associated with QT interval prolongation was significant. There appears to be a very low risk of QT prolongation >500 ms at rest in this cohort.
Topics: Adolescent; Age Factors; Athletes; Electrocardiography; Female; Humans; Long QT Syndrome; Male; Nonprescription Drugs; Physical Examination; Prescription Drugs; Prevalence; Risk Factors; Sports
PubMed: 26206443
DOI: 10.1016/j.jadohealth.2015.04.003