A presumably benign human ether-a-go-go-related gene mutation (R176W) with a malignant primary manifestation of long QT syndrome

2011 ◽  
Vol 22 (3) ◽  
pp. 360-363 ◽  
Author(s):  
Birgit C. Donner ◽  
Christoph Marshall ◽  
Klaus G. Schmidt
Keyword(s):  
Long Qt Syndrome ◽  
Qt Interval ◽  
Clinical Phenotype ◽  
Torsade De Pointes ◽  
Holter Monitoring ◽  
Related Gene ◽  
Long Qt ◽  
Torsade De Pointes Tachycardia ◽  
Qt Syndrome ◽  
Mild Clinical Phenotype

AbstractA 12-year-old girl presented with a first prolonged syncope. She was successfully resuscitated by external defibrillation after recording torsade de pointes tachycardia. Repeated electrocardiograms and a 12-channel Holter monitoring showed an intermittent prolongation of the QT interval. Genetic analysis identified a heterozygous point mutation in the KCNH2 gene, which is thought to be associated with a rather mild clinical phenotype of the long QT syndrome.

Predicting Torsade de Pointes in Acquired Long QT Syndrome: Optimal Identification of Critical QT Interval Prolongation

Cardiology ◽  
2012 ◽  
Vol 122 (1) ◽  
pp. 3-11 ◽  
Author(s):  
John Chiladakis ◽  
Andreas Kalogeropoulos ◽  
Fani Zagkli ◽  
Nikolaos Koutsogiannis ◽  
Konstantinos Chouchoulis ◽  
...  
Keyword(s):  
Long Qt Syndrome ◽  
Qt Interval ◽  
Torsade De Pointes ◽  
Interval Prolongation ◽  
Long Qt ◽  
Acquired Long Qt Syndrome ◽  
Qt Interval Prolongation ◽  
Qt Syndrome

Video-Assisted Modified Left Sympathectomy for Long-QT Syndrome

2000 ◽  
Vol 8 (1) ◽  
pp. 52-53 ◽  
Author(s):  
Guo Xing Weng ◽  
Hang Ding ◽  
Juan Qi ◽  
Chun Xuan Xu
Keyword(s):  
Ventricular Tachycardia ◽  
Long Qt Syndrome ◽  
Qt Interval ◽  
Torsade De Pointes ◽  
Horner’S Syndrome ◽  
Horner's Syndrome ◽  
Long Qt ◽  
Video Assisted ◽  
Qt Syndrome

A 22-year-old female suffering from idiopathic long-QT syndrome complicated by frequent syncope, torsade-de-pointes-type ventricular tachycardia, and asthma, was successfully treated by video-assisted extensive left second and third thoracic sympathetic ganglionectomy, instead of left stellate and first thoracic ganglio-nectomy, to avoid postoperative Horner's syndrome. The QT interval was significantly shortened from 0.6 to 0.43 seconds four days after the surgery. It remained at 0.43 seconds during a 3-month follow-up with no recurrence of tachycardia or syncope.

scholarly journals Long QT in stunned myocardium: unrecognised cause of acquired long QT syndrome

2014 ◽  
Vol 83 (3) ◽  
pp. 250-254
Author(s):  
Jerzy Sacha
Keyword(s):  
Long Qt Syndrome ◽  
Qt Interval ◽  
Ventricular Tachyarrhythmia ◽  
Torsade De Pointes ◽  
Stunned Myocardium ◽  
Long Qt ◽  
Wave Inversion ◽  
Qt Interval Prolongation ◽  
Evolutionary Changes ◽  
Qt Syndrome

Long QT syndrome (LQTS) is a heart disorder characterized by a prolongation of the QT interval on ECG and a predisposition to ventricular tachyarrhythmias, which may lead to syncope, cardiac arrest or sudden cardiac death. This condition may be inherited or induced by external factors such as drugs, electrolyte imbalances and some acquired cardiac diseases. The review addresses LQTS caused by acute cardiac illnesses which are associated with a large amount of stunned myocardium, i.e. the reperfused myocardial infarction and the group of stress-related cardiomyopathies. In these cases, specific ECG evolutionary changes may be observed, i.e. dynamic deep T-wave inversion and QT interval prolongation which predispose to fatal polymorphic ventricular tachyarrhythmia, i.e. torsade de pointes. However, lethal arrhythmias are relatively rare in these instances and probably concern patients with an underlying predisposition to LQTS. The pathological mechanisms of both repolarization abnormalities and ventricular arrhythmias as well as the practical approach how to interpret electrocardiographic changes and identify high risk patients are discussed in this review.

scholarly journals Clinical Aspects and Case Management in a Patient with Long QT Syndrome, Probably Genetic

2019 ◽  
Vol 16 (4) ◽  
pp. 65-72
Author(s):  
Violeta Ion ◽  
Cristina Pascari ◽  
Florina Cristescu
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Long Qt Syndrome ◽  
Qt Interval ◽  
Beta Blockers ◽  
Correct Diagnosis ◽  
Torsade De Pointes ◽  
Clinical Aspects ◽  
Long Qt ◽  
Obstructive Hypertrophic Cardiomyopathy ◽  
Qt Syndrome

AbstractWe present the evaluation of a 82 years old patient, diagnosed with long QT syndrome at 75 years (QTc =550 ms) and obstructive hypertrophic cardiomyopathy from 2011, complicated with ventricular fibrillation and torsade de pointes (QTc =840ms). In this situation, it was decided to follow via electrocardiography (ECG) her first and second degree relatives (daughter - 53 years old – QTc =505 ms, granddaughter - 23 years old – QTc =448ms), and to inform them about the importance of performing the cardiac echography to exclude a possible obstructive hypertrophic cardiomyopathy, and of the genetic testing. After the implantation of the ICD (implantable cardiac defibrillator) and on treatment with beta-blockers, the patient did not repeat any arrhythmic events, but still had the same electrocardiographic aspect of long QT interval. Due to the fact that the patient had a lot of other diseases, the administration of treatment which can prolong the QT interval was avoided. Long QT syndrome represents a congenital or acquired pathology, which sometimes can be induced by some drugs, and requires a correct diagnosis in order to establish an individualized pharmaceutical or surgical treatment, and to prevent the risk of sudden death by cardiac arrhythmias.

scholarly journals Acquired Long QT Syndrome: A Review of the Literature

2020 ◽  
Vol 3 (1) ◽  
pp. 67-70
Author(s):  
Rajendram R
Keyword(s):  
Cardiac Arrest ◽  
Long Qt Syndrome ◽  
Qt Interval ◽  
Torsade De Pointes ◽  
Polymorphic Ventricular Tachycardia ◽  
Beta Blockade ◽  
Illustrative Case ◽  
Long Qt ◽  
Life Threatening ◽  
Qt Syndrome

The QT interval represents the duration of ventricular depolarization and repolarization. It is measured from the beginning of the QRS complex to the end of the T wave. Prolongation of the QT interval may be congenital or acquired. This increases the risk of polymorphic ventricular tachycardia (i.e torsades de pointes) and cardiac arrest. To increase the awareness of this life-threatening phenomenon I outline an illustrative case in which acquired prolongation of the QT interval due to electrolyte derangement and administration of ciprofloxacin resulted in cardiac arrest due to torsade de pointes. Management of a patient with a long QT syndrome includes Immediate cessation of drugs that prolong the QT interval; cardiac monitoring, serial 12 lead ECGs and transthoracic echocardiography; measurement of serum electrolytes; intravenous potassium replacement; intravenous magnesium replacement; beta-blockade. Causes of acquired prolongation of the QT interval are common in critically ill patients. It is important to recognize this and consider screening with 12 lead ECG to reduce the risk of life-threatening ventricular arrhythmias.

scholarly journals Risk of drug-induced Long QT Syndrome associated with the use of repurposed COVID-19 drugs: A systematic review

2020 ◽  
Author(s):  
Veronique Michaud ◽  
Pamela Dow ◽  
Sweilem B. Al Rihani ◽  
Malavika Deodhar ◽  
Meghan Arwood ◽  
...  
Keyword(s):  
Long Qt Syndrome ◽  
Qt Interval ◽  
Adverse Event Reporting System ◽  
Torsade De Pointes ◽  
World Health ◽  
Delayed Rectifier ◽  
Long Qt ◽  
Drug Induced ◽  
Repurposed Drugs ◽  
Qt Syndrome

ABSTRACTBackgroundThe World Health Organization first declared SARS-CoV-2 (COVID-19) a pandemic on March 11, 2020. There are currently no vaccines or therapeutic agents proven efficacious to treat COVID-19. So, whether existing approved drugs could be repurposed and used off-label for the treatment of novel COVID-19 disease is being explored.MethodsA thorough literature search was performed to gather information on the pharmacological properties and toxicity of 6 drugs (azithromycin, chloroquine, favipiravir, hydroxychloroquine, lopinavir/ritonavir, remdesivir) proposed to be repurposed to treat COVID-19. Researchers emphasized affinity of these drugs to block the rapid component of the delayed rectifier cardiac potassium current (IKr) encoded by the human ether-a-go-go gene (hERG), their propensity to prolong cardiac repolarization (QT interval) and cause torsade de pointes (TdP). Risk of drug-induced Long QT Syndrome (LQTS) for these drugs was quantified by comparing six indices used to assess such risk and by querying the U.S. Food and Drug Administration (FDA) Adverse Event Reporting System database with specific key words. Data are also provided to compare the level of risk for drug-induced LQTS by these drugs to 23 other, well-recognized, torsadogenic compounds.ResultsEstimators of LQTS risk levels indicated a very-high or high risk for all COVID-19 repurposed drugs except for azithromycin, although cases of TdP have been reported following the administration of this drug. There was an excellent agreement among the various indices used to assess risk of drug-induced LQTS for the six repurposed drugs and the 23 torsadogenic compounds.ConclusionThe risk-benefit assessment for the use of repurposed drugs to treat COVID-19 is complicated since benefits are currently anticipated, not proven. Mandatory monitoring of the QT interval shall be performed as such monitoring is possible for hospitalized patients or by the use of biodevices for outpatients initiated on these drugs.

scholarly journals Emotional stress as a cause of syncope and torsade de pointes in patients with long QT syndrome

2015 ◽  
Vol 72 (2) ◽  
pp. 192-195 ◽  
Author(s):  
Mihailo Vukmirovic ◽  
Irena Tomasevic-Vukmirovic ◽  
Lazar Angelkov ◽  
Filip Vukmirovic
Keyword(s):  
Emotional Stress ◽  
Long Qt Syndrome ◽  
Qt Interval ◽  
Beta Blockers ◽  
Torsade De Pointes ◽  
Polymorphic Ventricular Tachycardia ◽  
Risk Propensity ◽  
Long Qt ◽  
Qt Syndrome ◽  
Premature Beats

Introduction. Long QT syndrome (LQTS) is a disorder of myocardial repolarization characterized by the prolongation of QT interval and high risk propensity of torsade de pointes (TdP) that can lead to syncope, cardiac arrest and sudden death. Episodes may be provoked by various stimuli depending on the type of the condition. Case report. A 25- year-old famele patient was hospitalized due to syncope that occurred immediately after her solo concert, first time in her life. The patient studied solo singing and after intensive preparations the first solo concert was organized. Electrocardiography (ECG) on admission registered frequent ventricular premature beats (VES), followed by polymorphic ventricular tachycardia - TdP that degenerated into ventricular fibrilation (VF). After immediate cardioversion magnesium and beta-blockers were administered. TdP was registered again several times preceded by VES. The corrected QT interval (QTc) was 516 msec. For secondary prevention of sudden cardiac death, a cardioverter defibrillator was implanted, and beta-blockers continued. After a 1-year follow-up there were no recurrent episodes of TdP, and measured QTc was reduced to 484 msec. Conclusion. Patients with syncope following intensive emotional stress should be evaluated for malignant arrhythmias in the context of LQTS.

scholarly journals Genetic and Molecular Aspects of Drug-Induced QT Interval Prolongation

2021 ◽  
Vol 22 (15) ◽  
pp. 8090
Author(s):  
Daniela Baracaldo-Santamaría ◽  
Kevin Llinás-Caballero ◽  
Julián Miguel Corso-Ramirez ◽  
Carlos Martín Restrepo ◽  
Camilo Alberto Dominguez-Dominguez ◽  
...  
Keyword(s):  
Long Qt Syndrome ◽  
Qt Interval ◽  
Molecular Mechanisms ◽  
Torsade De Pointes ◽  
Individual Variability ◽  
Long Qt ◽  
Drug Induced ◽  
Qt Interval Prolongation ◽  
Qt Syndrome ◽  
The Many

Long QT syndromes can be either acquired or congenital. Drugs are one of the many etiologies that may induce acquired long QT syndrome. In fact, many drugs frequently used in the clinical setting are a known risk factor for a prolonged QT interval, thus increasing the chances of developing torsade de pointes. The molecular mechanisms involved in the prolongation of the QT interval are common to most medications. However, there is considerable inter-individual variability in drug response, thus making the application of personalized medicine a relevant aspect in long QT syndrome, in order to evaluate the risk of every individual from a pharmacogenetic standpoint.

EKG-gyöngyszem: szerzett hosszú-QT-szindróma

2018 ◽  
Vol 159 (39) ◽  
pp. 1607-1610 ◽  
Author(s):  
János Tomcsányi ◽  
Kristóf Tomcsányi
Keyword(s):  
Long Qt Syndrome ◽  
Torsade De Pointes ◽  
Long Qt ◽  
Drug Induced ◽  
Torsade De Pointes Tachycardia ◽  
Qt Syndrome

Abstract: Authors report the case of a patient with drug-induced long QT syndrome. This case highlights the importance of ECG signs of LQTS that may lead to torsade de pointes tachycardia. The patient received the QT prolonging moxifloxacine and the QT remained long even after the offending drug was discontinued. Orv Hetil. 2018; 159(39): 1607–1610.

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