scholarly journals Comparative Characteristics of Beta-Blockers in Patients with Congenital Long QT Syndrome

2021 ◽  
Vol 17 (3) ◽  
pp. 492-497
Author(s):  
A. Yu. Proshlyakov ◽  
P. Sh. Chomakhidze ◽  
N. A. Novikova
Keyword(s):  
Long Qt Syndrome ◽  
Cardiac Death ◽  
Antiarrhythmic Drugs ◽  
Beta Blockers ◽  
Review Article ◽  
Polymorphic Ventricular Tachycardia ◽  
Comparative Characteristic ◽  
Long Qt ◽  
Qt Syndrome ◽  
Congenital Long Qt Syndrome

Congenital long QT syndrome is a pathology that requires special attention and knowledge about the safety and effectiveness of various medications. Prolongation of the QT interval due to congenital or acquired causes is an important factor in the development of an unfavorable life forecast with the formation of an elongated QT syndrome. With an unfavorable course, patients suffer from loss of consciousness, episodes of tachycardia. Often, stable polymorphic ventricular tachycardia develops. The risk of sudden cardiac death in this pathology can vary from 0.33% to 5%. In people who have suffered an episode of cardiac arrest, and do not have a permanent prescribed antiarrhythmic therapy, the mortality rate reaches 50% within 15 years. Preventive administration of antiarrhythmic drugs is not always effective. A positive result of treatment depends on the severity of long QT syndrome and its genotype. Beta-blockers are often prescribed to patients of different ages with various cardiac pathologies, including for the prevention of arrhythmia in long QT syndrome. Beta-blockers differ in various pharmacokinetic and pharmacodynamic parameters (lipophilicity/hydrophilicity, selectivity, presence/absence of internal sympathomimetic activity), which, along with the variant of the disease genotype, can affect their effectiveness and safety in the considered pathology. This review article presents the results of major studies on the safety and effectiveness of different groups of beta blockers in various variants of long QT syndrome. The preferred beta-blockers for various genotypes of the syndrome were determined, and a comparative characteristic of beta-blockers for their safety and preventive effectiveness was given.

scholarly journals A novel KCNH2 frameshift mutation (c.46delG) associated with high risk of sudden death in a family with congenital long QT syndrome type 2

2021 ◽  
Vol 22 (1) ◽  
Author(s):  
Hyun Sok Yoo ◽  
Nancy Medina ◽  
María Alejandra von Wulffen ◽  
Natalia Ciampi ◽  
Analia Paolucci ◽  
...  
Keyword(s):  
Sudden Cardiac Death ◽  
Long Qt Syndrome ◽  
Cardiac Death ◽  
Frameshift Mutation ◽  
Alpha Subunit ◽  
Syndrome Type ◽  
Long Qt ◽  
Qt Syndrome ◽  
Congenital Long Qt Syndrome

Abstract Background The congenital long QT syndrome type 2 is caused by mutations in KCNH2 gene that encodes the alpha subunit of potassium channel Kv11.1. The carriers of the pathogenic variant of KCNH2 gene manifest a phenotype characterized by prolongation of QT interval and increased risk of sudden cardiac death due to life-threatening ventricular tachyarrhythmias. Results A family composed of 17 members with a family history of sudden death and recurrent syncopes was studied. The DNA of proband with clinical manifestations of long QT syndrome was analyzed using a massive DNA sequencer that included the following genes: KCNQ1, KCNH2, SCN5A, KCNE1, KCNE2, ANK2, KCNJ2, CACNA1, CAV3, SCN1B, SCN4B, AKAP9, SNTA1, CALM1, KCNJ5, RYR2 and TRDN. DNA sequencing of proband identified a novel pathogenic variant of KCNH2 gene produced by a heterozygous frameshift mutation c.46delG, pAsp16Thrfs*44 resulting in the synthesis of a truncated alpha subunit of the Kv11.1 ion channel. Eight family members manifested the phenotype of long QT syndrome. The study of family segregation using Sanger sequencing revealed the identical variant in several members of the family with a positive phenotype. Conclusions The clinical and genetic findings of this family demonstrate that the novel frameshift mutation causing haploinsufficiency can result in a congenital long QT syndrome with a severe phenotypic manifestation and an elevated risk of sudden cardiac death.

scholarly journals Epinephrine-Induced Polymorphic Ventricular Tachycardia in a Patient With Congenital Long QT Syndrome

2009 ◽  
Vol 39 (9) ◽  
pp. 386 ◽  
Author(s):  
Jae Hee Kim ◽  
Sun Hee Park ◽  
Kyun Hee Kim ◽  
Won Suk Choi ◽  
Jung Kyu Kang ◽  
...  
Keyword(s):  
Ventricular Tachycardia ◽  
Long Qt Syndrome ◽  
Polymorphic Ventricular Tachycardia ◽  
Long Qt ◽  
Qt Syndrome ◽  
Congenital Long Qt Syndrome

scholarly journals A Hiccup in Hiccup Management: Cardiac Arrest from Previously Undiagnosed Congenital Long QT Syndrome

2018 ◽  
Vol 2018 ◽  
pp. 1-4 ◽  
Author(s):  
Robert Hughes ◽  
Johnathan M. Sheele
Keyword(s):  
Cardiac Arrest ◽  
High Risk ◽  
Sudden Cardiac Death ◽  
Long Qt Syndrome ◽  
Cardiac Death ◽  
Long Qt ◽  
Congenital Condition ◽  
Qt Syndrome ◽  
Congenital Long Qt Syndrome

We report the case of a person who went into cardiac arrest after being given chlorpromazine for hiccups and was subsequently diagnosed with congenital Long QT Syndrome. Long QT Syndrome is an uncommon, congenital condition that carries a high risk of sudden cardiac death. Clinicians need to recognize the risk that chlorpromazine may prolong the QTc and prepare to manage potential complications.

Anesthesia for Patients with Congenital Long QT Syndrome

2005 ◽  
Vol 102 (1) ◽  
pp. 204-210 ◽  
Author(s):  
Susan J. Kies ◽  
Christina M. Pabelick ◽  
Heather A. Hurley ◽  
Roger D. White ◽  
Michael J. Ackerman
Keyword(s):  
Sudden Cardiac Death ◽  
Long Qt Syndrome ◽  
Cardiac Death ◽  
Anesthetic Management ◽  
Torsade De Pointes ◽  
Polymorphic Ventricular Tachycardia ◽  
Treatment Modalities ◽  
Beta Blockade ◽  
Long Qt ◽  
Qt Syndrome

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.

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.

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