scholarly journals GENETIC REASONS OF SUDDEN CARDIAC DEATH

2017 ◽  
Vol 19 (2) ◽  
pp. 15-22
Author(s):  
S N Kolyubaeva
Keyword(s):  
Ventricular Tachycardia ◽  
Sudden Cardiac Death ◽  
Long Qt Syndrome ◽  
Brugada Syndrome ◽  
Cardiac Death ◽  
Polymorphic Ventricular Tachycardia ◽  
Normal Heart ◽  
Short Qt Syndrome ◽  
Long Qt ◽  
Qt Syndrome

The review presents the recent data on genetic reasons of sudden cardiac death. Mutations discuss in gens associated with sudden cardiac death. Channalopathies, such as Brugada syndrome, long QT syndrome, short QT syndrome and catecholaminergic polymorphic ventricular tachycardia are characterized by arrhythmias in normal heart resulting from genetic anomalies in ion channels

Monogenec Arrhythmic Syndromes: From Molecular and Genetic Aspects to Bedside

Acta Naturae ◽  
2016 ◽  
Vol 8 (2) ◽  
pp. 62-74 ◽  
Author(s):  
E. Z. Golukhova ◽  
O. I. Gromova ◽  
R. A. Shomahov ◽  
N. I. Bulaeva ◽  
L. A. Bockeria
Keyword(s):  
Ventricular Tachycardia ◽  
Sudden Cardiac Death ◽  
Ventricular Fibrillation ◽  
Long Qt Syndrome ◽  
Cardiac Death ◽  
Heart Rhythm ◽  
The United States ◽  
Polymorphic Ventricular Tachycardia ◽  
Long Qt ◽  
Qt Syndrome

The abrupt cessation of effective cardiac function that is generally due to heart rhythm disorders can cause sudden and unexpected death at any age and is referred to as a syndrome called sudden cardiac death (SCD). Annually, about 400,000 cases of SCD occur in the United States alone. Less than 5% of the resuscitation techniques are effective. The prevalence of SCD in a population rises with age according to the prevalence of coronary artery disease, which is the most common cause of sudden cardiac arrest. However, there is a peak in SCD incidence for the age below 5 years, which is equal to 17 cases per 100,000 of the population. This peak is due to congenital monogenic arrhythmic canalopathies. Despite their relative rarity, these cases are obviously the most tragic. The immediate causes, or mechanisms, of SCD are comprehensive. Generally, it is arrhythmic death due to ventricular tachyarrythmias - sustained ventricular tachycardia (VT) or ventricular fibrillation (VF). Bradyarrhythmias and pulseless electrical activity account for no more than 40% of all registered cardiac arrests, and they are more often the outcome of the abovementioned arrhythmias. Our current understanding of the mechanisms responsible for SCD has emerged from decades of basic science investigation into the normal electrophysiology of the heart, the molecular physiology of cardiac ion channels, the fundamental cellular and tissue events associated with cardiac arrhythmias, and the molecular genetics of monogenic disorders of the heart rhythm (for example, the long QT syndrome). This review presents an overview of the molecular and genetic basis of SCD in the long QT syndrome, Brugada syndrome, short QT syndrome, catecholaminergic polymorphic ventricular tachycardia and idiopathic ventricular fibrillation, and arrhythmogenic right ventricular dysplasia, and sudden cardiac death prevention strategies by modern techniques (including implantable cardioverter-defibrillator).

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.

Risk stratification for sudden cardiac death in primary electrical disorders

ESC CardioMed ◽  
2018 ◽  
pp. 2322-2327
Author(s):  
Peter J. Schwartz ◽  
Lia Crotti
Keyword(s):  
Ventricular Tachycardia ◽  
High Risk ◽  
Sudden Cardiac Death ◽  
Risk Stratification ◽  
Cardiac Death ◽  
Low Risk ◽  
Polymorphic Ventricular Tachycardia ◽  
Long Qt ◽  
Risk Patients ◽  
Qt Syndrome

Risk stratification is extremely important for primary electrical diseases because the low-risk patients are likely to remain asymptomatic through life whereas the high-risk patients often die suddenly, unless properly treated. This chapter addresses what is currently known about risk stratification for the long QT syndrome, the short QT syndrome, catecholaminergic polymorphic ventricular tachycardia, and Brugada syndrome.

scholarly journals Inherited heart rhythm and conduction disorders in children with infectious diseases

2020 ◽  
pp. 126-133
Author(s):  
S. N. Chuprova ◽  
E. P. Rudneva ◽  
Yu. V. Lobzin
Keyword(s):  
Sudden Cardiac Death ◽  
Body Temperature ◽  
Infectious Diseases ◽  
Long Qt Syndrome ◽  
Cardiac Arrhythmias ◽  
Brugada Syndrome ◽  
Cardiac Death ◽  
Long Qt ◽  
Qt Syndrome ◽  
Inherited Arrhythmias

Introduction. One of the causes of sudden cardiac death in children is inherited arrhythmias. In view of the links between the increase in body temperature and the manifestation of some inherited cardiac arrhythmias (including typical electrocardiographic changes), the frequency of inherited cardiac arrhythmias in children with infectious diseases have been analyzed.The relevance of the study: is initiated by the necessity of timely diagnosis of inherited cardiac arrhythmias and conduction in children in order to prevent sudden cardiac death in them.The purpose of the study: to determine the frequency of inherited arrhythmias in children with infectious diseases based on clinical and electrocardiographic analysis.Materials and methods: 3584 electrocardiograms (ECGs) of children with infectious diseases (average age 8.5 ± 5.3 years old; boys – 57.5%, girls – 42.5%) hospitalized in the Pediatric Research and Clinical Center for Infectious Diseases were analyzed. Patients with changes in the ECGs were given additional examination depending on the intended diagnosis (inherited arrhythmias): 24-Hour Holter ECG monitoring, stress test, echocardiography. The family history was also clarified, and the parents’ ECG was analyzed.Results and conclusions. ECG changes, which are typical for Brugada syndrome (type 1), were detected in two children (0.05%) at first. Long QT syndrome was also detected in two children (0,05%). Mutations in the SCN5A gene were identified in children with Brugada syndrome, and in the KCNQ1 gene with long QT syndrome. An episode of monomorphic ventricular tachycardia was recorded at night in a 5-year-old girl with atrioventricular block 1 degree, hypoadaptation of the QT interval with repeated Holter ECG monitoring during sleep. Cases of life-threatening ventricular arrhythmias have previously been described in the literature in patients with Brugada syndrome. An increase in body temperature leads to disruption of the sodium ion channels which underlie the development of this syndrome, thereby, on the one hand, increasing the risk of life-threatening arrhythmias and sudden cardiac death, on the other hand, to the clinical manifestation of the disease, allowing the diagnosis to be made in time. In the cases of long QT syndrome, in our study, the increase in the corrected QT interval (QTc) is most likely due to a change in heart rate rather than a direct effect of an increase in body temperature on the ion channels.

scholarly journals Impact of Dietary Factors on Brugada Syndrome and Long QT Syndrome

Nutrients ◽  
2021 ◽  
Vol 13 (8) ◽  
pp. 2482
Author(s):  
Sara D’Imperio ◽  
Michelle M. Monasky ◽  
Emanuele Micaglio ◽  
Gabriele Negro ◽  
Carlo Pappone
Keyword(s):  
Risk Factors ◽  
Sudden Cardiac Death ◽  
Long Qt Syndrome ◽  
Cardiac Arrhythmias ◽  
Brugada Syndrome ◽  
Cardiac Death ◽  
Dietary Factors ◽  
Long Qt ◽  
Qt Syndrome ◽  
The Relationship

A healthy regime is fundamental for the prevention of cardiovascular diseases (CVD). In inherited channelopathies, such as Brugada syndrome (BrS) and Long QT syndrome (LQTS), unfortunately, sudden cardiac death could be the first sign for patients affected by these syndromes. Several known factors are used to stratify the risk of developing cardiac arrhythmias, although none are determinative. The risk factors can be affected by adjusting lifestyle habits, such as a particular diet, impacting the risk of arrhythmogenic events and mortality. To date, the importance of understanding the relationship between diet and inherited channelopathies has been underrated. Therefore, we describe herein the effects of dietary factors on the development of arrhythmia in patients affected by BrS and LQTS. Modifying the diet might not be enough to fully prevent arrhythmias, but it can help lower the risk.

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