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 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).

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.

Predictors of sudden cardiac death and ventricular tachyarrhythmias in patients with hypertrophic cardiomyopathy

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
N Rineiska ◽  
E Zakharava ◽  
S Komissarova ◽  
I Haidel
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
High Risk ◽  
Sudden Cardiac Death ◽  
Myocardial Fibrosis ◽  
Cardiac Death ◽  
T1 Mapping ◽  
Adverse Outcome ◽  
Low Risk ◽  
Ventricular Tachyarrhythmias ◽  
Risk Patients

Abstract Background Determining additional predictors that allow more accurate identification of patients who need primary prevention of sudden cardiac death (SCD) and ventricular tachyarrhythmias (VT) in patients with hypertrophic cardiomyopathy (HCM) is one of the most important tasks. High-risk patients can be more accurately identified using modern non-invasive research methods. Purpose Identification of new risk predictors for SCD and ventricular tachyarrhythmias based on cardiac magnetic resonance (CMR) with late gadolinium enhancement (LGE) and T1-mapping in patients with hypertrophic cardiomyopathy. Materials and methods Clinical data of 98 HCM subjects (58 males and 40 females, median age 46.5 [35.2; 54.7] treated in our centre in the period between 2013 and 2017 have been studied. All patients underwent CMR with LGE and T1-mapping. Results According to the existing ESC-2014 scale, 45 (46%) of the 98 patients included in the study had a low risk, an intermediate risk was identified in 26 (26%) patients and a high risk of SCD in 27 (28%). During the follow-up period 16 episodes of sudden cardiac death were recorded. Of these, only 8 (50%) patients had a high risk on the ESC-2014 scale. High-risk patients show significantly greater myocardial fibrosis (Me 28.5%; [21.9; 44.1]) compared with patients with intermediate (Me 17.6% [8.0; 22.5]) and low risk of SCD (Me 11.7%; [5.8; 17.6]), p<0.001. A threshold level of fibrosis volume associated with an adverse outcome was determined, which was 15% (based on determining the maximum rank of statistics). For patients with 15% fibrosis volume, the cumulative survival rate on the Kaplan-Mayer curve over the observation period was 96% (95% CI 88.6–100), while for patients with fibrosis volume ≥15% - 72.4% (95% CI 60.6–86.4). The study showed that in 15 (93.7%) patients out of 16 who had an adverse outcome, the volume of myocardial fibrosis was ≥15%. Episodes of nonsustained ventricular tachycardia were detected in 48 patients (48.7%) according to daily ECG monitoring. To identify patients with ventricular tachyarrhythmias by ROC analysis, the threshold value of extracellular myocardial volume was determined using CMR with LGE and T1 mapping, which was 32.5% (sensitivity 74% and specificity 86%). According to multivariate analysis, one of the independent predictors of ventricular tachyarrhythmias was the level of extracellular myocardial volume ≥32.5% (HR 1.2; 95% CI 1.03–1.4). Conclusion Predictors detected by CMR with LGE and T1 mapping can identify patients with HCM with a high risk of sudden cardiac death and ventricular tachyarrhythmias. Funding Acknowledgement Type of funding source: None

scholarly journals Role of spatial dispersion of repolarization in inherited and acquired sudden cardiac death syndromes

2007 ◽  
Vol 293 (4) ◽  
pp. H2024-H2038 ◽  
Author(s):  
Charles Antzelevitch
Keyword(s):  
Sudden Cardiac Death ◽  
Cardiac Death ◽  
Spatial Dispersion ◽  
Ventricular Myocardium ◽  
Polymorphic Ventricular Tachycardia ◽  
Long Qt ◽  
Dispersion Of Repolarization ◽  
Qt Syndrome ◽  
Transmural Dispersion

This review examines the role of spatial electrical heterogeneity within the ventricular myocardium on the function of the heart in health and disease. The cellular basis for transmural dispersion of repolarization (TDR) is reviewed, and the hypothesis that amplification of spatial dispersion of repolarization underlies the development of life-threatening ventricular arrhythmias associated with inherited ion channelopathies is evaluated. The role of TDR in long QT, short QT, and Brugada syndromes, as well as catecholaminergic polymorphic ventricular tachycardia (VT), is critically examined. In long QT syndrome, amplification of TDR is often secondary to preferential prolongation of the action potential duration (APD) of M cells; in Brugada syndrome, however, it is thought to be due to selective abbreviation of the APD of the right ventricular epicardium. Preferential abbreviation of APD of the endocardium or epicardium appears to be responsible for the amplification of TDR in short QT syndrome. In catecholaminergic polymorphic VT, reversal of the direction of activation of the ventricular wall is responsible for the increase in TDR. In conclusion, long QT, short QT, Brugada, and catecholaminergic polymorphic VT syndromes are pathologies with very different phenotypes and etiologies, but they share a common final pathway in causing sudden cardiac death.

Implantable Cardioverter–Defibrillators for Primary Prevention of Sudden Cardiac Death

2010 ◽  
Vol 6 (3) ◽  
pp. 92
Author(s):  
Felix Gramley ◽  
Andreas Goette ◽  
◽  
Keyword(s):  
Primary Prevention ◽  
High Risk ◽  
Sudden Cardiac Death ◽  
Cardiac Death ◽  
Heart Diseases ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Polymorphic Ventricular Tachycardia ◽  
Implantable Cardioverter Defibrillators ◽  
Qt Syndrome ◽  
Cardioverter Defibrillators

Sudden cardiac death (SCD) remains a major cause of death in the industrialised world. Implantable cardioverter–defibrillators (ICDs) have been shown to be an effective therapy option for the primary prevention of SCD in patients at high risk of SCD. This review will discuss adequate risk stratification in various disease states, such as coronary artery disease, ischaemic and non-ischaemic cardiomyopathies (dilated cardiomyopathy, hypertrophic cardiomyopathy, arrhythmogenic right ventricular cardiomyopathy), channelopathies (Brugada syndrome, long- QT syndrome, short-QT syndrome, catecholaminergic polymorphic ventricular tachycardia) and congenital heart diseases, to identify patients at high risk of SCD and selection criteria for ICD therapy. The most important clinical primary prevention trials will be highlighted. Finally, complications of device therapy and quality of life issues will be addressed.

Abstract 654: Ventricular Arrhythmia Following Alcohol Septal Ablation for Hypertrophic Obstructive Cardiomyopathy: Clinical Predictors of Events

Circulation ◽  
2008 ◽  
Vol 118 (suppl_18) ◽  
Author(s):  
Peter A Noseworthy ◽  
Michael Rosenberg ◽  
Jeremy N Ruskin ◽  
Igor F Palacios ◽  
Theofanie Mela ◽  
...  
Keyword(s):  
High Risk ◽  
Sudden Cardiac Death ◽  
Ventricular Arrhythmia ◽  
Cardiac Death ◽  
Massachusetts General Hospital ◽  
Hypertrophic Obstructive Cardiomyopathy ◽  
Low Risk ◽  
Alcohol Septal Ablation ◽  
Septal Myectomy ◽  
Risk Patients

Alcohol septal ablation (ASA) is a non-surgical alternative to septal myectomy for treatment of symptomatic, drug refractory hypertrophic obstructive cardiomyopathy (HOCM). The effect of ASA on the risk of ventricular arrhythmia (VA) is unknown. There is a theoretic concern for pro-arrhythmia at the site of myocardial injury. We examined the rates of sudden cardiac death (SCD) and VA (defined as VA requiring ICD therapy or documented VT/VF) among all 88 patients treated with ASA at Massachusetts General Hospital between 1998 and 2004. Of these patients, 37 had either an ICD or a permanent pacemaker in situ that enabled surveillance for VA events. Patients were classified as either high-risk (one of the following: VA or positive EP study, syncope due to suspected VA, family history of SCD, septal thickness ≥30mm by echocardiography, or failure to augment sBP >20mmHg on stress testing), or low-risk (no high-risk features). There was no mortality attributable to sudden cardiac death (mean follow up 35±21mo). The event rate for VA in low-risk patients was 3.0% per year (2/18 patients), and 6.3% annually among high-risk patients (4/16 patients). Device data were not available on three patients. In a stepwise Cox proportional hazards multivariate analysis that included pre-ASA arrhythmia, peri-ASA arrhythmia, pre-ASA gradient, change in gradient after ASA, peak CK-MB, and post-ASA gradient, only post-ASA gradient predicted future VA (HR 1.06, 95% CI 1.03–1.10, p <0.001). In this single center experience, ASA was not associated with SCD or with high rates of VA. The post-ASA gradient correlates with the risk of ventricular arrhythmia after ASA.

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