[Catecholaminergic polymorphic ventricular tachycardia]. 2012

G Frommeyer, and C Pott, and E Schulze-Bahr, and L Eckardt
Abteilung für Rhythmologie Department für Kardiologie und Angiologie, Universitätsklinikum Münster, Albert-Schweitzer Campus 1, 48149, Münster, Germany. gerrit.frommeyer@ukmuenster.de

Catecholaminergic polymorphic ventricular tachycardia (PCVT) is a rare, congenital ventricular tachyarrhythmia which occurs in the setting of adrenergic activation. It potentially leads to syncope and/or sudden cardiac death (SCD). PCVT represents one of the most dangerous congenital ion channel diseases. Mutations of the ryanodine receptor gene (RYR2), the calsequestrin gene (CASQ2), and the triadin gene (TRDN) have been identified as an underlying correlate. β-Blockers are employed as therapy and are sometimes combined with class IC antiarrhythmic drugs, or calcium antagonists of the verapamil type. ICD implantation is recommended in case of persisting syncope in the presence of β-blocker therapy or survived SCD. Left thoracic sympathectomy represents a subsidiary interventional therapy for individual cases. In addition, modifications of the patient's lifestyle including avoidance of physical stress and heart rates> 120/min are recommended.

UI MeSH Term Description Entries
D005858 Germany A country in central Europe, bordering the Baltic Sea and the North Sea, between the Netherlands and Poland, south of Denmark. The capital is Berlin.
D006333 Heart Failure A heterogeneous condition in which the heart is unable to pump out sufficient blood to meet the metabolic need of the body. Heart failure can be caused by structural defects, functional abnormalities (VENTRICULAR DYSFUNCTION), or a sudden overload beyond its capacity. Chronic heart failure is more common than acute heart failure which results from sudden insult to cardiac function, such as MYOCARDIAL INFARCTION. Cardiac Failure,Heart Decompensation,Congestive Heart Failure,Heart Failure, Congestive,Heart Failure, Left-Sided,Heart Failure, Right-Sided,Left-Sided Heart Failure,Myocardial Failure,Right-Sided Heart Failure,Decompensation, Heart,Heart Failure, Left Sided,Heart Failure, Right Sided,Left Sided Heart Failure,Right Sided Heart Failure
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D012307 Risk Factors An aspect of personal behavior or lifestyle, environmental exposure, inborn or inherited characteristic, which, based on epidemiological evidence, is known to be associated with a health-related condition considered important to prevent. Health Correlates,Risk Factor Scores,Risk Scores,Social Risk Factors,Population at Risk,Populations at Risk,Correlates, Health,Factor, Risk,Factor, Social Risk,Factors, Social Risk,Risk Factor,Risk Factor Score,Risk Factor, Social,Risk Factors, Social,Risk Score,Score, Risk,Score, Risk Factor,Social Risk Factor
D015994 Incidence The number of new cases of a given disease during a given period in a specified population. It also is used for the rate at which new events occur in a defined population. It is differentiated from PREVALENCE, which refers to all cases in the population at a given time. Attack Rate,Cumulative Incidence,Incidence Proportion,Incidence Rate,Person-time Rate,Secondary Attack Rate,Attack Rate, Secondary,Attack Rates,Cumulative Incidences,Incidence Proportions,Incidence Rates,Incidence, Cumulative,Incidences,Person time Rate,Person-time Rates,Proportion, Incidence,Rate, Attack,Rate, Incidence,Rate, Person-time,Rate, Secondary Attack,Secondary Attack Rates
D015996 Survival Rate The proportion of survivors in a group, e.g., of patients, studied and followed over a period, or the proportion of persons in a specified group alive at the beginning of a time interval who survive to the end of the interval. It is often studied using life table methods. Cumulative Survival Rate,Mean Survival Time,Cumulative Survival Rates,Mean Survival Times,Rate, Cumulative Survival,Rate, Survival,Rates, Cumulative Survival,Rates, Survival,Survival Rate, Cumulative,Survival Rates,Survival Rates, Cumulative,Survival Time, Mean,Survival Times, Mean,Time, Mean Survival,Times, Mean Survival
D016019 Survival Analysis A class of statistical procedures for estimating the survival function (function of time, starting with a population 100% well at a given time and providing the percentage of the population still well at later times). The survival analysis is then used for making inferences about the effects of treatments, prognostic factors, exposures, and other covariates on the function. Analysis, Survival,Analyses, Survival,Survival Analyses
D017180 Tachycardia, Ventricular An abnormally rapid ventricular rhythm usually in excess of 150 beats per minute. It is generated within the ventricle below the BUNDLE OF HIS, either as autonomic impulse formation or reentrant impulse conduction. Depending on the etiology, onset of ventricular tachycardia can be paroxysmal (sudden) or nonparoxysmal, its wide QRS complexes can be uniform or polymorphic, and the ventricular beating may be independent of the atrial beating (AV dissociation). Idiopathic Ventricular Tachycardia,Nonsustained Ventricular Tachycardia,Paroxysmal Supraventricular Tachycardia,Ventricular Tachyarrhythmias,Ventricular Tachycardia,Idiopathic Ventricular Tachycardias,Nonsustained Ventricular Tachycardias,Paroxysmal Supraventricular Tachycardias,Supraventricular Tachycardia, Paroxysmal,Tachyarrhythmia, Ventricular,Tachycardia, Idiopathic Ventricular,Tachycardia, Nonsustained Ventricular,Tachycardia, Paroxysmal Supraventricular,Ventricular Tachyarrhythmia,Ventricular Tachycardia, Idiopathic,Ventricular Tachycardia, Nonsustained,Ventricular Tachycardias
D020022 Genetic Predisposition to Disease A latent susceptibility to disease at the genetic level, which may be activated under certain conditions. Genetic Predisposition,Genetic Susceptibility,Predisposition, Genetic,Susceptibility, Genetic,Genetic Predispositions,Genetic Susceptibilities,Predispositions, Genetic,Susceptibilities, Genetic

Related Publications

G Frommeyer, and C Pott, and E Schulze-Bahr, and L Eckardt
May 2016, Circulation journal : official journal of the Japanese Circulation Society,
G Frommeyer, and C Pott, and E Schulze-Bahr, and L Eckardt
December 2010, Cardiac electrophysiology clinics,
G Frommeyer, and C Pott, and E Schulze-Bahr, and L Eckardt
May 2010, European journal of pediatrics,
G Frommeyer, and C Pott, and E Schulze-Bahr, and L Eckardt
May 2005, Heart rhythm,
G Frommeyer, and C Pott, and E Schulze-Bahr, and L Eckardt
May 2007, Herz,
G Frommeyer, and C Pott, and E Schulze-Bahr, and L Eckardt
June 2017, Pediatric emergency care,
G Frommeyer, and C Pott, and E Schulze-Bahr, and L Eckardt
January 2020, Cardiology in review,
G Frommeyer, and C Pott, and E Schulze-Bahr, and L Eckardt
January 2008, Progress in cardiovascular diseases,
G Frommeyer, and C Pott, and E Schulze-Bahr, and L Eckardt
October 2012, Circulation. Arrhythmia and electrophysiology,
G Frommeyer, and C Pott, and E Schulze-Bahr, and L Eckardt
April 2022, Arrhythmia & electrophysiology review,
Copied contents to your clipboard!