Report of the 1995 World Health Organization/International Society and Federation of Cardiology Task Force on the Definition and Classification of cardiomyopathies.

Sudden cardiac death describes the unexpected natural death from a cardiac cause within a short time period, generally ≤ 1 h from the onset of symptoms, in a person without any prior condition that would appear fatal [1, 2]. Such a rapid death is often attributed to a cardiac arrhythmia, but with the advent of monitoring capabilities from implantable cardioverter-defibrillators (ICDs), it is now well recognized that classifications based on clinical circumstances can be misleading and often impossible, because 40% of sudden deaths can be unwitnessed [3]. Only an ECG or a ventricular electrogram recorded from an implanted device at the time of death can provide definitive information about an arrhythmia. Prodromal symptoms are often nonspecific, and even those taken to indicate ischemia (chest pain), a tachyarrhythmia (palpitations), or congestive heart failure symptoms (dyspnea) can only be considered suggestive. For these reasons, total mortality, rather than classifications of cardiac and arrhythmic mortality, should be used as primary objectives for many outcome studies.

Sudden Cardiac Death

It is important that the medical profession plays a significant role in critically evaluating the use of diagnostic procedures and therapies as they are introduced and tested in the detection, management, or prevention of disease states. Rigorous and expert analysis of the available data documenting absolute and relative benefits and risks of those procedures and therapies can produce helpful guidelines that improve the effectiveness of care, optimize patient outcomes, and favorably affect the overall cost of care by focusing resources on the most effective strategies.

The American College of Cardiology Foundation (ACCF) and the American Heart Association (AHA) have jointly engaged in the production of such guidelines in the area of cardiovascular disease since 1980. The ACC/AHA Task Force on Practice Guidelines, whose charge is to develop, update, or revise practice guidelines for important cardiovascular diseases and procedures, directs this effort. The Task Force is pleased to have this guideline developed in conjunction with the European Society of Cardiology (ESC). Writing committees are charged with the task of performing an assessment of the evidence and acting as an independent group of authors to develop or update written recommendations for clinical practice.

Experts in the subject under consideration have been selected from all 3 organizations to examine subject-specific data and write guidelines. The process includes additional representatives from other medical practitioner and specialty groups when appropriate. Writing committees are specifically charged to perform a formal literature review, weigh the strength of evidence for or against a particular treatment or procedure, and include estimates of expected health outcomes where data exist. Patient-specific modifiers, comorbidities, and issues of patient preference that might influence the choice of particular tests or therapies are considered as well as frequency of follow-up and cost effectiveness. When available, information from studies on cost will be considered; however, review …

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ACC/AHA/ESC 2006 guidelines for management of patients with ventricular arrhythmias and the prevention of sudden cardiac death

Background— In 1994, an International Task Force proposed criteria for the clinical diagnosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) that facilitated recognition and ...

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Diagnosis of Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia Proposed Modification of the Task Force Criteria

In biology, classification systems are used to promote understanding and systematic discussion through the use of logical groups and hierarchies In clinical medicine, similar principles are used to standardise the nomenclature of disease For more than three decades, heart muscle diseases have been classified into primary or idiopathic myocardial diseases (cardiomyopathies) and secondary disorders that have similar morphological appearances, but which are caused by an identifiable pathology such as coronary artery disease or myocardial infiltration (specific heart muscle diseases) In this document, The European Society of Cardiology Working Group on Myocardial and Pericardial Diseases presents an update of the existing classification scheme The aim is to help clinicians look beyond generic diagnostic labels in order to reach more specific diagnoses

/pdf/classification-of-the-cardiomyopathies-a-position-statement-5d1tlwp7az.pdf

Classification of the cardiomyopathies: a position statement from the european society of cardiology working group on myocardial and pericardial diseases

Diagnosis of arrhythmogenic right ventricular dysplasia/cardiomyopathy. Task Force of the Working Group Myocardial and Pericardial Disease of the European Society of Cardiology and of the Scientific Council on Cardiomyopathies of the International Society and Federation of Cardiology.

https://heart.bmj.com/content/heartjnl/71/3/215.full.pdf

Spectrum of Clinicopathologic Manifestations of Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia: A Multicenter Study

Background Arrhythmogenic right ventricular dysplasia, a disorder that may lead to severe ventricular arrhythmias and sudden death, is characterized by the progressive replacement of myocardial cells by fat and fibrous tissue. We examined whether the loss of myocardial cells in this disease could result from cell death by apoptosis (programmed cell death). Methods Specimens obtained at autopsy from the right ventricular myocardium of eight patients with arrhythmogenic right ventricular dysplasia and four age-matched normal subjects were analyzed. To identify individual cells undergoing apoptosis, we performed in situ end-labeling of fragmented DNA on paraffin sections using biotinylated deoxyuridine triphosphate and the enzyme terminal deoxynucleotidyl transferase. We also examined the level of expression of CPP-32, a cysteine protease required for apoptotic cell death in mammalian cells, using immunohistochemical techniques. Results Apoptosis was detected in the right ventricular myocardium of six of the...

Evidence of Apoptosis in Arrhythmogenic Right Ventricular Dysplasia

In this issue of Circulation , Burke et al1 report the results of a morphological and morphometric analysis of RV myocardium to differentiate fatty infiltration of the RV free wall from fibrofatty infiltration, which is the histological marker of ARVC. A similar analysis was previously presented by Thiene et al,2 classifying their patients as having “lipomatous” as opposed to “fibrolipomatous” replacement of the myocardium and putting the two entities in the same group. Unless small areas of fibrosis are overlooked, the two subgroups of Burke et al and Thiene et al have the histological picture of myocardium intermingled with and/or replaced by fat without associated fibrosis. In this population of patients who died suddenly, we are faced with the observation that strands of myocardial fibers within fat without fibrosis may lead to sudden death. This possibility has rather important consequences, because a large proportion of seemingly normal hearts have fatty infiltration of the RV myocardium.3 It may be that the fatty pattern in these patients represents the early stage of the disease before the appearance of fibrotic tissue. Therefore, a large proportion of normal individuals may in fact have the prerequisite for the development of RV cardiomyopathies.

It is well known that a large majority of patients with ARVD have histological evidence suggestive of inflammation. It may be that if myocarditis is superimposed on the background of myocardium interspersed by fat, strands of cardiomyocytes involved in myocarditis will produce fibrosis and will transform the purely fatty to the fibrofatty form. Activation of neutrophils induced by myocarditis will enhance the risk of arrhythmogenesis.4 Therefore, the two groups identified by Burke et al may represent two consecutive stages of myocardial disease progression.

It is possible that multiple attacks of myocarditis could lead to the destruction of an increasing …

Arrhythmogenic Right Ventricular Cardiomyopathies Clinical Forms and Main Differential Diagnoses

Arrhythmogenic right ventricular dysplasia is responsible for ventricular tachycardia affecting an apparently healthy heart. It can sometimes lead to sudden death, which may be the presenting symptomatology of the disease. It results from fibro-adipose infiltration of the free wall of the right ventricle, and sometimes of the septum, possibly secondary to myocarditis. The prognosis depends upon the quality of the left ventricle. If it is healthy, the only risk is that of arrhythmia. Treatment using anti-arrhythmic drugs is most often effective and, with proper management, the prognosis is good and the risk of sudden death eliminated. If the left ventricle is abnormal, there is the risk that dysplasia associated with arrhythmia will progress to right then congestive cardiac failure in the context of a dilated idiopathic cardiomyopathy with ventricular tachycardias originating on the right side. Arrhythmogenic right ventricular dysplasia is a notable cause of sudden death in athletes. Routine screening of such individuals is justified, as is that of those with high risk occupations (locomotive and vehicle drivers, etc.).

Fontaliran F

Papers

Diagnosis of arrhythmogenic right ventricular dysplasia/cardiomyopathy. Task Force of the Working Group Myocardial and Pericardial Disease of the European Society of Cardiology and of the Scientific Council on Cardiomyopathies of the International Society and Federation of Cardiology.

Spectrum of Clinicopathologic Manifestations of Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia: A Multicenter Study

Evidence of Apoptosis in Arrhythmogenic Right Ventricular Dysplasia

Arrhythmogenic Right Ventricular Cardiomyopathies Clinical Forms and Main Differential Diagnoses

Right ventricular dysplasia