Contemporary Definitions and Classification of the Cardiomyopathies: An American Heart Association Scientific Statement From the Council on Clinical Cardiology, Heart Failure and Transplantation Committee; Quality of Care and Outcomes Research and Functional Genomics and Translational Biology Int... (original) (raw)

Contemporary Definitions and Classification of the Cardiomyopathies

Circulation, 2006

Classifications of heart muscle diseases have proved to be exceedingly complex and in many respects contradictory. Indeed, the precise language used to describe these diseases is profoundly important. A new contemporary and rigorous classification of cardiomyopathies (with definitions) is proposed here. This reference document affords an important framework and measure of clarity to this heterogeneous group of diseases. Of particular note, the present classification scheme recognizes the rapid evolution of molecular genetics in cardiology, as well as the introduction of several recently described diseases, and is unique in that it incorporates ion channelopathies as a primary cardiomyopathy.

Genetics and cardiac channelopathies

Genetics in Medicine, 2010

Sudden cardiac death is a major contributor to mortality in industrialized nations; in fact, it is the cause of more deaths than acquired immune deficiency syndrome, lung and breast cancer, and stroke together. Frequently, the autopsy becomes the principal diagnostic tool because macroscopic and microscopic analyses reveal the underlying cause of death. However, a significant number of sudden cardiac deaths remain unexplained. These cases are referred to as "natural" or arrhythmogenic. In the young, in up to 50% of sudden cardiac death cases, sudden death is the first and only clinical manifestation of an inherited cardiac disease that had remained undetected by conventional clinical investigations. To improve diagnosis, genetic testing has recently been added to these clinical tools. During the last two decades, there has been considerable progress in the understanding about genetics of sudden cardiac death. With that new information, the probands and their family members can make an informed decision regarding their care and know whether and to what extent they are at risk of suffering from the disease. Thus, genetic technology and expertise have become essential for the diagnosis of some forms of inherited cardiac diseases and to provide a basis for subsequent prevention strategies. This review focuses on recent advances in the understanding of cardiopathies owing to genetic investigations. Genet Med 2010:12(5):260 -267.

The molecular basis of cardiac arrhythmias in patients with cardiomyopathy

Current heart failure reports, 2004

Cardiac arrhythmias are a leading cause of mortality and morbidity in Western society. In some specific instances, these arrhythmias are caused by abnormalities of cardiac ion channels, such as sodium, calcium, and potassium channels, which carry ionic currents and are fundamental determinants of cardiac excitability. Abnormalities of these ion channels are attributed to mutations in the genes encoding the channel protein and cause altered function of channels, which can predispose to arrhythmias. During heart failure, many channels also malfunction because of altered expression, resulting in lethal arrhythmias.

Cardiomyopathies: A Historical Journey

2024

This chapter in question provides a comprehensive overview of the evolution of cardiomyopathy classifications, tracing the historical development of key concepts that have shaped the primary categorizations recognized in medical literature. It offers a critical analysis of the present state of cardiomyopathy classifications, exploring the nuances and contentious points within the latest frameworks proposed by leading health authorities such as the European Society of Cardiology (ESC) and the American Heart Association (AHA). The narrative also encompasses the inception and transformation of the term 'cardiomyopathy' itself, culminating in the adoption of morphological-functional types that are crucial to the clinical management of these heart muscle disorders.