Frontiers in Myocardia

Frontiers in Myocardia

Volume: 3

Compendium on Cardiomyopathies - Basics, Therapeutics, and Perspectives

Compendium on Cardiomyopathies provides an easily readable source of material for practicing physicians, clinicians, cardiologists, intensivists and emergency medicine specialists. It presents an ...
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Arrhythmogenic Right Ventricular Cardiomyopathy

Pp. 141-172 (32)

Iroegbu Chukwuemeka Daniel, Ming Wu, Zhang Hao, Liu Jingsong and Jinfu Yang

Abstract

Arrhythmogenic right ventricular cardiomyopathy is a myocardial disease that primarily affects the right ventricle where the right ventricular free wall is partially or almost entirely replaced by fatty or fibrofatty tissue, providing a substrate for lifethreatening ventricular arrhythmias in young, apparently healthy individuals and athletes. However, whether fatty or fibrofatty infiltration of the right ventricle has been considered to be a sufficient morphologic hallmark of arrhythmogenic right ventricular cardiomyopathy is still a source of controversy as it is crucial that arrhythmogenic right ventricular cardiomyopathy is kept distinct from both fatty infiltrations of the right ventricle and Adipositas cordis. The autosomal dominant inheritance pattern is typical of this myocardial disease, and the identification of causative mutations in cell adhesion proteins has shed new light on its pathogenesis with familiar studies highlighting the need to broaden the diagnostic criteria, which are highly specific but lacking in sensitivity. This chapter will provide a detailed insight into arrhythmogenic right ventricular cardiomyopathies pathology, pathophysiology, clinical presentations, diagnosis, and management. It will however also highlight the genetic basis of arrhythmogenic right ventricular cardiomyopathy, risk stratification, phenotypic manifestations, and arrhythmia mechanisms with an analytical review of the evolving role of cardiac magnetic resonance and late gadolinium enhancement with contrastenhanced imaging in the diagnostic workup of arrhythmogenic right ventricular cardiomyopathy.

Keywords:

Arrhythmia, Adipositas, Cardiomyopathy, Cather ablation, Desmosomes, Death, Defibrillator, Diagnosis, Electrocardiography, Electrophysiology, Genetics, Heart failure, Pathology, Pathophysiology, Prevention, Right ventricle, Sudden cardiac death, Tachyarrhythmias.

Affiliation:

Department of Cardiovascular Surgery, The Second Xiangya Hospital, Central South University, Middle Renmin Road 139, 410011 Changsha, China