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.
