Dear Colleagues,
Since the first clinical description of arrhythmogenic cardiomyopathy (ACM) as a cause of juvenile sudden death in the early 80s, important knowledge on the clinical, pathological, and genetic features of this disease has been gained.
Different from the first descriptions, three different phenotypes are now recognized: the “classical” right-dominant form, the biventricular phenotype, and the left-dominant variant.
The shifting concept of ACM from a right ventricular or biventricular disease to an isolated LV clinical entity has led to overlap with other cardiac clinical entities, such as dilated cardiomyopathy and inflammatory cardiac diseases. Genetic studies have revealed that the majority of disease genes encode for desmosomal proteins, even if non-desmosomal genes have been identified. Furthermore, patients can show recurrent myocarditis-like episodes of chest pain with documented myocardial injury, called “hot phase episodes”. Finally, exercise has been confirmed as an important modulating factor that may favor disease penetrance in positive genetic carriers, worsen ventricular dysfunction, and trigger ventricular arrhythmias.
In this special issue, we will analyze in detail the current knowledge in the clinical and instrumental diagnosis, genetic background, arrhythmic risk stratification, and existing therapies in ACM. Furthermore, we will outline the future directions for research in the diagnosis and treatment of this disease.
Prof. Barbara Bauce and Prof. Alessandro Zorzi
Guest Editors
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