Abstract
“Arrhythmogenic right ventricular dysplasia” (ARVD), a heart muscle disorder characterized by the presence of fibro-fatty tissue and ventricular electrical vulnerability related to sudden death, was first described in 1977 by a French team. Since then, other terms such as “arrhythmogenic right ventricular cardiomyopathy” (ARVC), “arrhythmogenic cardiomyopathy” (AC), “left-dominant arrhythmogenic cardiomyopathy” (LDAC), and “arrhythmogenic left ventricular dysplasia” (ALVD) have been introduced. These changes in nomenclature of the same disease entity are based on different explanations of pathomorphologic patterns. The dysplasia theory claims cardiac growth “maldevelopment” whereas the cardiomyopathy has been seen as an atrophy from acquired injury (myocyte death) and repair (fibrofatty replacement). The other area of divergent opinion is with regards to involvement of both ventricles rather than being an isolated right ventricular anomaly that may result in increased likelihood of diagnosing the concealed form manifesting with pre-dominant left ventricular arrhythmias. Multiple line of evidences support common disease path-ways: Presence of fibro-fatty and superimposed myocarditis, desmosome mutations and malfunc-tion. These compelling data regarding the heart growth, and pathological, clinical, phenotype/ genotype correlates have advanced our understanding of arrhythmogenic ventricular dysplasia/ cardiomyopathy and increased the diagnostic accuracy as well as providing an avenue for future development of new mechanism-based therapies.
Highlights
Arrhythmogenic right ventricular dysplasia (ARVD), an inherited arrhythmogenic disease of heart muscle that can lead to sudden cardiac death (SCD), has been variously named as arrhythmogenic right ventricular cardiomyopathy (ARVC), arrhythmogenic cardiomyopathy (AC), “left-dominant arrhythmogenic cardiomyopathy” (LDAC), “arrhythmogenic left ventricular dysplasia” (ALVD)
It is widely agreed that the morphologic substrate of ARVD/C is the presence of fibro-fatty tissue predominantly involving predominantly involving the right ventricle (Figure 1), its pathogenic mechanisms are yet to be fully elucidated
During a surgical procedure to map and treat ventricular tachycardia (VT) at the Hôpital de La Salpêtrière [3], arrhythmogenic right ventricular dysplasia was first recognized as a “trouble in development” by Fontaine’s team (1977 in a book chapter)
Summary
Arrhythmogenic right ventricular dysplasia (ARVD), an inherited arrhythmogenic disease of heart muscle that can lead to sudden cardiac death (SCD), has been variously named as arrhythmogenic right ventricular cardiomyopathy (ARVC), arrhythmogenic cardiomyopathy (AC), “left-dominant arrhythmogenic cardiomyopathy” (LDAC), “arrhythmogenic left ventricular dysplasia” (ALVD). After the discovery of the autosomal recessive pattern of transmission in the Greek island of Naxos, the first gene (plakoglobin) associated with the development of ARVD was identified. This was followed by the discovery of multiple other mutations affecting the desmosomal proteins. The plethora of academic discourse about nomenclature of ARVD/C from its discovery to date is hardly reflected in any other disease entity in the medical literature. The broader term “arrhythmogenic ventricular dysplasia/cardiomyopathy” (AVD/C) will be used when appropriate to name ARVD, ARVC, AC, LDAC or ALVD
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