Why the study?
Diagnostic modalities, diagnostic criteria, clinical understanding across age groups, and genotype-phenotype correlations for arrhythmogenic cardiomyopathy have continued to evolve beyond initial perceptions of predominant right ventricular involvement.
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Design
Review
May inform age-specific arrhythmogenic cardiomyopathy care; leaves open need for prospective validation before practice change.
Arrhythmogenic cardiomyopathy (AC) is a disease that involves electromechanical uncoupling of cardiomyocytes. This leads to characteristic histologic changes that ultimately lead to the arrhythmogenic clinical features of the disease. Initially thought to affect the right ventricle predominantly, more recent data show that it can affect both the ventricles or the left ventricle alone. Throughout the recent era, diagnostic modalities and criteria for AC have continued to evolve and our understanding of its clinical features in different age groups as well as the genotype to the phenotype correlations have improved. In this review, we set out to detail the epidemiology, etiologies, presentations, evaluation, and management of AC across the age continuum.
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Tadros et al. (2023) studied this question.
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