Journal
EUROPEAN RADIOLOGY
Volume 33, Issue 1, Pages 270-282Publisher
SPRINGER
DOI: 10.1007/s00330-022-08958-2
Keywords
Arrhythmogenic right ventricular dysplasia; Magnetic resonance imaging; Sudden death
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Arrhythmogenic cardiomyopathy (ACM) is a genetically determined heart muscle disease characterized by fibro-fatty myocardial replacement, clinically associated with malignant ventricular arrhythmias and sudden cardiac death. Cardiac magnetic resonance (CMR) has emerged as the key imaging technique for the diagnosis of ACM, providing detailed evaluation of both right ventricular (RV) and left ventricular (LV) morphology, function, and tissue characteristics, as well as supplemental value for disease variants.
Arrhythmogenic cardiomyopathy (ACM) is a genetically determined heart muscle disease characterized by fibro-fatty myocardial replacement, clinically associated with malignant ventricular arrhythmias and sudden cardiac death. Originally described a disease with a prevalent right ventricular (RV) involvement, subsequently two other phenotypes have been recognized, such as the left dominant and the biventricular phenotypes, for which a recent International Expert consensus document provided upgrade diagnostic criteria (the 2020 Padua Criteria). In this novel workup for the diagnosis of the entire spectrum of phenotypic variants of ACM, including left ventricular (LV) variants, cardiac magnetic resonance (CMR) has emerged as the cardiac imaging technique of choice, due to its capability of detailed morpho-functional and tissue characterization evaluation of both RV and LV. In this review, the key role of CMR in the diagnosis of ACM is outlined, including the supplemental value for the characterization of the disease variants. An ACM-specific CMR study protocol, as well as strengths and weaknesses of each imaging technique, is also provided.
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