Abstract
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 as 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. In the novel 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 case series, the key role of CMR in the diagnosis of ACM is outlined, including the supplemental value for the characterization of the disease variants, acute phase, and differential diagnosis with phenocopies.
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Acknowledgments
The authors are thankful with Dr. Nicolò Martini, Dr. Francesca Graziano, Dr. Samuele Meneghin, Dr. Giulia Brunetti, Dr. Chiara Cappelletto, and Dr. Maria Perotto for the excellent contribution in cases presentation and kind support in CMR images and video retrieval.
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CineSSFP 4-chamber cine view (MP4 157 kb)
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CineSSFP 4-chamber cine view (MP4 57 kb)
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CineSSFP long-axis cine stack (MP4 1603 kb)
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CineSSFP long-axis cine stack (MP4 1534 kb)
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Cipriani, A., De Luca, A., Curcio, A., Marra, M.P. (2023). Arrhythmogenic Cardiomyopathies. In: Barison, A., Dellegrottaglie, S., Pontone, G., Indolfi, C. (eds) Case-based Atlas of Cardiovascular Magnetic Resonance. Springer, Cham. https://doi.org/10.1007/978-3-031-32593-9_8
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DOI: https://doi.org/10.1007/978-3-031-32593-9_8
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