Cardiac magnetic resonance imaging of arrhythmogenic cardiomyopathy: evolving diagnostic perspectives

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) invo...

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Veröffentlicht in:European radiology 2023-01, Vol.33 (1), p.270-282
Hauptverfasser: Cipriani, Alberto, Mattesi, Giulia, Bariani, Riccardo, Cecere, Annagrazia, Martini, Nicolò, De Michieli, Laura, Da Pozzo, Stefano, Corradin, Simone, De Conti, Giorgio, Zorzi, Alessandro, Motta, Raffaella, De Lazzari, Manuel, Bauce, Barbara, Iliceto, Sabino, Basso, Cristina, Corrado, Domenico, Perazzolo Marra, Martina
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container_end_page 282
container_issue 1
container_start_page 270
container_title European radiology
container_volume 33
creator Cipriani, Alberto
Mattesi, Giulia
Bariani, Riccardo
Cecere, Annagrazia
Martini, Nicolò
De Michieli, Laura
Da Pozzo, Stefano
Corradin, Simone
De Conti, Giorgio
Zorzi, Alessandro
Motta, Raffaella
De Lazzari, Manuel
Bauce, Barbara
Iliceto, Sabino
Basso, Cristina
Corrado, Domenico
Perazzolo Marra, Martina
description 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. Key Points • Arrhythmogenic cardiomyopathy includes three different phenotypes: dominant right, biventricular, and dominant left. • In 2020, diagnostic criteria have been updated and cardiac magnetic resonance has emerged as the cardiac imaging technique of choice. • This aim of this review is to provide an update of the current state of art regarding the use of CMR in ACM, with a particular focus on novel diagnostic criteria, CMR protocols, and prognostic significance of CMR findings in ACM.
doi_str_mv 10.1007/s00330-022-08958-2
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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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subjects Arrhythmogenic Right Ventricular Dysplasia - diagnostic imaging
Arrhythmogenic Right Ventricular Dysplasia - genetics
Cardiac
Cardiac muscle
Cardiomyopathy
Criteria
Death, Sudden, Cardiac - pathology
Diagnosis
Diagnostic Radiology
Diagnostic systems
Heart diseases
Heart Ventricles
Humans
Imaging
Imaging techniques
Internal Medicine
Interventional Radiology
Magnetic Resonance Imaging
Medical diagnosis
Medical imaging
Medicine
Medicine & Public Health
Muscles
Neuroradiology
Phenotype
Phenotypes
Radiology
Resonance
Ultrasound
Ventricle
title Cardiac magnetic resonance imaging of arrhythmogenic cardiomyopathy: evolving diagnostic perspectives
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