Cardiac magnetic resonance features of left dominant arrhythmogenic cardiomyopathy: differential diagnosis with myocarditis

• Published in: The International Journal of Cardiovascular Imaging Vol. 38; no. 2; pp. 397 - 405
• Main Authors: Andreini, Daniele, Conte, Edoardo, Casella, Michela, Mushtaq, Saima, Pontone, Gianluca, Dello Russo, Antonio, Nicoli, Flavia, Carità, Patrizia, Catto, Valentina, Vettor, Giulia, Gasperetti, Alessio, Sommariva, Elena, Rizzo, Stefania, Basso, Cristina, Tondo, Claudio, Pepi, Mauro
• Format: Journal Article
• Language: English
• Published: Dordrecht Springer Netherlands 01.02.2022; Springer Nature B.V
• Subjects: Abnormalities; Biopsy; Cardiac Imaging; Cardiology; Cardiomyopathy; Diagnosis; Diagnosis, Differential; Differential diagnosis; EKG; Heart diseases; Humans; Imaging; Infiltration; Magnetic resonance; Magnetic Resonance Imaging; Magnetic Resonance Imaging, Cine; Magnetic Resonance Spectroscopy; Medicine; Medicine & Public Health; Morphology; Myocarditis; Myocarditis - diagnostic imaging; Original Paper; Patients; Predictive Value of Tests; Radiology; Resonance; Substrates; Ventricle; Ventricular arrhythmias; Left-dominant arrhythmogenic cardiomyopathy; Cardiac magnetic resonance
• ISSN: 1569-5794; 1573-0743; 1875-8312
• DOI: 10.1007/s10554-021-02408-8

Cardiac magnetic resonance (CMR) findings suggesting a suspected left-dominant arrhythmogenic cardiomyopathy (LDAC) may be difficult to distinguish from those related to previous myocarditis; however, especially in patients with ventricular arrhythmias (VA) with ECG morphology consistent with a left ventricle (LV) origin differential diagnosis is fundamental. Aim of the study was to identify potential imaging features at CMR specific for LDAC diagnosis. Between January 2011 and December 2019, we enrolled 15 consecutive stable patients with a recent diagnosis of significant VA and ECG morphology consistent with a LV origin, detection of potential LV arrhythmic substrate at CMR and undergoing a clinically-indicated LV endomyocardial biopsy showing tissue abnormalities consistent with the diagnosis of LDAC. From the same CMR-endomyocardial biopsy registry, a second group of 30 consecutive patients who underwent CMR and biopsy with a histological diagnosis of previous myocarditis were identified. (1) Subepicardial LGE at the level of the posterolateral wall of the LV was detected in 13 cases of LDAC vs. 21 cases of myocarditis; (2) fat infiltration, and particularly subepicardial posterolateral fat infiltration, was found in almost all LDAC patients vs. one myocarditis only (p < 0.01). (3) No differences in other CMR findings or in any clinical or echocardiographic parameters were found between patients with a biopsy consistent with LDAC vs. myocarditis. In patients with significant VA and ECG morphology consistent with a LV origin, the presence of morpho-functional involvement of the subepicardial layer of LV posterolateral wall at CMR (LGE, fat infiltration, wall dyskinesis) supports LDAC diagnosis.