title: Utility of cardiac magnetic resonance imaging to differentiate cardiac sarcoidosis from arrhythmogenic right ventricular cardiomyopathy.
abstract: Some patients diagnosed with arrhythmogenic right ventricular cardiomyopathy (ARVC) are eventually found to have cardiac sarcoidosis (CS). Accurate differentiation between these 2 conditions has implications for immunosuppressive therapy and familial screening. We sought to determine whether cardiac magnetic resonance imaging (MRI) could be used to identify the characteristic findings to accurately differentiate between CS and ARVC. Consecutive patients with a diagnostic MRI scan indicating CS and/or ARVC constituted the cohort. All patients diagnosed with CS had histologic confirmation of sarcoidosis, and all patients with ARVC met the diagnostic task force criteria. The cardiac MRI data were retrospectively analyzed to identify possible differentiating characteristics. Of the patients, 40 had CS and 21 had ARVC. Those with CS were older and had more left ventricular scar. The presence of mediastinal lymphadenopathy or left ventricular septal involvement was seen exclusively in the patients with CS (p <0.001). A family history of sudden cardiac death was seen only in the ARVC group (p = 0.012). The right ventricular ejection fraction and ventricular volumes were also significantly different between the 2 groups. In conclusion, patients with CS have significantly different cardiac MRI characteristics than patients with ARVC. The cardiac volume, in addition to the degree and location of cardiac involvement, can be used to distinguish between these 2 disease entities. The presence of mediastinal lymphadenopathy and left ventricular septal scar favors a diagnosis of CS and not ARVC. Consideration of CS should be given if these MRI findings are observed during the evaluation for possible ARVC.
authors.affiliation: Section of Cardiac Electrophysiology, University of Colorado, Denver, Colorado, USA.
authors.forename: David A
authors.initials: DA
authors.lastname: Steckman
authors.forename: Preston M
authors.initials: PM
authors.lastname: Schneider
authors.forename: Joseph L
authors.initials: JL
authors.lastname: Schuller
authors.forename: Ryan G
authors.initials: RG
authors.lastname: Aleong
authors.forename: Duy T
authors.initials: DT
authors.lastname: Nguyen
authors.forename: Gianfranco
authors.initials: G
authors.lastname: Sinagra
authors.forename: Giancarlo
authors.initials: G
authors.lastname: Vitrella
authors.forename: Francesca
authors.initials: F
authors.lastname: Brun
authors.forename: Maria A
authors.initials: MA
authors.lastname: Cova
authors.forename: Lorenzo
authors.initials: L
authors.lastname: Pagnan
authors.forename: Luisa
authors.initials: L
authors.lastname: Mestroni
authors.forename: Paul D
authors.initials: PD
authors.lastname: Varosy
authors.forename: William H
authors.initials: WH
authors.lastname: Sauer
mesh_terms: D000328:Adult; D000368:Aged; D019571:Arrhythmogenic Right Ventricular Dysplasia / Q000150:complications / Q000175:diagnosis* / Q000503:physiopathology; D009202:Cardiomyopathies / Q000150:complications / Q000175:diagnosis* / Q000503:physiopathology; D015331:Cohort Studies; D016757:Death, Sudden, Cardiac / Q000209:etiology; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D008206:Lymphatic Diseases / Q000209:etiology; D008279:Magnetic Resonance Imaging; D008297:Male; D008477:Mediastinal Diseases / Q000209:etiology; D008875:Middle Aged; D012189:Retrospective Studies; D012507:Sarcoidosis / Q000150:complications / Q000175:diagnosis* / Q000503:physiopathology; D012680:Sensitivity and Specificity; D013318:Stroke Volume / Q000502:physiology