Background: Cardiac sarcoidosis (CS) may resemble the clinical presentation of arrhythmogenic right ventricular cardiomyopathy (ARVC). Objective: goal of our study was identification of clinical variables to better discriminate between patients with genetically-determined ARVC and CS fulfilling definite ARVC 2010 TFC. Methods: In this multicenter study, 10 patients with CS fulfilling definite 2010 ARVC TFC were age-and gender matched with 10 genetically-proven ARVC patients. A cardiac 18F-FDG PET-scan was required to be included in this study. Results: The 2010 ARVC TFC did not reliably differentiate between the two diseases. CS patients presented with longer PR-intervals, advanced AVB, and a longer QRS-duration (p <0.001; and p=0.009, respectively), while T wave inversions (TWI) in peripheral leads were more common in ARVC (p=0.009). CS patients presented with more extensive LV involvement and a lower LVEF, while ARVC patients had a larger RVOT (p=0.044). PET scan positivity was only present in CS patients (90% vs 0%). Conclusion: The 2010 TFC do not reliably differentiate between CS patients fulfilling 2010 TFC and hereditary ARVC. A prolonged PR interval, advanced AVB, longer QRS duration, RV apical involvement, a reduced LVEF, and a positive 18F-FDG PET scan should raise the suspicion of CS, whereas larger RVOT dimensions and peripheral TWI favor the diagnosis of hereditary ARVC.

Differentiating Hereditary Arrhythmogenic Right Ventricular Cardiomyopathy from Cardiac Sarcoidosis Fulfilling 2010 ARVC Task Force Criteria / A. Gasperetti, V. Rossi, A. Chiodini, M. Casella, S. Costa, D. Akdis, R. Büchel, A. Deliniere, E. Pruvot, C. Gruner, C. Carbucicchio, R. Manka, A.D. Russo, C. Tondo, C. Brunckhorst, F. Tanner, F. Duru, A.M. Saguner. - In: HEART RHYTHM. - ISSN 1547-5271. - 18:2(2021), pp. 231-238. [10.1016/j.hrthm.2020.09.015]

Differentiating Hereditary Arrhythmogenic Right Ventricular Cardiomyopathy from Cardiac Sarcoidosis Fulfilling 2010 ARVC Task Force Criteria

A. Gasperetti;C. Tondo;
2021

Abstract

Background: Cardiac sarcoidosis (CS) may resemble the clinical presentation of arrhythmogenic right ventricular cardiomyopathy (ARVC). Objective: goal of our study was identification of clinical variables to better discriminate between patients with genetically-determined ARVC and CS fulfilling definite ARVC 2010 TFC. Methods: In this multicenter study, 10 patients with CS fulfilling definite 2010 ARVC TFC were age-and gender matched with 10 genetically-proven ARVC patients. A cardiac 18F-FDG PET-scan was required to be included in this study. Results: The 2010 ARVC TFC did not reliably differentiate between the two diseases. CS patients presented with longer PR-intervals, advanced AVB, and a longer QRS-duration (p <0.001; and p=0.009, respectively), while T wave inversions (TWI) in peripheral leads were more common in ARVC (p=0.009). CS patients presented with more extensive LV involvement and a lower LVEF, while ARVC patients had a larger RVOT (p=0.044). PET scan positivity was only present in CS patients (90% vs 0%). Conclusion: The 2010 TFC do not reliably differentiate between CS patients fulfilling 2010 TFC and hereditary ARVC. A prolonged PR interval, advanced AVB, longer QRS duration, RV apical involvement, a reduced LVEF, and a positive 18F-FDG PET scan should raise the suspicion of CS, whereas larger RVOT dimensions and peripheral TWI favor the diagnosis of hereditary ARVC.
arrhyhtmogenic right ventricular cardiomyopathy; cardiac sarcoidosis; cardiomyopathy; genetic; international task force criteria
Settore MED/11 - Malattie dell'Apparato Cardiovascolare
2021
22-set-2020
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/2434/769843
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