TY - JOUR
T1 - Differentiating hereditary arrhythmogenic right ventricular cardiomyopathy from cardiac sarcoidosis fulfilling 2010 ARVC Task Force Criteria
AU - Gasperetti, Alessio
AU - Rossi, Valentina A.
AU - Chiodini, Alessandra
AU - Casella, Michela
AU - Costa, Sarah
AU - Akdis, Deniz
AU - Büchel, Ronny
AU - Deliniere, Antoine
AU - Pruvot, Etienne
AU - Gruner, Christiane
AU - Carbucicchio, Corrado
AU - Manka, Robert
AU - Dello Russo, Antonio
AU - Tondo, Claudio
AU - Brunckhorst, Corinna
AU - Tanner, Felix
AU - Duru, Firat
AU - Saguner, Ardan M.
N1 - Funding Information:
Funding sources: The Zurich ARVC Program is sponsored by grants from the Georg and Bertha Schwyzer-Winiker Foundation , the Baugarten Foundation , and Dr Hans-Peter Wild, Switzerland.
Funding Information:
Disclosures: Dr Saguner reports educational grants from Abbott , Bayer Healthcare, Biosense Webster , Biotronik, Boston Scientific , Medtronic, and Pfizer- BMS outside this work; and owns stock from Gilead Sciences . All other authors have no conflicts of interest to disclose.
Publisher Copyright:
© 2020 Heart Rhythm Society
Copyright:
Copyright 2021 Elsevier B.V., All rights reserved.
PY - 2021/2
Y1 - 2021/2
N2 - Background: The clinical presentation of cardiac sarcoidosis (CS) may resemble that of arrhythmogenic right ventricular cardiomyopathy (ARVC). Objective: The purpose of this study was to identify clinical variables to better discriminate between patients with genetically determined ARVC and those with CS fulfilling definite 2010 ARVC Task Force Criteria (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-fluorodeoxyglucose positron emission tomographic (18F-FDG PET) scan was required for patients to be included in the study. Results: The 2010 ARVC TFC did not reliably differentiate between the 2 diseases. CS patients presented with longer PR intervals, advanced atrioventricular block (AVB), and longer QRS duration (P <.001 and P = .009, respectively), whereas T-wave inversions (TWIs) in the peripheral leads were more common in ARVC patients (P = .009). CS patients presented with more extensive left ventricular involvement and lower left ventricular ejection fraction (LVEF), whereas ARVC patients had a larger right ventricular outflow tract (RVOT) (P = .044). PET scan positivity was only present in CS patients (90% vs 0%). Conclusion: The 2010 ARVC TFC do not reliably differentiate between CS patients fulfilling 2010 ARVC TFC and those with hereditary ARVC. Prolonged PR interval, advanced AVB, longer QRS duration, right ventricular apical involvement, reduced LVEF, and positive 18F-FDG PET scan should raise the suspicion of CS, whereas larger RVOT dimensions, subtricuspid involvement and peripheral TWI favor a diagnosis of hereditary ARVC.
AB - Background: The clinical presentation of cardiac sarcoidosis (CS) may resemble that of arrhythmogenic right ventricular cardiomyopathy (ARVC). Objective: The purpose of this study was to identify clinical variables to better discriminate between patients with genetically determined ARVC and those with CS fulfilling definite 2010 ARVC Task Force Criteria (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-fluorodeoxyglucose positron emission tomographic (18F-FDG PET) scan was required for patients to be included in the study. Results: The 2010 ARVC TFC did not reliably differentiate between the 2 diseases. CS patients presented with longer PR intervals, advanced atrioventricular block (AVB), and longer QRS duration (P <.001 and P = .009, respectively), whereas T-wave inversions (TWIs) in the peripheral leads were more common in ARVC patients (P = .009). CS patients presented with more extensive left ventricular involvement and lower left ventricular ejection fraction (LVEF), whereas ARVC patients had a larger right ventricular outflow tract (RVOT) (P = .044). PET scan positivity was only present in CS patients (90% vs 0%). Conclusion: The 2010 ARVC TFC do not reliably differentiate between CS patients fulfilling 2010 ARVC TFC and those with hereditary ARVC. Prolonged PR interval, advanced AVB, longer QRS duration, right ventricular apical involvement, reduced LVEF, and positive 18F-FDG PET scan should raise the suspicion of CS, whereas larger RVOT dimensions, subtricuspid involvement and peripheral TWI favor a diagnosis of hereditary ARVC.
KW - Arrhythmogenic right ventricular cardiomyopathy
KW - Cardiac sarcoidosis
KW - Cardiomyopathy
KW - Genetic
KW - International task force criteria
UR - http://www.scopus.com/inward/record.url?scp=85099335274&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85099335274&partnerID=8YFLogxK
U2 - 10.1016/j.hrthm.2020.09.015
DO - 10.1016/j.hrthm.2020.09.015
M3 - Article
C2 - 32976989
AN - SCOPUS:85099335274
VL - 18
SP - 231
EP - 238
JO - Heart Rhythm
JF - Heart Rhythm
SN - 1547-5271
IS - 2
ER -