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Differentiating hereditary arrhythmogenic right ventricular cardiomyopathy from cardiac sarcoidosis fulfilling 2010 ARVC Task Force Criteria


Gasperetti, Alessio; Rossi, Valentina; Chiodini, Alessandra; Casella, Michela; Costa, Sarah; Akdis, Deniz; Büchel, Ronny; Deliniere, Antoine; Pruvot, Etienne; Gruner, Christiane; Carbucicchio, Corrado; Manka, Robert; Russo, Antonio Dello; Tondo, Claudio; Brunckhorst, Corinna; Tanner, Felix; Duru, Firat; Saguner, Ardan M (2021). Differentiating hereditary arrhythmogenic right ventricular cardiomyopathy from cardiac sarcoidosis fulfilling 2010 ARVC Task Force Criteria. Heart Rhythm, 18(2):231-238.

Abstract

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 $^{18}$F-fluorodeoxyglucose positron emission tomographic ($^{18}$F-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 $^{18}$F-FDG PET scan should raise the suspicion of CS, whereas larger RVOT dimensions and peripheral TWI favor a diagnosis of hereditary ARVC.

Abstract

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 $^{18}$F-fluorodeoxyglucose positron emission tomographic ($^{18}$F-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 $^{18}$F-FDG PET scan should raise the suspicion of CS, whereas larger RVOT dimensions and peripheral TWI favor a diagnosis of hereditary ARVC.

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Additional indexing

Item Type:Journal Article, refereed, original work
Communities & Collections:04 Faculty of Medicine > University Hospital Zurich > Clinic for Cardiology
Dewey Decimal Classification:610 Medicine & health
Scopus Subject Areas:Health Sciences > Cardiology and Cardiovascular Medicine
Health Sciences > Physiology (medical)
Language:English
Date:1 February 2021
Deposited On:01 Feb 2021 17:12
Last Modified:02 Feb 2021 21:01
Publisher:Elsevier
ISSN:1547-5271
OA Status:Closed
Publisher DOI:https://doi.org/10.1016/j.hrthm.2020.09.015
PubMed ID:32976989

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