Endomyocardial biopsy: The forgotten piece in the arrhythmogenic cardiomyopathy puzzle

Michela Casella, Marco Bergonti, Antonio Dello Russo, Riccardo Maragna, Alessio Gasperetti, Paolo Compagnucci, Valentina Catto, Filippo Trombara, Antonio Frappampina, Edoardo Conte, Marco Fogante, Elena Sommariva, Stefania Rizzo, Monica De Gaspari, Andrea Giovagnoni, Daniele Andreini, Giulio Pompilio, Luigi Di Biase, Andrea Natale, Cristina BassoClaudio Tondo

Research output: Contribution to journalArticlepeer-review

6 Scopus citations


BACKGROUND: Endomyocardial biopsy (EMB) is part of 2010 Task Force Criteria (TFC) for arrhythmogenic right ventricular cardiomyopathy (ARVC). However, its usage has been curtailed because of its low presumed diagnostic yield, and it is now a poorly used tool. This study aims to analyze the contribution of EMB to the final diagnosis of ARVC. METHODS AND RESULTS: We included 104 consecutive patients evaluated for a suspicion of ARVC, who were referred for EMB. Patients with suspected left dominant pattern were excluded from the primary analysis. Subjects were initially stratified ac-cording to TFC without considering EMB. After EMB, patients were reclassified accordingly, and the reclassification rate was calculated. EMB yielded a diagnostic finding in 92 patients (85.5%). After including EMB evaluation, 20 (43%) more patients “at risk” received a definite diagnosis of ARVC. Overall, 59 patients received a definite diagnosis of ARVC, 34% only after EMB. EMB appeared to be the better-performing exam with respect to the final diagnosis (β, 2.2; area uder the curve, 0.73; P<0.05). The reclassification improvement after EMB measured 28%. TFC score increased from 3.5±1.3 to 4.3±1.4 (P<0.001). Notably, active inflammation was present in 6 (10%) patients. Minor complications were reported in only 2% of the cohort. In patients with suspected left-dominant disease, conventional TFC performed poorly. CONCLUSIONS: Electroanatomic voltage mapping– guided EMB was safe and yielded an optimal diagnostic yield. It allowed up-grading of the diagnosis of nearly one-third of the patients considered “at risk.” Classical TFC without EMB performed poorly in patients with the left dominant form of ARVC.

Original languageEnglish (US)
Article numbere021370
JournalJournal of the American Heart Association
Issue number19
StatePublished - Oct 2021


  • Arrhythmogenic cardiomyopathy
  • Cardiac magnetic resonance
  • Electroanatomic mapping
  • Endomyocardial biopsies
  • Right ventricular arrhythmogenic cardiomyopathy
  • Task force criteria

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine


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