Differentiating hereditary arrhythmogenic right ventricular cardiomyopathy from cardiac sarcoidosis fulfilling 2010

Alessio Gasperetti1, Valentina A Rossi1, Alessandra Chiodini1

  • 1University Heart Center, University Hospital Zurich, Switzerland.

Heart Rhythm
|September 25, 2020
PubMed

Insights

Diagnosing cardiac sarcoidosis (CS) and arrhythmogenic right ventricular cardiomyopathy (ARVC) is challenging. Specific clinical findings and 18F-FDG PET scans help differentiate these conditions, improving diagnostic accuracy.

Area of Science:

  • Cardiology
  • Medical Imaging
  • Genetics

Background:

  • Cardiac sarcoidosis (CS) and arrhythmogenic right ventricular cardiomyopathy (ARVC) share overlapping clinical presentations.
  • Distinguishing between CS and ARVC is crucial for appropriate patient management.

Purpose of the Study:

  • To identify clinical variables that effectively differentiate patients with CS from those with genetically confirmed ARVC.
  • To evaluate the utility of the 2010 ARVC Task Force Criteria (TFC) in distinguishing these two conditions.

Main Methods:

  • A multicenter study matched 10 CS patients (fulfilling 2010 ARVC TFC) with 10 genetically proven ARVC patients.
  • Cardiac 18F-fluorodeoxyglucose positron emission tomographic (18F-FDG PET) scans were mandatory for study inclusion.

Main Results:

  • The 2010 ARVC TFC did not reliably differentiate between CS and ARVC.
  • CS patients showed longer PR intervals, advanced atrioventricular block, longer QRS duration, reduced LVEF, and more left ventricular involvement.
  • ARVC patients had more peripheral T-wave inversions and larger RVOT dimensions; 90% of CS patients had positive 18F-FDG PET scans, while 0% of ARVC patients did.

Conclusions:

  • The 2010 ARVC TFC are insufficient for differentiating CS from hereditary ARVC.
  • Prolonged PR interval, advanced AVB, longer QRS, reduced LVEF, and positive 18F-FDG PET scans suggest CS.
  • Larger RVOT, subtricuspid involvement, and peripheral TWI favor ARVC.
Abstract

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