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Atrial Dysfunction in Arrhythmogenic Right Ventricular Cardiomyopathy

Tarek Zghaib1, Mimount Bourfiss2, Jeroen F van der Heijden2

  • 1Division of Cardiology, Johns Hopkins University School of Medicine, Baltimore, MD (T.Z., H.T., H.C.).

Circulation. Cardiovascular Imaging
|October 26, 2018
PubMed

Insights

Arrhythmogenic right ventricular cardiomyopathy (ARVC) patients show enlarged atria and reduced atrial function. Atrial size and function predict future atrial arrhythmias (AA) in ARVC.

Area of Science:

  • Cardiology
  • Cardiovascular Imaging
  • Genetics

Background:

  • Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited heart muscle disease primarily affecting the ventricles.
  • Limited evidence exists regarding atrial involvement in ARVC.
  • Investigating atrial changes in ARVC is crucial for understanding disease progression and predicting arrhythmias.

Purpose of the Study:

  • To characterize atrial involvement in ARVC using functional cardiac magnetic resonance (CMR).
  • To determine the relationship between atrial size/function and ventricular variables.
  • To identify CMR-based predictors of atrial arrhythmias (AA) in ARVC patients.

Main Methods:

  • Analysis of cine CMR images from 66 ARVC patients and 24 controls.
  • Evaluation of biatrial volumes, ejection fractions, peak longitudinal strain, and strain rates using tissue tracking.
  • Prospective follow-up for the occurrence of AA over a median of 6.8 years.

Main Results:

  • ARVC patients exhibited significantly higher biatrial volumes compared to controls.
  • Reduced right atrial (RA) conduit function, RA and left atrial (LA) reservoir function, and RA and LA pump function were observed in ARVC patients.
  • Higher atrial volumes and impaired LA reservoir and RA conduit function predicted incident AA in multivariable analysis.

Conclusions:

  • ARVC patients demonstrate enlarged atria with diminished function on functional CMR.
  • Atrial parameters, including size and function, are significant predictors of incident AA in ARVC.
  • These findings highlight the importance of atrial involvement in ARVC pathophysiology and arrhythmia risk.
Abstract

Keywords:
atrial fibrillationcardiomyopathyheart failurepainthrombosis

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