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Atrial Dysfunction in Arrhythmogenic Right Ventricular Cardiomyopathy.

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

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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.