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Left Ventricular Dysfunction in Arrhythmogenic Cardiomyopathy: Association With Exercise Exposure, Genetic Basis, and

Øyvind H Lie1,2, Monica Chivulescu1,2, Christine Rootwelt-Norberg1,2

  • 1Department of Cardiology Oslo University Hospital, Rikshospitalet Norway.

Journal of the American Heart Association
|April 6, 2021
PubMed

Insights

Greater exercise is linked to worse left ventricular (LV) function in arrhythmogenic cardiomyopathy (AC) patients at diagnosis. LV function decline predicts arrhythmias, especially in desmoplakin genotype carriers.

Area of Science:

  • Cardiology
  • Genetics
  • Cardiovascular Imaging

Background:

  • Arrhythmogenic cardiomyopathy (AC) presents with biventricular dysfunction, exercise intolerance, and high risk of ventricular tachyarrhythmias and sudden death.
  • Factors influencing left ventricular (LV) disease manifestation and prognosis in AC remain incompletely understood.
  • Left ventricular dysfunction is a critical determinant of adverse outcomes in AC.

Purpose of the Study:

  • To investigate the association between exercise exposure and genotype with LV dysfunction in AC.
  • To determine the impact of LV disease progression on the risk of adverse arrhythmic events.
  • To identify predictors of LV dysfunction progression and subsequent ventricular tachyarrhythmias in AC.

Main Methods:

  • Longitudinal study of 168 AC patients with comprehensive echocardiographic follow-up (7.6 years).
  • Assessment of LV function using global longitudinal strain (GLS) and correlation with exercise data.
  • Analysis of genetic data, focusing on desmoplakin genotype, and its interaction with LV function progression.

Main Results:

  • Higher exercise exposure correlated with worse baseline LV function (GLS worsening per 5 MET-hours/week, P=0.02).
  • LV function significantly worsened over time (0.08% per year, P<0.001), with accelerated progression in desmoplakin genotype patients (P<0.001).
  • Deterioration in LV function predicted incident ventricular tachyarrhythmias (OR 1.1 per 1% GLS worsening, P=0.02).

Conclusions:

  • Exercise exposure is associated with baseline LV dysfunction but not the rate of LV progression in AC.
  • LV dysfunction progression is most pronounced in patients with desmoplakin genotypes.
  • Monitoring LV function deterioration is crucial for risk stratification of ventricular tachyarrhythmias in AC.

Keywords:
arrhythmogenic cardiomyopathyarrhythmogenic right ventricular cardiomyopathyleft ventricular dysfunctionventricular arrhythmia

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