Heart Failure Is Common and Under-Recognized in Patients With Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia

Circ Heart Fail. 2017 Sep;10(9):e003819. doi: 10.1161/CIRCHEARTFAILURE.116.003819.


Background: Heart failure (HF) prevalence in arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) varies depending on study cohort and is not well characterized. This study sought to determine prevalence and predictors of HF in ARVC/D.

Methods and results: Clinical HF, defined as at least 1 HF sign or symptom, was retrospectively adjudicated for 289 patients meeting ARVC/D Task Force Criteria. HF was present in 142 patients (49%): 113 had isolated RV involvement and 29 had evidence of LV dysfunction. Average age of HF onset was 40±14 years. Most commonly reported symptoms were exertional dyspnea (78%) and fatigue (73%). Only 40% (n=57/142) had signs of volume overload. Left-sided HF signs were rare. Patients with clinical HF before ARVC/D diagnosis (n=31) were older (P=0.005) and met fewer Task Force Criteria (P=0.013) than those who developed HF after ARVC/D presentation. Female sex (odds ratio, 2.2; 95% confidence interval, 1.21-4.01; P=0.01) and lateral precordial T-wave inversions (odds ratio, 9.87; 95% confidence interval, 1.07-91.1; P=0.043) were associated with increased odds of HF. Additionally, patients with symptomatic LV dysfunction had higher odds of lateral precordial T-wave inversions (odds ratio, 18.4; 95% confidence interval, 2.92-116.18; P=0.002). Patients with HF were more likely to undergo heart transplantation (15/142 versus 1/147; P<0.001) or die during study follow-up period (7 versus 0; P=0.007).

Conclusions: HF symptoms, especially exertional dyspnea, are common in ARVC/D; yet, classic left-sided signs are typically absent and less than half have evidence of volume overload. Given the unique predominately right-sided phenotype, a large portion of patients with HF may be under-recognized.

Keywords: arrhythmias, cardiac; cardiomyopathies; dyspnea; fatigue; heart failure.

MeSH terms

  • Adult
  • Arrhythmogenic Right Ventricular Dysplasia / complications*
  • Arrhythmogenic Right Ventricular Dysplasia / diagnosis
  • Arrhythmogenic Right Ventricular Dysplasia / physiopathology
  • Echocardiography
  • Electrocardiography
  • Female
  • Follow-Up Studies
  • Heart Conduction System / physiopathology*
  • Heart Failure / epidemiology
  • Heart Failure / etiology*
  • Heart Failure / physiopathology
  • Humans
  • Magnetic Resonance Imaging, Cine
  • Male
  • Prevalence
  • Retrospective Studies
  • Risk Factors
  • United States / epidemiology
  • Ventricular Function, Left / physiology*