title: Left Ventricular Involvement in Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy Assessed by Echocardiography Predicts Adverse Clinical Outcome.
abstract: BACKGROUND
  Among studies describing the phenotype of arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C), significant discrepancy exists regarding the extent and impact of left ventricular (LV) involvement. The capability of conventional and new quantitative echocardiographic techniques to accurately detect LV involvement in ARVD/C remains unknown. The aim of this study was to test the hypothesis that accurate detection of LV involvement on echocardiography identifies patients at additional risk for cardiac events during follow-up.
  
  
  METHODS
  Thirty-eight patients with ARVD/C, 16 pathogenic mutation-positive relatives, and 55 healthy control subjects were prospectively enrolled. Conventional echocardiography with additional deformation imaging was performed in all subjects to detect LV involvement. In a subgroup (n = 27), cardiac magnetic resonance imaging was performed with late enhancement. All patients and relatives were prospectively followed for events (sustained ventricular tachycardia, appropriate implantable cardioverter-defibrillator intervention, sudden cardiac death, and heart transplantation).
  
  
  RESULTS
  Conventional echocardiography detected LV involvement in 32% of patients with ARVD/C and in none of the relatives. Deformation imaging revealed LV involvement in 68% of patients with ARVD/C and 25% of relatives and was correlated closely with late enhancement on cardiac magnetic resonance imaging. During a mean follow-up period of 5.9 ± 2.3 years, 20 patients with ARVD/C (53%) experienced events, and no events occurred in the relatives. LV involvement detected by deformation imaging (hazard ratio, 4.9; 95% CI, 1.7-14.2) and right ventricular outflow tract enlargement (hazard ratio, 1.2; 95% CI, 1.1-1.3) were the only independent predictors of outcomes.
  
  
  CONCLUSIONS
  Deformation imaging detected a high incidence of LV involvement in patients with ARVD/C and their relatives. Compared with conventional echocardiography, deformation imaging is superior in detecting minor LV involvement. LV involvement and an enlarged right ventricular outflow tract are independent prognostic markers of outcomes.
authors.affiliation: Department of Cardiology, University Medical Center Utrecht, Utrecht, The Netherlands.
authors.forename: Thomas P
authors.initials: TP
authors.lastname: Mast
authors.affiliation: Department of Cardiology, University Medical Center Utrecht, Utrecht, The Netherlands. Electronic address: a.j.teske-2@umcutrecht.nl.
authors.forename: Arco J
authors.initials: AJ
authors.lastname: Teske
authors.affiliation: Department of Cardiology, University Medical Center Utrecht, Utrecht, The Netherlands.
authors.forename: Jeroen F
authors.initials: JF
authors.lastname: vd Heijden
authors.affiliation: Department of Cardiology, University Medical Center Utrecht, Utrecht, The Netherlands.
authors.forename: Judith A
authors.initials: JA
authors.lastname: Groeneweg
authors.affiliation: Department of Cardiology, University Medical Center Utrecht, Utrecht, The Netherlands.
authors.forename: Anneline S J M
authors.initials: AS
authors.lastname: Te Riele
authors.affiliation: Department of Radiology, University Medical Center Utrecht, Utrecht, The Netherlands.
authors.forename: Birgitta K
authors.initials: BK
authors.lastname: Velthuis
authors.affiliation: ICIN - Netherlands Heart Institute, Utrecht, The Netherlands.
authors.forename: Richard N W
authors.initials: RN
authors.lastname: Hauer
authors.affiliation: Department of Cardiology, University Medical Center Utrecht, Utrecht, The Netherlands.
authors.forename: Pieter A
authors.initials: PA
authors.lastname: Doevendans
authors.affiliation: Department of Cardiology, University Medical Center Utrecht, Utrecht, The Netherlands.
authors.forename: Maarten J
authors.initials: MJ
authors.lastname: Cramer
mesh_terms: D000328:Adult; D019571:Arrhythmogenic Right Ventricular Dysplasia / Q000000981:diagnostic imaging* / Q000473:pathology / Q000503:physiopathology; D016757:Death, Sudden, Cardiac / Q000453:epidemiology; D018450:Disease Progression; D004452:Echocardiography / Q000379:methods*; D005260:Female; D005500:Follow-Up Studies; D006352:Heart Ventricles / Q000000981:diagnostic imaging* / Q000473:pathology / Q000503:physiopathology; D006801:Humans; D015994:Incidence; D019028:Magnetic Resonance Imaging, Cine; D008297:Male; D008875:Middle Aged; D009426:Netherlands / Q000453:epidemiology; D011237:Predictive Value of Tests; D012189:Retrospective Studies; D016277:Ventricular Function, Left / Q000502:physiology*
keywords: ARVD/C; Arrhythmogenic right ventricular cardiomyopathy; Deformation imaging; Echocardiography; LV involvement; Prognosis