title: Clinical characterisation and risk stratification of patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy ≥50 years of age.
abstract: OBJECTIVE
  With the increased use of genetic testing for arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C), this disease is being increasingly recognised among elderly patients. However, elderly ARVD/C patients were underrepresented in prior cohorts. We aimed to describe the phenotypical characteristics and outcomes among ARVD/C patients surviving ≥50 years.
  
  
  METHODS
  We assessed detailed phenotypical data of 29 patients who (1) presented at ≥50 years of age; and (2) fulfilled 2010 Task Force Criteria (TFC) for ARVD/C by last follow-up. Primary outcome was the occurrence of a major ventricular arrhythmia (sudden cardiac death, resuscitated sudden cardiac arrest or sustained ventricular tachycardia).
  
  
  RESULTS
  The majority (55 %) of elderly ARVD/C subjects were male, with a mean age of 59.0 ± 5.8 years at presentation. Study participants fulfilled a median of six (IQR 5-8) TFC criteria by last follow-up, of which arrhythmia criteria were most frequent (97 %), followed by structural criteria (83 %), depolarisation criteria (72 %) and repolarisation criteria (69 %). By last follow-up, 15 (52 %) patients had experienced major ventricular arrhythmias. Most patients (n = 12) presented with this arrhythmia, while three experienced the event during 5.4 ± 3.2 years of follow-up. Compared with patients without an arrhythmic event, patients with major arrhythmias were more likely to be proband (p < 0.001) and male (p = 0.042). Likewise, survival free from sustained ventricular arrhythmia was lower among probands and males.
  
  
  CONCLUSIONS
  Phenotypic characteristics of elderly ARVD/C patients are characterised by depolarisation abnormalities and structural cardiac changes. Ventricular arrhythmias in this elderly cohort are associated with male gender and proband status.
authors: Department of Cardiology, University Medical Center Utrecht, Utrecht, The Netherlands.
authors: M J
authors: MJ
authors: van der Pols
authors: Department of Cardiology, University Medical Center Utrecht, Utrecht, The Netherlands.
authors: T P
authors: TP
authors: Mast
authors: Department of Cardiology, University Medical Center Utrecht, Utrecht, The Netherlands.
authors: P
authors: P
authors: Loh
authors: Department of Cardiology, University Medical Center Utrecht, Utrecht, The Netherlands.
authors: J F
authors: JF
authors: van der Heijden
authors: Department of Cardiology, University Medical Center Utrecht, Utrecht, The Netherlands.
authors: M J
authors: MJ
authors: Cramer
authors: Netherlands Heart Institute, Utrecht, The Netherlands.
authors: R N
authors: RN
authors: Hauer
authors: Department of Cardiology, University Medical Center Utrecht, Utrecht, The Netherlands. ariele3@umcutrecht.nl.|Department of Cardiology, Johns Hopkins University School of Medicine, Baltimore, MD, USA. ariele3@umcutrecht.nl.|Netherlands Heart Institute, Utrecht, The Netherlands. ariele3@umcutrecht.nl.
authors: A S J M
authors: AS
authors: Te Riele
keywords: Arrhythmogenic right ventricular dysplasia; Cardiomyopathy; Clinical phenotype; Elderly; Ventricular arrhythmia