title: Prevalence of atrial arrhythmias in arrhythmogenic right ventricular dysplasia/cardiomyopathy.
abstract: BACKGROUND
  Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is an inherited cardiomyopathy, characterized by right ventricular dysfunction and ventricular arrhythmias. Limited information is available concerning atrial arrhythmias in ARVD/C.
  
  
  OBJECTIVE
  The purpose of this study was to characterize spontaneous atrial arrhythmias in a large registry population of ARVD/C patients.
  
  
  METHODS
  Patients (n = 248) from the Johns Hopkins ARVD/C registry who met the diagnostic criteria and had undertaken genotype analysis were included. Medical records of each were reviewed to ascertain incidence and characteristics of atrial arrhythmia episodes. Detailed demographic, phenotypic, and structural information was obtained from registry data.
  
  
  RESULTS
  Thirty-five patients with ARVD/C (14%) experienced one or more types of atrial arrhythmia during median follow-up of 5.78 (interquartile range 8.52) years. Atrial fibrillation was the most common atrial arrhythmia, occurring in 80% of ARVD/C patients with atrial arrhythmias. Patients developed atrial arrhythmias at a mean age of 43.0 ± 14.0 years. Atrial arrhythmia patients obtained a total of 22 inappropriate implantable cardioverter-defibrillator shocks during follow-up. Older age at last follow-up (P <.001) and male gender (P = .044) were associated with atrial arrhythmia development. Patients with atrial arrhythmias had a higher occurrence of death (P = .028), heart failure (P <.001), and left atrial enlargement on echocardiography (P = .004).
  
  
  CONCLUSIONS
  Atrial arrhythmias are common in ARVD/C and present at a younger age than in the general population. They are associated with male gender, increasing age, and left atrial enlargement. Atrial arrhythmias are clinically important as they are associated with inappropriate implantable cardioverter-defibrillator shocks and increased risk of both death and heart failure.
authors.affiliation: New College, University of Oxford, Oxford, United Kingdom.
authors.forename: Christian F
authors.initials: CF
authors.lastname: Camm
authors.forename: Cynthia A
authors.initials: CA
authors.lastname: James
authors.forename: Crystal
authors.initials: C
authors.lastname: Tichnell
authors.forename: Brittney
authors.initials: B
authors.lastname: Murray
authors.forename: Aditya
authors.initials: A
authors.lastname: Bhonsale
authors.forename: Anneline S J M
authors.initials: AS
authors.lastname: te Riele
authors.forename: Daniel P
authors.initials: DP
authors.lastname: Judge
authors.forename: Harikrishna
authors.initials: H
authors.lastname: Tandri
authors.forename: Hugh
authors.initials: H
authors.lastname: Calkins
mesh_terms: D000328:Adult; D019571:Arrhythmogenic Right Ventricular Dysplasia / Q000150:complications* / Q000503:physiopathology; D001281:Atrial Fibrillation / Q000453:epidemiology* / Q000209:etiology / Q000503:physiopathology; D002593:Cetrimonium Compounds; D004338:Drug Combinations; D004562:Electrocardiography; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D015994:Incidence; D008297:Male; D008396:Maryland / Q000453:epidemiology; D008875:Middle Aged; D009226:Myristates; D009539:Nicotinic Acids; D012189:Retrospective Studies; D018570:Risk Assessment / Q000379:methods*; D012307:Risk Factors; D012841:Simethicone; D013229:Stearic Acids
chemical_list: D002593:Cetrimonium Compounds; D004338:Drug Combinations; D009226:Myristates; D009539:Nicotinic Acids; D013229:Stearic Acids; C058060:Prevasore; D012841:Simethicone
keywords: 12-lead electrocardiogram; AF; ARVD/C; Arrhythmogenic right ventricular cardiomyopathy; Arrhythmogenic right ventricular dysplasia; Atrial arrhythmia; Atrial fibrillation; ECG; ICD; IQR; PKP2; SVT; TFC; VF; VT; arrhythmogenic right ventricular dysplasia/cardiomyopathy; atrial fibrillation; implantable cardioverter-defibrillator; interquartile range; plakophilin-2; supraventricular tachycardia; task force criteria; ventricular fibrillation; ventricular tachycardia