title: Long-term follow-up and risk assessment of arrhythmogenic right ventricular dysplasia/cardiomyopathy: personal experience from different primary and tertiary centres.
abstract: OBJECTIVE
  Limited data are available on the course of arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) because of the low frequency of this diagnosis. A long-term follow-up was analysed in typical ARVD/C patients from different primary and tertiary centres, and risk factors for sudden cardiac death (SCD) and end-stage heart failure were assessed.
  
  
  RESULTS
  A total of 313 patients (197 males) with a mean age of 44.8 +/- 16.5 years were included, with symptoms including aborted SCD (7%), ventricular arrhythmias (47%), additional chest pain (42%), syncopes (17%), and atrial arrhythmias (12%); follow-up duration was 8.5 years. The total annual mortality rate was 0.3%, from SCD in 0.2% and heart failure in 0.1%. In symptomatic or high-risk patients, the annual rate of malignant ventricular arrhythmias was 6 and 9%, respectively. In multivariate analysis, a risk factor for SCD was left ventricular dysfunction. The annual heart failure rate was low at 0.5%; four patients died, two had heart transplants and one tricuspid reconstruction. Intrathoracic cardioverter-defibrillator (ICD) therapy was initiated in 35 patients. Adequate shocks after 6-72 months were delivered in 77%; in the majority aborted sudden death with documented ventricular fibrillation and unstable ventricular tachycardia were underlying arrhythmias.
  
  
  CONCLUSIONS
  The clinical course of ARVD/C is characterized by a high rate of recurrent malignant ventricular arrhythmias in initially symptomatic and high-risk cases, and is uneventful in primarily asymptomatic affected individuals. The spectrum of clinical symptoms represents a warning symptom initiating the so-called 'hot phase' of the disease in many cases. ICD treatment is highly effective in cases of aborted SCD and unstable ventricular tachycardia.
authors.affiliation: Department of Cardiology, Academic Teaching Hospital of Quedlinburg, University of Magdeburg, Quedlinburg, Germany. s.peters@klinikum-quedlinburg.de
authors.forename: Stefan
authors.initials: S
authors.lastname: Peters
mesh_terms: D000293:Adolescent; D000319:Adrenergic beta-Antagonists / Q000627:therapeutic use; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000806:Angiotensin-Converting Enzyme Inhibitors / Q000627:therapeutic use; D019571:Arrhythmogenic Right Ventricular Dysplasia / Q000150:complications / Q000401:mortality* / Q000628:therapy*; D002648:Child; D016757:Death, Sudden, Cardiac / Q000453:epidemiology* / Q000517:prevention & control; D017147:Defibrillators, Implantable; D004232:Diuretics / Q000627:therapeutic use; D004562:Electrocardiography; D005260:Female; D005500:Follow-Up Studies; D005858:Germany / Q000453:epidemiology; D006333:Heart Failure / Q000453:epidemiology* / Q000628:therapy; D016027:Heart Transplantation / Q000706:statistics & numerical data; D006801:Humans; D008297:Male; D008875:Middle Aged; D015999:Multivariate Analysis; D012189:Retrospective Studies; D018570:Risk Assessment; D013575:Syncope / Q000150:complications; D014261:Tricuspid Valve / Q000601:surgery; D018487:Ventricular Dysfunction, Left / Q000150:complications
chemical_list: D000319:Adrenergic beta-Antagonists; D000806:Angiotensin-Converting Enzyme Inhibitors; D004232:Diuretics