title: Arrhythmogenic right ventricular cardiomyopathy/dysplasia in Saudi Arabia: a single-center experience with long-term follow-up.
abstract: OBJECTIVE
  Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is a rare genetic disorder that primarily involves the right ventricle (RV). It is characterized by progressive replacement of RV myocardium by fibrofatty tissues. It commonly presents with ventricular tachycardia (VT) of RV origin and may result in RV failure. The aim of this study is to evaluate the clinical characteristics of adult patients with ARVC/D treated at the Heart Centre, King Faisal Specialist Hospital and Research Centre (KFSH&RC), Riyadh, Saudi Arabia.
  
  
  METHODS
  This is a retrospective study of patients with ARVC/D diagnosed and treated at the KFSH&RC Heart Centre in Riyadh.
  
  
  METHODS
  Twenty-two cases with ARVC/D with regular follow-up at our Heart Centre from January 2007 to May 2010 were included in this study. The diagnosis of ARVC/D was made according to the revised International Task Force Criteria. The clinical data were collected from patients' charts and electronic medical records.
  
  
  RESULTS
  The majority of patients were males (18; 82%). The diagnosis of ARVC/D was definite in 18 patients (82%), borderline in 2 (9%), and possible in 2 (9%). The mean age at diagnosis was 33.3 years. The follow-up period ranged from 29 to 132 months, with a mean follow-up period of 84 months. Ten patients presented with sustained VT, and 3 were survivors of cardiac arrest. Electrocardiogram abnormalities were present in 16/22 patients (72.7%). Echocardiographic changes meeting major diagnostic criteria were seen in 16 patients (76%). Cardiac magnetic resonance imaging was performed in 11 patients, and showed changes compatible with major diagnostic criteria in 7 patients (64%). Implantable cardioverter defibrillators (ICDs) were implanted in 17 patients; 8 had appropriate ICD shocks and 5 had inappropriate ICD shocks. Antitachycardia pacing was effective in terminating most of the VT/ventricular fibrillation episodes.
  
  
  CONCLUSIONS
  ARVC/D is a rare but increasingly recognized heart muscle disease seen in Saudi Arabia and other parts of the world. It is associated with a highly nonspecific presentation. VT of RV origin is a common presentation for this disease. Antiarrhythmic medications and ICD implantation are the main management options.
authors.affiliation: Dr. Bandar Al-Ghamdi, MBC 16 Heart Centre, King Faisal Specialist Hospital and Research Centre, PO Box 3354, Riyadh 11211, Saudi Arabia, T: +966-11-442-4838, balghamdi@kfshrc.edu.sa.
authors.forename: Bandar
authors.initials: B
authors.lastname: Al-Ghamdi
authors.forename: Azam
authors.initials: A
authors.lastname: Shafquat
authors.forename: Yaseen
authors.initials: Y
authors.lastname: Mallawi
mesh_terms: D000293:Adolescent; D000328:Adult; D000889:Anti-Arrhythmia Agents / Q000627:therapeutic use; D019571:Arrhythmogenic Right Ventricular Dysplasia / Q000150:complications / Q000175:diagnosis* / Q000628:therapy*; D017115:Catheter Ablation; D017147:Defibrillators, Implantable; D004452:Echocardiography; D004562:Electrocardiography; D005260:Female; D005500:Follow-Up Studies; D005820:Genetic Testing; D006323:Heart Arrest / Q000188:drug therapy; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D017410:Practice Guidelines as Topic; D012189:Retrospective Studies; D012529:Saudi Arabia; D017180:Tachycardia, Ventricular / Q000209:etiology*; D013997:Time Factors; D055815:Young Adult
chemical_list: D000889:Anti-Arrhythmia Agents
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