title: Angiotensin-converting enzyme gene polymorphism in arrhythmogenic right ventricular dysplasia: is DD genotype helpful in predicting syncope risk?
abstract: BACKGROUND
  Arrhythmogenic right ventricular dysplasia (ARVD) is a heritable disorder characterised by fibrofatty replacement of right ventricular myocytes and increased risk of ventricular arrhythmias and sudden cardiac death. Angiotensin-converting enzyme (ACE) gene insertion/deletion (I/D) polymorphism affects myocardial ACE levels. DD genotype favours myocardial fibrosis and is associated with malignant ventricular tachycardia. The aim of this study was to explore ACE gene polymorphism in ARVD patients.
  
  
  METHODS
  Twenty-nine patients with ARVD and 24 controls were included. All ARVD patients had documented sustained ventricular tachycardia. Thirteen patients had syncopal episodes. Six patients were resuscitated from sudden cardiac death. ACE gene polymorphism was identified by polymerase chain reaction technique.
  
  
  RESULTS
  There was no significant difference in DD genotype frequency between ARVD patients and controls (44.8% vs. 45.8%, p=0.94). However, DD genotype frequency was significantly higher in ARVD patients with syncopal episodes compared to those without syncope (69.2% vs. 25.0%, p=0.017, odds ratio:6.750, 95% confidence interval: 1.318-34.565). DD genotype was detected in higher frequency also in patients with a family history of sudden cardiac death (66.7% vs. 39.1%,p=0.36).
  
  
  CONCLUSIONS
  High prevalence of DD genotype in ARVD patients with syncope suggests that ACE I/D polymorphism might be useful in identifying high-risk patients for syncope.
authors.affiliation: Department of Cardiology, Faculty of Medicine, Marmara University, Istanbul, Turkey. bestes@doctor.com
authors.forename: Beste
authors.initials: B
authors.lastname: Ozben
authors.forename: Ibrahim
authors.initials: I
authors.lastname: Altun
authors.forename: Veysel
authors.initials: V
authors.lastname: Sabri Hancer
authors.forename: Ahmet Kaya
authors.initials: AK
authors.lastname: Bilge
authors.forename: Azra Meryem
authors.initials: AM
authors.lastname: Tanrikulu
authors.forename: Reyhan
authors.initials: R
authors.lastname: Diz-Kucukkaya
authors.forename: Ali Serdar
authors.initials: AS
authors.lastname: Fak
authors.forename: Ercument
authors.initials: E
authors.lastname: Yilmaz
authors.forename: Kamil
authors.initials: K
authors.lastname: Adalet
mesh_terms: D000328:Adult; D000483:Alleles; D019571:Arrhythmogenic Right Ventricular Dysplasia / Q000201:enzymology* / Q000235:genetics*; D005260:Female; D020022:Genetic Predisposition to Disease; D006801:Humans; D008297:Male; D007703:Peptidyl-Dipeptidase A / Q000235:genetics*; D011110:Polymorphism, Genetic; D013575:Syncope / Q000150:complications*
chemical_list: D007703:Peptidyl-Dipeptidase A