title: Angiotensin-converting enzyme gene polymorphisms and prognosis in chronic thromboembolic pulmonary hypertension.
abstract: BACKGROUND
  Angiotensin-converting enzyme (ACE) plays an important role in vascular remodeling in pulmonary hypertension, and ACE gene polymorphism is associated with exercise-induced pulmonary hypertension in Japanese patients with chronic obstructive pulmonary disease. The present study was designed to investigate if ACE-insertion (I)/deletion (D) polymorphism might be related to the susceptibility, severity, and disease outcome in chronic thromboembolic pulmonary hypertension (CTEPH).
  
  
  RESULTS
  ACE-I/D genotypes were determined in 95 consecutive CTEPH patients (46 underwent surgery, 49 received medical treatment) and 97 controls. The frequencies of genotypes and alleles were not significantly different between patients and controls. Clinical characteristics were compared among ACE genotypes (II, ID, DD). ACE D allele carrier (ID plus DD) was associated with a lower 6-min walk test distance compared with D allele non-carrier (II) (330+/-102 (mean +/- SD) vs 381 +/-85 m, p=0.046). Kaplan-Meier analysis in the medically treated group showed significantly deteriorated survival for D allele carriers compared with D allele non-carriers (p=0.0389). Multivariate analysis revealed that age (p=0.013), pulmonary vascular resistance (p=0.008), and D allele carrier status (p=0.021) were independent predictors of survival.
  
  
  CONCLUSIONS
  ACE D allele carrier is possibly one of the prognostic factors for medically treated CTEPH patients.
authors.affiliation: Department of Respirology, Graduate School of Medicine, Chiba University, Japan. ntanabe@faculty.chiba-u.jp
authors.forename: Nobuhiro
authors.initials: N
authors.lastname: Tanabe
authors.forename: Shinya
authors.initials: S
authors.lastname: Amano
authors.forename: Koichiro
authors.initials: K
authors.lastname: Tatsumi
authors.forename: Satoshi
authors.initials: S
authors.lastname: Kominami
authors.forename: Natsuhiko
authors.initials: N
authors.lastname: Igarashi
authors.forename: Ryuhi
authors.initials: R
authors.lastname: Shimura
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Matsubara
authors.forename: Yasunori
authors.initials: Y
authors.lastname: Kasahara
authors.forename: Yuichi
authors.initials: Y
authors.lastname: Takiguchi
authors.forename: Takayuki
authors.initials: T
authors.lastname: Kuriyama
mesh_terms: D000328:Adult; D000483:Alleles; D044466:Asian People; D002908:Chronic Disease; D005260:Female; D005787:Gene Frequency; D020022:Genetic Predisposition to Disease; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology / Q000235:genetics*; D008297:Male; D008875:Middle Aged; D007703:Peptidyl-Dipeptidase A / Q000235:genetics*; D011110:Polymorphism, Genetic; D011379:Prognosis; D029424:Pulmonary Disease, Chronic Obstructive / Q000150:complications / Q000235:genetics; D011655:Pulmonary Embolism / Q000209:etiology / Q000235:genetics*; D012307:Risk Factors
chemical_list: C488034:ACE protein, human; D007703:Peptidyl-Dipeptidase A