title: Initial clinical manifestations and mid- and long-term results after surgical repair of double-chambered right ventricle in children and adults.
abstract: OBJECTIVE
  By means of retrospective analysis of our institutional experience, we reviewed the clinical manifestation and outcomes of patients subsequent to surgical repair of double-chambered right ventricle.
  
  
  METHODS
  Between 1988 and 2005, we performed surgical repair in 35 of 37 patients diagnosed with double-chambered right ventricle. The patients ranged in age from 4 to 69 years, with a mean of 21.3 years. Most presented in infancy, with initial manifestation of a short systolic murmur in 34 (92%) of all cases. Pressure gradients were measured invasively across the right ventricular outflow tract of between 30 and 140 mmHg, with a median of 60 mmHg. An associated ventricular septal defect was present in 26 patients (70%). Of the group, 4 patients were aged over 40 years, and 2 had previously undergone operative closure of a ventricular septal defect.
  
  
  RESULTS
  The operative interval ranged from 2 months to 41 years, with a median of 9 years. In all, we resected muscular bundles and enlarged the right ventricular outflow tract. There was no hospital or late death. Median follow-up subsequent to surgery was 7 years, with a range from 0.4 to 11 years. No patient required further surgery to relieve any obstruction of the right ventricular outflow tract, nor long term medical therapy or pacing because of cardiac arrhythmia.
  
  
  CONCLUSIONS
  Surgical repair of a double-chambered right ventricle yields excellent haemodynamic and functional results over the mid to long term.
authors.affiliation: Department of Congenital Heart Disease, Deutsches Herzzentrum Berlin, Berlin, Germany.
authors.forename: Ragiab
authors.initials: R
authors.lastname: Telagh
authors.forename: Vladimir
authors.initials: V
authors.lastname: Alexi-Meskishvili
authors.forename: Ronald
authors.initials: R
authors.lastname: Hetzer
authors.forename: Peter E
authors.initials: PE
authors.lastname: Lange
authors.forename: Felix
authors.initials: F
authors.lastname: Berger
authors.forename: Hashim
authors.initials: H
authors.lastname: Abdul-Khaliq
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D002648:Child; D002675:Child, Preschool; D015150:Echocardiography, Doppler; D017548:Echocardiography, Transesophageal; D005260:Female; D006330:Heart Defects, Congenital / Q000601:surgery*; D006352:Heart Ventricles / Q000601:surgery*; D006801:Humans; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D016896:Treatment Outcome