title: Double-chambered right ventricle in 73 patients: spectrum of the disease and surgical results of transatrial repair.
abstract: OBJECTIVE
  To review the spectrum of double-chambered right ventricle (DCRV) and the outcome of surgical repair in patients diagnosed between February 1988 and March 1999.
  
  
  METHODS
  The charts of patients with DCRV were studied.
  
  
  METHODS
  Tertiary care hospital.
  
  
  METHODS
  A total of 73 patients were identified. Sixty-nine underwent surgical repair, while four are awaiting surgery. The repair was through a transatrial approach in 61 patients, while in eight an additional ventriculotomy was performed.
  
  
  RESULTS
  An associated ventricular septal defect (VSD) was present in 56 of 73 patients (77%). These patients were significantly younger (P<0.05) than the 17 patients without a VSD. Among patients with a VSD, the 31 requiring patch closure were significantly younger than the 25 patients having direct closure. Five older patients among those with intact septum had impaired right ventricular (RV) function as well as higher intraventricular gradients. At surgery the intraventricular obstruction was relieved by myomectomy. There was no hospital or late mortality. Following surgery, at a mean follow-up of 13.6 months, no increase in the intraventricular gradient was detected by Doppler echocardiography.
  
  
  CONCLUSIONS
  The development of DCRV is associated with VSD in early life. The probability of the presence of a VSD decreases with age. The disease is progressive, resulting in increased intracavitary gradient within the RV and in RV impairment if it is not treated in a timely fashion. Transatrial repair is safe with excellent midterm results. In the presence of high gradients within the RV, a ventriculotomy may be necessary to obtain acceptable results.
authors.affiliation: King Faisal Specialist Hospital & Research Centre, Riyadh, Saudi Arabia. ogalal@hotmail.com
authors.forename: O
authors.initials: O
authors.lastname: Galal
authors.forename: Z
authors.initials: Z
authors.lastname: Al-Halees
authors.forename: L
authors.initials: L
authors.lastname: Solymar
authors.forename: L
authors.initials: L
authors.lastname: Hatle
authors.forename: A
authors.initials: A
authors.lastname: Mieles
authors.forename: A
authors.initials: A
authors.lastname: Darwish
authors.forename: M E
authors.initials: ME
authors.lastname: Fawzy
authors.forename: F
authors.initials: F
authors.lastname: Al Fadley
authors.forename: E
authors.initials: E
authors.lastname: de Vol
authors.forename: A A
authors.initials: AA
authors.lastname: Schmaltz
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D015150:Echocardiography, Doppler; D005260:Female; D005500:Follow-Up Studies; D006325:Heart Atria / Q000601:surgery; D006330:Heart Defects, Congenital / Q000175:diagnosis / Q000601:surgery*; D006345:Heart Septal Defects, Ventricular / Q000175:diagnosis / Q000601:surgery; D006352:Heart Ventricles / Q000002:abnormalities* / Q000601:surgery; D006439:Hemodynamics / Q000502:physiology; D006801:Humans; D007223:Infant; D008297:Male; D008875:Middle Aged; D011183:Postoperative Complications / Q000209:etiology*; D018497:Ventricular Dysfunction, Right / Q000175:diagnosis / Q000601:surgery