title: The influence of different strategies on clinical outcome in patients undergoing total cavopulmonary connection.
abstract: BACKGROUND
  We report on results of a prospective clinical trial designed to demonstrate the influence of various strategies in "Total Cavopulmonary Connection" (TCPC) for palliative therapy of patients with "single ventricle" physiology.
  
  
  METHODS
  From 1989 to 1997, a total of 47 patients (mean age 4.8 +/- 3.6 years) underwent definitive TCPC at our unit. 31 patients (66%) underwent one-stage TCPC, in 16 patients (34%) we performed a two-stage modified Fontan operation; 21 patients had central fenestration (4 mm). Inhalative NO therapy in the immediate postoperative period was adopted in 1993.
  
  
  RESULTS
  Overall 5-year survival was 76.4%, after two-stage TCPC 87.5%, and 81.3% in patients undergoing fenestrated procedures. Two of three patients survived perioperative Fontan take-down. We lost 11 patients (nine early and two late deaths): three patients died primarily because of neurologic dysfunction and eight patients because of cardiac failures. Under perioperative NO therapy there was no early death. After a mean follow-up of 35.9 +/- 23.3 months, 76% of all patients were in NYHA I and 21 % in NYHA I-II. 89.7% had sinus rhythm. 42% of our patients suffered from temporary pleuropericardial effusions.
  
  
  CONCLUSIONS
  Definitive palliation with TCPC achieves acceptable clinical results. Two-stage repair, fenestration, and postoperative inhalative NO therapy - each have a positive influence on early and long-term survival.
authors.affiliation: Clinical Department of Cardiac Surgery, Karl Franzens University & Medical School of Graz, Austria. igor.knez@kfunigraz.ac.at
authors.forename: I
authors.initials: I
authors.lastname: Knez
authors.forename: D
authors.initials: D
authors.lastname: Dacar
authors.forename: H
authors.initials: H
authors.lastname: Mächler
authors.forename: A
authors.initials: A
authors.lastname: Gamillscheg
authors.forename: A
authors.initials: A
authors.lastname: Beitzke
authors.forename: G
authors.initials: G
authors.lastname: Zobel
authors.forename: S
authors.initials: S
authors.lastname: Rödl
authors.forename: A
authors.initials: A
authors.lastname: Poier-Knez
authors.forename: H
authors.initials: H
authors.lastname: Gombotz
authors.forename: H
authors.initials: H
authors.lastname: Metzler
authors.forename: P
authors.initials: P
authors.lastname: Rehak
authors.forename: B
authors.initials: B
authors.lastname: Rigler
mesh_terms: D000280:Administration, Inhalation; D000293:Adolescent; D000328:Adult; D002315:Cardiopulmonary Bypass; D002648:Child; D002675:Child, Preschool; D015716:Electrocardiography, Ambulatory; D005260:Female; D005500:Follow-Up Studies; D018610:Heart Bypass, Right / Q000379:methods; D006330:Heart Defects, Congenital / Q000401:mortality / Q000503:physiopathology / Q000601:surgery*; D006352:Heart Ventricles / Q000002:abnormalities* / Q000601:surgery; D017052:Hospital Mortality; D006801:Humans; D007223:Infant; D008297:Male; D009569:Nitric Oxide / Q000008:administration & dosage / Q000627:therapeutic use; D011183:Postoperative Complications / Q000401:mortality / Q000517:prevention & control; D011446:Prospective Studies; D015996:Survival Rate; D016896:Treatment Outcome; D014665:Vasodilator Agents / Q000008:administration & dosage / Q000627:therapeutic use
chemical_list: D014665:Vasodilator Agents; D009569:Nitric Oxide