title: [Functional univentricular heart: immediate and long term results, in the different stages of sequential correction].
abstract: BACKGROUND
  The functionally univentricular heart represents an heterogeneous group of anomalies that have in common the feature that only one of the chambers within the ventricular mass is capable of supporting independently either the pulmonary or systemic circulation.
  
  
  OBJECTIVE
  Evaluation from the neonatal period of the immediate and long term surgical results of the sequential total cavo-pulmonary connection in the various anatomical forms of the functional univentricular hearts.
  
  
  METHODS
  From May 1998 to May 2009, 84 patients have been followed since the neonatal period and in a prospective retro-prospective way (bidirectional), in which 181 sequential surgical procedures were performed in three stages: Neonatal, Bidirectional Glenn and Total cava pulmonary connection. We analyzed the following variables: age, performed surgical procedures, risk stratification (RACHS), surgical and inter stage mortality and complications in the follow up. The categorical variables are reported as percentages with their confident interval (CI) 95%, the quantitative variables as median ratio. A Kaplan Meyer survival curve was performed.
  
  
  RESULTS
  A total of 181 procedures were screened. Stage 1: 68 patients, median age 5.5 days (r = 2-60) mortality 14.7% (CI95: 6.3%-23%). Stage 2: 70 patients, median age 4 month (r= 3-12), mortality 1.4% (CI95 0%-4.1%) Stage 3: 43 patients, median age 3 years (r = 2-6), mortality 9,3% (CI95%: 0.7%-18%). The interstage mortality was 11.9% (CI95: 5.1%- 8.8%). The total mortality was 29.7% (CI95 19.3% -38.7%). The mortality by RACHS: RACHS 2:1.6% (CI95: 0%-4.7%); RACHS 3: 5,5% (CI95: 0.3%-10.7%); RACHS 4: 0% and RACHS 6: 23.8% (CI95: 10.9%- 35.1%).Survival index at 10 years 69%
  
  
  CONCLUSIONS
  The sequential surgical approach from the neonatal period has changed the natural history of this entity with reasonable survival rate and mortality according to RACHS. The medium term follow up is encouraging and may improve in the future.
authors.affiliation: Departamento de Cardiología Infantil.
authors.forename: Francisco
authors.initials: F
authors.lastname: Comas
authors.forename: Gustavo
authors.initials: G
authors.lastname: Sivori
authors.forename: Alejandro
authors.initials: A
authors.lastname: Ithuralde
authors.forename: María Alicia
authors.initials: MA
authors.lastname: García-Nani
authors.forename: María
authors.initials: M
authors.lastname: Balestrini
authors.forename: Carlos
authors.initials: C
authors.lastname: Seara
authors.forename: Pablo
authors.initials: P
authors.lastname: García-Delucis
authors.forename: Carlos
authors.initials: C
authors.lastname: Nojek
authors.forename: Mariano
authors.initials: M
authors.lastname: Ithuralde
mesh_terms: D006348:Cardiac Surgical Procedures / Q000379:methods; D006352:Heart Ventricles / Q000002:abnormalities* / Q000601:surgery*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D011446:Prospective Studies; D012189:Retrospective Studies; D013997:Time Factors; D016896:Treatment Outcome