title: Improving outcomes in functional single ventricle and total anomalous pulmonary venous connection.
abstract: BACKGROUND
  We have previously reported that the outcome of infants with functional single ventricle and total anomalous pulmonary venous connection is poor relative to that of other single ventricle patients. Younger age at initial operation and obstructed total anomalous pulmonary venous connection were found to be risk factors for mortality. A review of our recent experience was undertaken to determine whether results in these patients are improving.
  
  
  METHODS
  Medical records of 18 patients admitted after 1997 were reviewed (group B) and compared with the previous group of 73 patients admitted between 1984 and 1997 (group A). Data were analyzed using a Cox proportional hazards model.
  
  
  RESULTS
  Median age at first operation was the same for both groups. The incidence of obstructed total anomalous pulmonary venous connection was not significantly different between groups (29% versus 33%, p = 0.70). Early survival is significantly improved for group B compared with group A (p = 0.015). Only group and younger age at initial operation were found to be risk factors for mortality. In the current group, 5 patients have undergone superior cavopulmonary connection with one death, 3 have undergone Fontan completion with no deaths, and heart or heart-lung transplantation was performed in 5 patients with two deaths.
  
  
  CONCLUSIONS
  Early survival in patients with single ventricle and total anomalous pulmonary venous connection has improved significantly in recent years, but intermediate survival is still approximately 50%. Selective management using staged reconstructive surgery and transplantation may result in improved survival. Further study may identify risk factors for mortality and improve selection of appropriate patients for each therapy.
authors.affiliation: Division of Cardiothoracic Surgery, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA. andrew.lodge@duke.edu
authors.forename: Andrew J
authors.initials: AJ
authors.lastname: Lodge
authors.forename: Jack
authors.initials: J
authors.lastname: Rychik
authors.forename: Susan C
authors.initials: SC
authors.lastname: Nicolson
authors.forename: Richard F
authors.initials: RF
authors.lastname: Ittenbach
authors.forename: Thomas L
authors.initials: TL
authors.lastname: Spray
authors.forename: J William
authors.initials: JW
authors.lastname: Gaynor
mesh_terms: D000015:Abnormalities, Multiple / Q000601:surgery; D002675:Child, Preschool; D005260:Female; D018729:Fontan Procedure; D016027:Heart Transplantation; D006352:Heart Ventricles / Q000002:abnormalities* / Q000601:surgery; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D015993:Life Tables; D008297:Male; D010166:Palliative Care; D011183:Postoperative Complications / Q000453:epidemiology; D011379:Prognosis; D016016:Proportional Hazards Models; D011667:Pulmonary Veins / Q000002:abnormalities* / Q000601:surgery; D012189:Retrospective Studies; D012307:Risk Factors; D016019:Survival Analysis; D017741:Survivors; D016896:Treatment Outcome