title: Effect of a selection and postoperative care protocol on survival of infants with hypoplastic left heart syndrome.
abstract: BACKGROUND
  We report the development and implementation of a program designed to assign patients preoperatively to either transplant or Norwood procedure based on a score derived from known risk factors and to enhance postoperative care of infants undergoing the Norwood procedure.
  
  
  METHODS
  A weighted score for each of six variables comprised the scoring system: ventricular function, tricuspid regurgitation, ascending aortic diameter, atrial septal defect blood flow characteristics, blood type, and age. The scoring system was used to prospectively assign mortality risk and lead to recommendation of either Norwood procedure or transplantation.
  
  
  RESULTS
  Survival following the Norwood procedure significantly improved after the management program was implemented (88% versus 40% at 48 hours, 57% versus 10% at 30 days, and 50% versus 10% at 1 year, p < 0.0001 at each time point). The survival of the group that received a score of 7 or less (high risk) who underwent the Norwood procedure was 78% at 48 hours, 44% at 30 days, and 33% at 1 year; survival rates among patients considered lower risk (greater than 7) were 100% at 48 hours and 80% at 30 days and 1 year. Transplant outcomes remained unchanged.
  
  
  CONCLUSIONS
  We report improved survival following the Norwood procedure after the implementation of an institutional management approach aimed at improving the outcome of infants with hypoplastic left heart syndrome and may help neutralize historical biases toward Norwood procedure or transplantation.
authors.affiliation: Department of Critical Care Medicine, Loma Linda University Children's Hospital, Loma Linda, California, USA. checchia_p@kids.wustl.edu
authors.forename: Paul A
authors.initials: PA
authors.lastname: Checchia
authors.forename: Ranae
authors.initials: R
authors.lastname: Larsen
authors.forename: Ruchir
authors.initials: R
authors.lastname: Sehra
authors.forename: Noha
authors.initials: N
authors.lastname: Daher
authors.forename: Steven R
authors.initials: SR
authors.lastname: Gundry
authors.forename: Anees J
authors.initials: AJ
authors.lastname: Razzouk
authors.forename: Leonard L
authors.initials: LL
authors.lastname: Bailey
mesh_terms: D002140:California; D003422:Critical Care / Q000379:methods; D005260:Female; D005500:Follow-Up Studies; D016027:Heart Transplantation; D006439:Hemodynamics / Q000502:physiology; D006801:Humans; D018636:Hypoplastic Left Heart Syndrome / Q000401:mortality / Q000503:physiopathology / Q000601:surgery*; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D010043:Outcome and Process Assessment, Health Care; D010166:Palliative Care; D018579:Patient Selection; D011182:Postoperative Care; D011183:Postoperative Complications / Q000401:mortality*; D012307:Risk Factors; D015996:Survival Rate