title: Birth weight and complexity are significant factors for the management of hypoplastic left heart syndrome.
abstract: BACKGROUND
  Classic options for treatment of hypoplastic left heart syndrome include the Norwood procedure (NW) and heart transplantation (HT). Recently off-pump palliative procedures were introduced in the management of these patients. Risk factors influencing the decision between the NW with staged reconstruction or off-pump palliation and HT were assessed.
  
  
  METHODS
  Between January 2002 and January 2006, 69 patients with hypoplastic left heart syndrome were referred for either a NW (n = 33) or HT (n = 36). Patients referred for HT underwent off-pump palliation (catheter-based, n = 20, or surgical hybrid procedures, n = 7) until a donor organ became available: including patent ductus arteriosus stenting (80.6%), atrial septostomy (41.7%), and branch pulmonary artery banding (55.6%).
  
  
  RESULTS
  Heart transplantation patients were more complex, based on a higher Aristotle Comprehensive Complexity score calculated at birth (HT 18.8 +/- 2.4 versus NW 17.7 +/- 1.7; p < 0.05). Presurgical death was significantly greater for HT referrals (HT 27.8% versus NW 3.0%; p < 0.01); however, there was no difference between the two groups in operative (HT 11.5% versus NW 21.9%; not significant) or overall mortality (HT 36.1% versus NW 24.2%; not significant). No independent risk factors for death were identified with HT; however, a lower birth weight (<2.5 kg) and a higher Aristotle score (>20) correlated with surgical death with NW (p < 0.01). Noticeably, surgical survival was 85.7% for infants with birth weight of 2.5 kg or greater undergoing NW.
  
  
  CONCLUSIONS
  Overall survival is similar for hypoplastic left heart syndrome patients referred for the NW or HT. Lower birth weight and higher complexity are risk factors for patients undergoing NW. Off-pump palliation followed by HT or further staged palliation should be considered for these high-risk hypoplastic left heart syndrome patients.
authors.affiliation: The Children's Hospital Heart Institute, The Children's Hospital, Denver, Colorado 80218-1088, USA.
authors.forename: John H
authors.initials: JH
authors.lastname: Artrip
authors.forename: David N
authors.initials: DN
authors.lastname: Campbell
authors.forename: D Dunbar
authors.initials: DD
authors.lastname: Ivy
authors.forename: Melvin C
authors.initials: MC
authors.lastname: Almodovar
authors.forename: Kak-Chen
authors.initials: KC
authors.lastname: Chan
authors.forename: Max B
authors.initials: MB
authors.lastname: Mitchell
authors.forename: David R
authors.initials: DR
authors.lastname: Clarke
authors.forename: François
authors.initials: F
authors.lastname: Lacour-Gayet
mesh_terms: D051017:Apoptosis Regulatory Proteins; D001724:Birth Weight; D006348:Cardiac Surgical Procedures / Q000379:methods*; D005260:Female; D016027:Heart Transplantation; D006801:Humans; D018636:Hypoplastic Left Heart Syndrome / Q000503:physiopathology / Q000601:surgery*; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D010166:Palliative Care; D012307:Risk Factors
chemical_list: D051017:Apoptosis Regulatory Proteins