title: Stage I palliation for hypoplastic left heart syndrome in low birth weight neonates: can we justify it?
abstract: OBJECTIVE
  Although the outcome of cardiac surgery in neonates with low birth weight (LBW) has improved, LBW remains a risk factor for surgical palliation. Few surgical series of LBW patients include those with hypoplastic left heart syndrome (HLHS). To identify variables associated with poor outcome in this group, we reviewed our experience with patients with HLHS and LBW who underwent Stage I Norwood palliation.
  
  
  METHODS
  Between January 1998 and December 2000, 20 consecutive LBW (<2500 g) neonates with HLHS (n=13) or HLHS variant (n=7) underwent surgical palliation. Retrospective review of all patient data and analysis to identify risk factors was performed.
  
  
  RESULTS
  Mean age at surgery was 5.1+/-4.6 days (range 1-17), mean weight was 1.98+/-0.44 kg (range 1.1-2.5), including nine patients under 2 kg. Ten patients were born at <35 weeks gestation. Anatomic diagnosis included HLHS in 13 patients (10 with aortic atresia), unbalanced atrioventricular canal defect in two, double outlet right ventricle in two and other variants in three. Mean ascending aortic size was 4.0+/-1.8 mm (range 1.5-8). Associated cardiac defects were present in three patients, and a genetic syndrome and/or congenital anomaly was present in four of them. Mean circulatory arrest time was 60+/-10 min. Extracorporeal support was used perioperatively in 10 patients. Early mortality was 9/20 (45%). At a mean follow up at 22+/-10 months (range 8-38), six patients underwent stage II, and are awaiting stage III; four patients have completed their Fontan. Anatomic variant, ascending aortic size, prematurity, age at surgery, weight, duration of circulatory arrest, cardiopulmonary bypass time and associated non-cardiac anomalies were not risk factors for poor outcome whereas restrictive pulmonary venous drainage and coronary artery anomalies were associated with decreased survival.
  
  
  CONCLUSIONS
  LBW newborns with HLHS and physiologic variants have an increased early surgical risk but have acceptable intermediate survival rates for subsequent palliation including Fontan. LBW and prematurity should not be contraindications to early surgical palliation.
authors.affiliation: Nemours Cardiac Center-Orlando, Arnold Palmer Hospital for Children and Women, 82 West Miller St, Orlando, FL 32806, USA. cpizarro@nemours.org
authors.forename: Christian
authors.initials: C
authors.lastname: Pizarro
authors.forename: Deborah A
authors.initials: DA
authors.lastname: Davis
authors.forename: Mark E
authors.initials: ME
authors.lastname: Galantowicz
authors.forename: Hamish
authors.initials: H
authors.lastname: Munro
authors.forename: Samuel S
authors.initials: SS
authors.lastname: Gidding
authors.forename: William I
authors.initials: WI
authors.lastname: Norwood
mesh_terms: D001011:Aorta / Q000002:abnormalities / Q000601:surgery; D002303:Cardiac Output, Low / Q000150:complications / Q000401:mortality / Q000601:surgery; D006348:Cardiac Surgical Procedures; D003330:Coronary Vessel Anomalies / Q000150:complications / Q000401:mortality / Q000601:surgery; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D018636:Hypoplastic Left Heart Syndrome / Q000150:complications / Q000401:mortality / Q000601:surgery*; D007229:Infant Welfare; D007230:Infant, Low Birth Weight; D007231:Infant, Newborn; D007902:Length of Stay; D008297:Male; D009017:Morbidity; D016019:Survival Analysis; D016896:Treatment Outcome; D014481:United States / Q000453:epidemiology; D018487:Ventricular Dysfunction, Left / Q000150:complications / Q000401:mortality / Q000601:surgery