title: [Hypoplastic left heart syndrome: 10 year experience with staged surgical management].
abstract: Hypoplastic left heart syndrome (HLHS) is a lethal congenital heart disease in 95% of non-treated patients. Surgical staging is the main form of treatment, consisting of a 3-stage approach, beginning with the Norwood operation. Long term survival of treated patients is unknown in our country.
  
  
  OBJECTIVE
  1) To review our experience in the management of all patients seen with HLHS between January 2000 and June 2012. 2) Identify risk factors for mortality.
  
  
  METHODS
  Retrospective analysis of a single institution experience with a cohort of patients with HLHS. Clinical, surgical, and follow-up records were reviewed.
  
  
  RESULTS
  Of the 76 patients with HLHS, 9 had a restrictive atrial septal defect (ASD), and 8 had an ascending aorta ≤2mm. Of the 65 out of 76 patients that were treated, 77% had a Norwood operation with pulmonary blood flow supplied by a right ventricle to pulmonary artery conduit, 17% had a Norwood with a Blalock-Taussig shunt, and 6% other surgical procedure. Surgical mortality at the first stage was 23%, and for Norwood operation 21.3%. For the period between 2000-2005, surgical mortality at the first stage was 36%, and between 2005-2010, 15% (P=.05). Actuarial survival was 64% at one year, and 57% at 5years. Using a multivariate analysis, a restrictive ASD and a diminutive aorta were high risk factors for mortality.
  
  
  CONCLUSIONS
  Our immediate and long term outcome for staged surgical management of HLHS is similar to that reported by large centres. There is an improvement in surgical mortality in the second half of our experience. Risk factors for mortality are also identified.
authors.affiliation: Departamento de Cardiología y Enfermedades Respiratorias, División de Pediatría, Facultad de Medicina, Pontificia Universidad Católica de Chile, Santiago, Chile. Electronic address: urcelay@med.puc.cl.
authors.forename: Gonzalo
authors.initials: G
authors.lastname: Urcelay
authors.affiliation: Departamento de Cardiología y Enfermedades Respiratorias, División de Pediatría, Facultad de Medicina, Pontificia Universidad Católica de Chile, Santiago, Chile.
authors.forename: Francisca
authors.initials: F
authors.lastname: Arancibia
authors.affiliation: Departamento de Cardiología y Enfermedades Respiratorias, División de Pediatría, Facultad de Medicina, Pontificia Universidad Católica de Chile, Santiago, Chile.
authors.forename: Javiera
authors.initials: J
authors.lastname: Retamal
authors.affiliation: Departamento de Cardiología y Enfermedades Respiratorias, División de Pediatría, Facultad de Medicina, Pontificia Universidad Católica de Chile, Santiago, Chile.
authors.forename: Daniel
authors.initials: D
authors.lastname: Springmuller
authors.affiliation: Departamento de Cardiología y Enfermedades Respiratorias, División de Pediatría, Facultad de Medicina, Pontificia Universidad Católica de Chile, Santiago, Chile.
authors.forename: Cristián
authors.initials: C
authors.lastname: Clavería
authors.affiliation: Departamento de Cardiología y Enfermedades Respiratorias, División de Pediatría, Facultad de Medicina, Pontificia Universidad Católica de Chile, Santiago, Chile.
authors.forename: Francisco
authors.initials: F
authors.lastname: Garay
authors.affiliation: Departamento de Cardiología y Enfermedades Respiratorias, División de Pediatría, Facultad de Medicina, Pontificia Universidad Católica de Chile, Santiago, Chile.
authors.forename: Patricia
authors.initials: P
authors.lastname: Frangini
authors.affiliation: División de Enfermedades Cardiovasculares, Facultad de Medicina, Pontificia Universidad Católica de Chile, Santiago, Chile.
authors.forename: Rodrigo
authors.initials: R
authors.lastname: González
authors.affiliation: Departamento de Cardiología y Enfermedades Respiratorias, División de Pediatría, Facultad de Medicina, Pontificia Universidad Católica de Chile, Santiago, Chile.
authors.forename: Felipe
authors.initials: F
authors.lastname: Heusser
authors.affiliation: Hospital Roberto del Rio, Santiago, Chile.
authors.forename: Claudio
authors.initials: C
authors.lastname: Arretz
authors.affiliation: Departamento de Cardiología y Enfermedades Respiratorias, División de Pediatría, Facultad de Medicina, Pontificia Universidad Católica de Chile, Santiago, Chile.
authors.forename: Pamela
authors.initials: P
authors.lastname: Zelada
authors.affiliation: División de Enfermedades Cardiovasculares, Facultad de Medicina, Pontificia Universidad Católica de Chile, Santiago, Chile.
authors.forename: Pedro
authors.initials: P
authors.lastname: Becker
mesh_terms: D001011:Aorta / Q000002:abnormalities; D006348:Cardiac Surgical Procedures / Q000379:methods*; D005260:Female; D005500:Follow-Up Studies; D006344:Heart Septal Defects, Atrial / Q000401:mortality / Q000503:physiopathology / Q000601:surgery*; D006801:Humans; D018636:Hypoplastic Left Heart Syndrome / Q000401:mortality / Q000503:physiopathology / Q000601:surgery*; D007231:Infant, Newborn; D008297:Male; D015999:Multivariate Analysis; D012189:Retrospective Studies; D012307:Risk Factors; D013997:Time Factors
keywords: Cardiopatías congénitas; Congenital heart diseases; Hypoplastic left heart syndrome; Mortalidad quirúrgica; Norwood procedure; Operación de Norwood; Surgical mortality; Síndrome de hipoplasia de corazón izquierdo