title: Outcome of staged reconstructive surgery for hypoplastic left heart syndrome following antenatal diagnosis.
abstract: OBJECTIVE
  Staged reconstructive surgery has radically altered the prognosis of hypoplastic left heart syndrome (HLHS). Antenatal diagnosis allows for appropriate counselling, and time to consider treatment options. We report outcome from a centre where most cases are antenatally diagnosed and delivered on site.
  
  
  METHODS
  Information was collated on 188 consecutive cases of HLHS between 1995 and 2000, including timing of diagnosis, outcome of pregnancy, and age and outcome at each stage of surgery. At Guy's Hospital, 174 cases were diagnosed antenatally, of whom 50 underwent surgery. Fourteen others (five diagnosed antenatally at other centres, and nine diagnosed postnatally) also underwent surgery.
  
  
  RESULTS
  Survival after stage I (the Norwood operation) was 52% (33/64). Postoperative survival after stage II (the hemi-Fontan operation, performed in 29), and stage III (the Fontan operation, performed in 10), was 100%. Two late deaths occurred 3 and 10 months after stage II, giving overall survival of 48% (31/64). At follow up, three children have neurological impairment, and one had poor right ventricular function necessitating cardiac transplantation.
  
  
  CONCLUSIONS
  Antenatal diagnosis allows informed decisions about treatment options, and facilitates preoperative care. Mortality following stage I is high, irrespective of timing of diagnosis, but medium term outcome for survivors is good.
authors.affiliation: Department of Congenital Heart Disease, Guy's Hospital, Guy's and St Thomas' NHS Trust, London, UK.
authors.forename: R
authors.initials: R
authors.lastname: Andrews
authors.forename: R
authors.initials: R
authors.lastname: Tulloh
authors.forename: G
authors.initials: G
authors.lastname: Sharland
authors.forename: J
authors.initials: J
authors.lastname: Simpson
authors.forename: S
authors.initials: S
authors.lastname: Rollings
authors.forename: E
authors.initials: E
authors.lastname: Baker
authors.forename: S
authors.initials: S
authors.lastname: Qureshi
authors.forename: E
authors.initials: E
authors.lastname: Rosenthal
authors.forename: C
authors.initials: C
authors.lastname: Austin
authors.forename: D
authors.initials: D
authors.lastname: Anderson
mesh_terms: D006348:Cardiac Surgical Procedures / Q000379:methods / Q000401:mortality; D002423:Cause of Death; D002675:Child, Preschool; D005260:Female; D006801:Humans; D018636:Hypoplastic Left Heart Syndrome / Q000000981:diagnostic imaging / Q000401:mortality / Q000601:surgery*; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D011247:Pregnancy; D012189:Retrospective Studies; D016019:Survival Analysis; D016896:Treatment Outcome; D016216:Ultrasonography, Prenatal / Q000379:methods / Q000401:mortality