Outcome of staged reconstructive surgery for hypoplastic left heart syndrome following antenatal diagnosis

R. Tulloh*, G. Sharland, E. Rosenthal

*Corresponding author for this work

Research output: Contribution to journalArticle (Academic Journal)peer-review

65 Citations (Scopus)


Background and Aims - 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.

Original languageEnglish
Pages (from-to)474-477
Number of pages4
JournalArchives of Disease in Childhood
Issue number6
Publication statusPublished - 17 Dec 2001


  • Antenatal diagnosis
  • Hypoplastic left heart syndrome
  • Norwood procedure


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