Arch Dis Child 2001;85:474-477 ( December )

Article

Outcome of staged reconstructive surgery for hypoplastic left heart syndrome following antenatal diagnosis R Andrews, R Tulloh, G Sharland, J Simpson, S Rollings, E Baker, S Qureshi, E Rosenthal, C Austin, D Anderson

Department of Congenital Heart Disease, Guy's Hospital, Guy's and St Thomas' NHS Trust, London, UK

Correspondence to: Dr Tulloh Robert.Tulloh{at}gstt.sthames.nhs.uk

Accepted 21 February 2001

BACKGROUND AND AIMSStaged 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.
METHODSInformation 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.
RESULTSSurvival 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.
CONCLUSIONSAntenatal 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.


Keywords: hypoplastic left heart syndrome; Norwood procedure; antenatal diagnosis

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© 2001 by Archives of Disease in Childhood
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