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

Background and Aims-Staged reconstructive surgery has radically altered the prognosis of hypoplastic left heart syndrome (HLHS). Antenatal diagnosis a llows 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 1 995 and 2000, including tin-ling of diagnosis, outcome of pregnancy, and ag e and outcome at each stage of surgery. At Guy's Hospital, 174 cases were d iagnosed antenatally, of whom 50 underwent surgery. Fourteen others (five d iagnosed antenatally at other centres, and nine diagnosed postnatally) also underwent surgery. Results-Survival after stage I (the Norwood operation) was 52% (33/64). Pos toperative 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 surviva l of 48% (31/64). At follow up, three children have neurological impairment , and one had poor right ventricular function necessitating cardiac transpl antation. Conclusions-Antenatal diagnosis allows informed decisions about treatment o ptions, and facilitates preoperative care. Mortality following stage I is h igh, irrespective of timing of diagnosis, but medium term outcome for survi vors is good.