Impact of antenatal diagnosis of hypoplastic left heart syndrome on the clinical presentation and surgical outcomes: The Australian experience

Aim:   Antenatal diagnosis of severe congenital heart disease enables planning of perinatal care of affected infants. Congenital heart surgery is highly centralised in Australia, and surgery for hypoplastic left heart syndrome (HLHS) currently takes place at a single institution, in order to ensure case volume. The study aims to review the impact of antenatal diagnosis on the early clinical course of infants with HLHS in Australia.
Methods:   Retrospective review was performed on all neonates who were admitted for management of HLHS between 2001 and 2005 at the Paediatric Cardiac Surgical Unit, The Royal Children's Hospital, Melbourne, Australia.
Results:   Sixty neonates with HLHS were admitted, in whom an antenatal diagnosis was present in 46 (77%). Treatment was withdrawn in seven infants, of whom three had prenatal, and 4 had post-natal diagnoses. Antenatally diagnosed infants were commenced on prostaglandin earlier than post-natally diagnosed infants (age 1 h and 55 h respectively), and on paediatric intensive care unit admission had a higher pH (7.31 vs. 7.20), a lower lactate (3.0 vs. 6.7), a lower inspired oxygen fraction (0.21 vs. 0.96) and were less likely to be ventilated (10.8% vs. 92.9%). Infants with an antenatal diagnosis had lower peak creatinine (70 vs. 120) and alanine aminotransferase (29 vs. 242). The survival to intensive care discharge and stage 2 palliation was 74% and 68% respectively, and was not influenced by timing of diagnosis.
Conclusions:   Antenatal diagnosis of HLHS was strongly associated with a superior pre-operative clinical status, but did not influence early survival after surgical palliation.