Objective To assess the rate of antenatal detection of hypoplastic left heart syndrome (HLHS) and its outcome antenatally and postnatally. This review was undertaken to improve counselling and provide better surgical survival information for expecting parents in the future.

Methods All cases of HLHS detected antenatally between January 2002 and December 2008 were included in the study. Fetal medicine and fetal cardiac databases at a tertiary fetal cardiology centre as well as CARIS central database in Wales were utilised to carry out the review. The notes were carefully scrutinised to rule out any confounding variables.

Results There were 55 cases of HLHS in South Wales over this period, 50 of which were detected antenatally. 24 of these were terminated following counselling and two ended up in still birth. Hence, there were 24 live births with HLHS. Of these, one died without undergoing any surgery. The remaining 23 cases underwent surgery but five children died. Eighteen of them are alive and are under follow-up.

HLHS is an isolated defect in majority of cases (38 out of 50). The rate of chromosomal abnormality is 10% (5 out of 50).

Conclusion HLHS is one of the most high-risk lesions in children with congenital heart disease. Antenatal detection rate for HLHS has been satisfactory at 90% in Wales. Termination rate remains high despite substantial improvement in survival after surgery. Post surgical survival stands at 78% in our series with a maximum follow-up of 8 years. Counselling is essential for parents to make an informed decision.