HLHS: first operative hurdle is the worst

Medium term outlook is good for babies surviving stage I reconstructive surgery for antenatally diagnosed hypoplastic left heart syndrome (HLHS). Reconstructive palliative surgery has greatly improved the outlook for this condition and makes antenatal diagnosis even more important to help parents make informed decisions based on up to date results.

Andrews et al report outcome data for antenatally diagnosed classic HLHS in the largest series so far—174 babies from Guy's Hospital, London, five from other units, (and nine postnatal diagnoses). Of these, 64 babies underwent stage I of a three stage surgical palliation, which ultimately directs systemic venous return straight to the pulmonary arteries and allows the right ventricle to act solely as the systemic pump.

Deaths associated with stage I were high: 30 within the first month (three during the operation, 18 within 24 hours, seven during the first week, and two at three weeks) and one at six weeks. Just over half the babies survived (52%, 33/64). All 29 babies who underwent stage II survived except two, and all 10 who underwent stage III survived. One child had a heart transplant, and 16 were awaiting stage III. Overall survival was 48% (31/64), or 49 % (27/55) for antenatal diagnoses. Three children had neurological problems at follow up.

Hopefully, future improvements in surgical technique and perioperative intensive care will continue to increase the chances of survival and reduce the risk of long term complications.