Abstract Hypoplastic left heart syndrome (HLHS) represents a severe spectrum of congenital cardiac malformations, which lead to systemic blood supply being dependent on patency of the arterial duct after birth. Antenatal diagnosis, advances in postnatal care and modifications of surgical palliation strategies have led to improved outcomes with the majority of patients born in the current era being expected to live into adulthood. This review discusses the surgical treatment strategies for HLHS and the wider implications these may have on the wellbeing of patients and families. The ‘Fontan' circulation constitutes a common endpoint for HLHS patients who often experience physiological circulatory compromise rather than pump failure of the univentricular heart. Heart transplantation has been performed in HLHS, but donor shortage in countries such as the United Kingdom make this a viable option only for a few patients. Protein losing enteropathy and plastic bronchitis may be signs of physiological failure in ‘Fontan' patients and require specialist input. Family-centred care should enable paediatricians to build relationship outside specialised cardiac centres and optimise healthcare experiences and outcomes through multidisciplinary care systems for these vulnerable patients.