In utero diagnosis of complex progressive cardiac disease such as hypoplastic left heart syndrome presents a novel opportunity for antepartum, intrapartum, and neonatal management. The clinical possibilities and potential for differing outcomes challenge the mother–foetus dyad with regard to informed consent. Previous studies reveal that rates of termination of pregnancy for foetuses with hypoplastic left heart syndrome vary widely in the United States and Europe, leading us to surmise that informed consent may be practised differently. The purpose of this paper is to review the ethical considerations and physician responsibilities of informed consent as they relate to prenatal and postnatal patients with hypoplastic left heart syndrome. Special consideration is paid to the informed consent process as practised by the obstetrician, perinatologist, paediatric cardiologist, and paediatric cardiac surgeon as it relates to termination of pregnancy, comfort care, and surgical palliation. We will argue that informed consent as it relates to hypoplastic left heart syndrome is far from standardised and that there exists a state of bioethical equipoise concerning the extent and limits of its application in the current clinical setting.
