Prenatal diagnosis of hypoplastic left heart syndrome is strongly encouraged through careful, routine level II ultrasound screening, especially in high-risk families. A decision for palliative care (leading to death) should be discouraged if cardiac anatomy or physiology is favorable, centers of expertise are available, sufficient resources exist, and there are no associated coexisting anomalies or genetic defects that permit little chance of meaningful survival. Staged surgical palliation is preferred over cardiac transplantation as the initial therapeutic approach.