Hypoplastic left heart syndrome (HLHS) is the only congenital heart lesion that requires the talents of the neonatologist, pediatric cardiologist, and cardiovascular surgeon operating and communicating as a team to effect the desired outcome of survival with normal neurodevelopment. Prenatal diagnosis, initial resuscitation, and preoperative management are key elements that allow the best opportunity for low surgical morbidity and mortality in the affected infant. Physicians and nurses caring for such infants must understand the physiology of oxygen delivery and the response of the neonatal pulmonary and systemic vascular bed to interventions that affect the balance between systemic and pulmonary blood flow. Outcomes with current surgical management, including the Norwood procedure and the Sano modification, are equivalent to those associated with the arterial switch procedure and repair of neonatal tetralogy of Fallot. Families of infants born with HLHS should be encouraged by the current results of palliation and long-term outcome.

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