Hypoplastic left heart syndrome is a severe form of congenital heart disease that results in single-ventricle physiology. Although surgical palliation in infants with this syndrome is widely agreed on in practice, variation occurs in both surgical and medical management. Perinatal factors that affect preoperative management include the subtype of the syndrome and the patient’s birth weight, gestational age, and genetic abnormalities. The general aspects of perioperative stabilization and management are monitoring, use of vasoactive infusions, ventilatory support, and nutrition. Management strategies for balancing single-ventricle physiology are aimed at addressing the 3 major causes of desaturation: diminished pulmonary blood flow, low mixed venous oxygen saturation, and pulmonary venous desaturation.

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