Patients with hypoplastic left heart syndrome undergo a series of operations to separate the pulmonary and systemic circulations. The first of at least 3 operations occurs in the newborn period, with a stage I palliation. The goal of stage I palliation is to provide pulmonary blood flow and create an unobstructed systemic outflow tract. Advances in surgical techniques and intraoperative and postoperative care have helped decrease morbidity and mortality for patients with hypoplastic left heart syndrome who have the stage I Norwood operation, but the patients continue to be at increased risk for hemodynamic collapse and adverse outcomes. This article discusses risk factors, surgical approach, postoperative nursing and medical management strategies, differences between and outcomes for the Norwood operation with the right ventricle to pulmonary artery conduit and the Norwood operation with a modified Blalock-Taussig shunt.

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