Initial management of a newborn infant with hypoplastic left heart syndrome should include:

A. Intravenous administration of PGE 1.

DISCUSSION: Postnatal stabilization of infants with hypoplastic left heart syndrome requires patency of the ductus arteriosus and balance of the systemic and the pulmonary circulation. Because the ductus is the only pathway from the right ventricle to the systemic circulation, duct patency must be maintained with intravenous PGE 1. To minimize the workload on the single ventricle and ensure adequate delivery of oxygen to the tissues, an equal delivery of blood to both the lungs and the body is sought. The normal postnatal decrease in pulmonary vascular resistance often results in overperfusion of the pulmonary circulation and underperfusion of the systemic circulation. Maneuvers that further decrease pulmonary vascular resistance, such as the addition of supplemental oxygen, lowering the PCO 2 to less than 35 mm. Hg, or eliminating any resistance at the atrial septum by balloon septostomy only worsens the imbalance.