Initial Management

1. Assess and maintain airway, breathing, and circulation (ABCs). Tracheal intubation and mechanical ventilation may be necessary, as might inotropic support. Investigate airway if foreign body aspiration is suspected.

2. Perform appropriate testing rapidly. Select studies that will give the most diagnostic information in the shortest amount of time. In some situations, an echocardiogram may be obtained more quickly than the hyperoxia test.

B. Prostaglandin Therapy. Cyanotic heart disease in a newborn is likely to be dependent on the ductus arteriosus for pulmonary or systemic blood flow. In a severely ill infant, prostaglandin E1 infusion (0.01-0.05 mcg/kg/min initially) should be started until diagnosis can be confirmed by echocardiogram. Lower infusion rates may avoid prostaglandin-induced apnea.

C. Treatment of Underlying Cause of Cyanosis. After diagnosis is determined, treat emergently as necessary or obtain specialist consultation.

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