One of the Last True Surgical Emergencies in Congenital Heart Disease

A female infant born at 38 weeks via uncomplicated spontaneous vaginal delivery presents hours after birth with cyanosis and respiratory distress.

Prenatal history was significant only for intrauterine growth restriction. The infant was intubated and brought to the NICU for stabilization. Physical exam revealed a cyanotic infant with oxygen saturation ranging from 76-83% on 40% FiO2 and PaO2 in mid 30s. Auscultation of the lungs was clear throughout. Cardiac examination revealed a regular rate and rhythm with a non-specific short Gr I/VI systolic murmur at the LUSB. The remainder of the physical exam was normal.

CXR showed a normal sized heart with interstitial edema in both lung fields.

EKG revealed a normal sinus rhythm with left axis deviation and right ventricular hypertrophy.

Select echocardiogram clips are shown below.

Video 1

Video 2

Figure 1

Figure 1

On the first day of life she underwent emergent surgical repair of her congenital cardiac lesion. After weaning from cardiopulmonary bypass, systemic blood pressure is 53/31 mmHg, PA pressure 57/29 mm Hg and left atrial pressure is 13 mm Hg. An arterial blood gas showed a pH = 7.47, pCO2 33 mm Hg, paO2 72 mm Hg.

Which of the following is the most appropriate next step in management?

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