Case 3--Pulmonary Hypertension in a Newborn

Clinical summary: A term male infant (2523 g) was transferred to UCSF on the third day of life for treatment of pulmonary hypertension. Apgar scores at birth were 6 at 1 minute and 9 at 4 minutes. Immediate respiratory distress was attributed to a left pneumothorax, which was treated. An imperforate anus was treated with a colostomy on day 2. Post-operatively, the infant could not be weaned from the ventilator. A chest film showed bilateral opacities. He was treated with 100% oxygen and Exosurf, and the PaO2 rose transiently to 200 mm Hg. An echocardiogram showed R-L atrial shunting, functional closure of the ductus, increased PA pressures, pulmonic insufficiency, and tricuspid regurgitation. After transfer he was given 100% oxygen. Arterial blood gases were pH 7.67, pCO2 21 mm Hg, and pO2 63 mm Hg. Pulse was 210/min and O2 saturation, 99%. There were no chest retractions. On day 5, the PaO2 fell to <30 mm Hg despite maximal ventilatory support. ECMO was begun, but it could not be discontinued. On day 16, a trial of nitric oxide also failed to produce sustained oxygenation. Angiography showed a "pruned tree" pattern (L>R). ECMO was discontinued on day 23 (after 17 da), and he died.

Radiology | Image 1 | Image 2 | Image 3 | Image 4 | Discussion

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