Pankaj Garg.
Oral Sildenafil for PPHN in neonates: selection of patients remains a dilemma?.
J Coll Physicians Surg Pak Jan ;18(2):132-3.

A 1450 grams near term small for gestational age, male baby was delivered at a small peripheral health facility by emergency cesarean section, for severe oligohydramnios and prolonged rupture of membranes, was brought in respiratory distress having a positive sepsis screen at admission. Chest X-ray revealed low volume lungs with generalized opacity suggesting pulmonary hypoplasia and a small right pneumothorax. Baby was given surfactant at eight hours of age and ventilated on Synchronized Intermittent Mechanical Ventilation (SIMV) mode with pressure support (Maquet, Servo I, Sweden). He required very high pressures PIP 28/5 rates 60 and 100 FiO2. Sequential X-rays showed an enlarging right pneumothorax that was managed with chest drainage. After a transient response, blood gases showed persistent severe hypoxemia (PO2 25-34 mmHg). Echocardiography confirmed PPHN with suprasystemic pulmonary pressures (gradient 25 mmHg). Baby was started on maximum vasopressor support, hyperventilated and given sodium bicarbonate in vain. Oral Sildenafil 0.5 mg/kg was tried at 18 hours of age and was repeated in 30 minutes with no response. Baby died at 36 hours of age from persistent hypoxemia and circulatory failure.

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