Arteriovenous malformation of the vein of Galen (AVG) is a rare entity in the newborn with a high morbidity and mortality. We present two cases of fatal AVG with persistent pulmonary hypertension of the newborn and significant pulmonary hypertension documented by autopsy histopathology. The pathophysiology is reviewed and a proposed mechanism of the association between AVG and pulmonary hypertension is discussed.
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