Persistent pulmonary hypertension of the newborn presenting as the primary manifestation of intracranial arteriovenous malformation of the Vein of Galen.

Arteriovenous malformations of the Vein of Galen continue to present diagnostic and therapeutic challenges in the neonatal period. Approximately 40-50% of all malformations of the Vein of Galen present in the neonatal period, usually with congestive heart failure. These neonates represent the most severe cases and are also the most difficult to manage. We report a case of a neonate with a Vein of Galen Malformation who presented with cyanosis, a cardiac murmur, and severe persistent pulmonary hypertension of the newborn. Cardiac failure developed later in the patient's course. The degree of pulmonary hypertension on echocardiography was used to time endovascular embolization of the Vein of Galen Malformation. Following embolization, his pulmonary hypertension subsided dramatically. We speculate that pulmonary hypertension associated with Vein of Galen Malformations has been underestimated in the morbidity and demise of these neonates, and should be more aggressively monitored and treated.