Persistent pulmonary hypertension of the newborn presenting as the primarymanifestation of intracranial arteriovenous malformation of the Vein of Galen

Arteriovenous malformations of the Vein of Galen continue to present diagno stic and therapeutic challenges in the neonatal period. Approximately 40-50 % of all mal formations of the Vein of Galen present in the neonatal period , usual ly with congestive heart failure. These neonates represent the most severe cases and are also the most difficult to manage. We report a case o f a neonate with a Vein of Galen Malformation who presented with cyanosis, a cardiac murmur, and severe persistent pulmonary hypertension of the newbo rn. Cardiac failure developed later in the patient's course. The degree of pulmonary hypertension on echocardiography was used to time endovascular em bolization of the Vein of Galen Malformation. Following embolization, his p ulmonary hypertension subsided dramatically. We speculate that pulmonary hy pertension associated with Vein of Galen Malformations has been underestima ted in the morbidity and demise of these neonates, and should be more aggre ssively monitored and treated.