Pulmonary Hypertension in Newborn Infants with Vein of Galen Malformation

Objective: Vein of Galen malformation (VGM) is a rare cerebrovascular malformation. Children with VGM presenting in newborn period demonstrate various clinical courses from normal development to severe neurologic impairment or death. The aim of our study was to describe the characteristics of pulmonary hypertension and its treatment response in newborn infants with VGM. Methods: We retrospectively reviewed medical records of newborn infants with VGM who were antenatal diagnosed and treated with endovascular embolization in a single tertiary center. Clinical characteristics including echocardiographic data were described. Results: A total of six patients were diagnosed with VGM. Endovascular embolization was performed early postnatal days (median, 6.5 days) in four patients who required cardiorespiratory support. Echocardiography revealed pulmonary hypertension in the four cases and two of them received targeted therapy against pulmonary hypertension including inhaled nitric oxide and other pulmonary vasodilators. Incomplete occlusion of VGM failed to reverse the pulmonary hypertension. Mortality occurred in two patients with severe pulmonary hypertension in whom incomplete occlusion for VGM lesion. The cause of death was multi-organ failure secondary to right heart failure. Conclusion: Pulmonary hypertension was a common manifestation in newborn case with symptomatic VGM. The efficacy of early embolization and management for pulmonary hypertension in the newborn cases with persistent pulmonary hypertension of the newborn requires further large studies.