When Pulmonary Hypertension in the Newborn Masks a Vein of Galen Aneurysmal Malformation!

Abstract

The vein of Galen malformation (VOGM) is a congenital vascular abnormality characterized by several arteriovenous (AV) shunts draining into a dilated porencephalic vein of Markowski, a fetal structure that typically regresses during intrauterine development and forms the precursor of the vein of Galen. While high-output cardiac failure is the classic presentation in neonates, persistent pulmonary hypertension (PPHN) may also occur, complicating the diagnosis. We report a 5-day-old neonate with severe PPHN as the initial manifestation of VOGM. Echocardiographic findings raised a strong suspicion of the malformation, which was subsequently confirmed by computed tomography (CT) angiography. Emergency endovascular embolization (EE) achieves shunt occlusion, resulting in normalized pulmonary pressures and the resolved respiratory distress. VOGM should be considered in neonates with refractory PPHN, particularly when it is associated with high-output cardiac failure. All neonates diagnosed with PPHN should be evaluated to rule out cranial and abdominal AV malformation. Early neuroimaging and endovascular intervention improve outcomes.