Vein of Galen aneurysmal malformation presenting as severe heart failure in a neonate

The vein of Galen aneurysmal malformation (VGAM) is a rare cerebral arteriovenous malformation that can be life-threatening if not diagnosed and treated early. VGAM usually presents in the neonatal period with high-output cardiac failure. We report the case of a full-term male neonate who presented with respiratory distress, and a fontanel bruit soon after birth. A chest radiograph revealed marked cardiomegaly. Transthoracic echocardiography showed dilatation of all four cardiac chambers and a patent ductus arteriosus. Transfontanellar doppler ultrasound and brain computed tomography confirmed the diagnosis of a VGAM. Clinical worsening took place despite aggressive hemodynamic and ventilatory support. The patient's Bicêtre Neonatal Evaluation Score for embolization was 2. Endovascular treatment could not be performed. The patient regretfully passed away. VGAM should be considered in the differential diagnosis of neonatal congestive heart failure with a structurally normal heart. Early diagnosis and treatment improve prognosis considerably.