Vein of Galen Malformations Presenting as Neonatal Heart Failure
CCCF ePoster library. Francoeur C. 11/11/19; 283453; EP10
Conall Francoeur
Conall Francoeur
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Topic: Clinical Case Report

Di Genova, Tanya1;Francoeur,Conall2;Altit,Gabriel3;Roy,Daniel4;Renaud,Claudia5
1. Pediatric Critical Care, McGill University, Montreal, Canada
2. Pediatric Critical Care, Université Laval, Quebec, Canada
3. Neonatology, McGill University, Montreal, Canada
4. Interventional Neuro-Radiology, Université de Montréal, Montreal, Canada
5. Pediatric Cardiology, McGill University, Montreal, Canada

Introduction: Vein of Galen malformation (VGM), a fistulous connection between cerebral arteries and the vein of Galen,  is an uncommon congenital vascular malformation. It is a rare cause of neonatal heart failure. Cardiac dysfunction severity is related to the degree of shunting though this malformation. Historically, the prognosis was guarded and the mortality, if untreated, high. Endovascular embolization has improved outcomes significantly.
Objective: describe features, evaluation, management and outcomes of VGMs.
Method: case review
Case description: an 8 day-old girl with unremarkable perinatal history was referred to cardiology for murmur evaluation. She had feeding intolerance, tachypnea and lethargy. Examination revealed respiratory distress, weak pulses, a hyperdynamic precordium, a gallop, a continuous murmur heard throughout the precordium and maximally upon auscultation of the anterior fontanel, and hepatomegaly. Echocardiography revealed dilatation of the superior caval vein, severe biventricular dysfunction, severe right ventricular dilation, supra-systemic pulmonary pressure and abnormal diastolic flow reversal in the descending aorta. Brain MRI confirmed a large VGM.
Instability led to intubation and the initiation of milrinone, epinephrine and inhaled nitric oxide. The patient underwent trans-arterial embolization of the main feeding arteries. Intervention led to immediate normalization in heart rate and blood pressure. Pulmonary hypertension and ventricular dysfunction resolved over 24 hours. At 18 months, neurodevelopment was normal.
Discussion: VGM is a rare cause of high-output heart failure and pulmonary hypertension and is associated with high mortality and morbidity1. Pulmonary hypertension is a complication in newborns with VGM2. The large left-to-right shunt results in increased venous return, high pulmonary flow and pulmonary congestion3. These disturbances lead to abnormal post-natal transition and prevent the expected drop in pulmonary vascular resistance.
Pre-intervention priorities include minimizing myocardial demand with appropriate ventilation, sedation and optimization of fluid status. Inhaled nitric oxide may reduce pulmonary vasoconstriction but may exacerbate pulmonary vascular congestion. Prostaglandin may be considered in RV failure, to ensure that the ductus arteriosus remains patent, and in LV failure, to improve systemic output. Milrinone may improve systolic and diastolic function, and induce systemic and pulmonary vasodilatation. Beta-adrenergic agents are not recommended.
The natural progression of VGM is characterized by high morbidity and mortality. Neonates are at particular risk4. Historically, neonates with cardiac failure were untreated due to concern for VGM-associated cerebral infarction and poor neurodevelopmental outcome. With improved management, pre-operative death rates have declined (33 to 5%)4.
Embolization interrupts the high-output state and results in normalization of cardiac function. With neuro-intervention and cardiac failure management, neonates can survive without neurological impairment3. Embolized patients had a reduction in mortality, good outcomes increased by more than 20% and complications decreased significantly5.
Conclusion: Neonates with cardiac failure secondary to VGMs require prompt diagnosis and management.  Clinicians must maintain a high index of suspicion because survival and neurodevelopmental outcomes significantly improve with endovascular neuro-intervention.

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1. Dahdah NS, Alesseh H, Dahms B, Saker F. Severe pulmonary hypertensive vascular disease in two newborns with aneurysmal vein of galen.Pediatr Cardiol.2001;22:538-541.
2. Hendson L, Emery DJ, Phillipos EZ, Bhargava R, Olley PM, Lemke RP. Persistent pulmonary hypertension of the newborn presenting as the primary manifestation of intracranial arteriovenous malformation of the Vein of Galen.American journal of perinatology.2000;17:405-410.
3. Frawley G, Dargaville P, Mitchell P, Tress B, Loughnan P. Clinical course and medical management of neonates with severe cardiac failure related to vein of Galen malformation.Archives of Disease in Childhood-Fetal and Neonatal Edition.2002;87:F144-F149.
4. Jun Yan RG, Jing Wen, Xi-sheng Li, Jing-feng Tang. The natural progression of VGAMs and the need for urgent medical attention: a systematic review and meta-analysis.J Neurointerv Surg.2017;9:564-570.
5. Yan J, Wen J, Gopaul R, Zhang CY, Xiao SW. Outcome and complications of endovascular embolization for vein of Galen malformations: a systematic review and meta-analysis.J Neurosurg.2015;123:872-890.

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