BACKGROUND Successful management of a vein of Galen malformation (VoGM) in the newborn patient requires a highly coordinated team approach involving neonatologists, pediatric cardiologists, pediatric neurologists, neurosurgeons, and interventionalists. Indication and timing of catheter intervention are topics of ongoing debate. OBSERVATIONS The authors highlighted two key echocardiographic markers believed to be practical indicators regarding the need for urgent catheter embolization in neonates with a VoGM. The first and preferred parameter was the tricuspid valve regurgitation (TR) gradient, an estimate of pulmonary artery hypertension. If the TR gradient exceeds systolic blood pressure (suprasystemic pulmonary hypertension [PH], i.e., >60 mm Hg), urgent intervention should be considered in eligible newborns. The second parameter was the left ventricular end-systolic eccentricity index (EI), a newly emerging echocardiographic marker and indirect correlate of PH. As an alternative to the TR gradient, an increased eccentricity index (>1.6) suggests severe right heart compromise, requiring emergency catheter embolization of the malformation. Postoperatively, the progressive reduction of both the TR gradient and the EI correlated with recovery. LESSONS In eligible newborns, urgent embolization of a VoGM is recommended in the presence of suprasystemic TR gradients and/or increased EI >1.6.

BACKGROUND Vein of Galen malformations (VoGMs) in newborns often represent life-threatening emergencies. Outcome is difficult to predict. The authors review 50 VoGM cases to correlate anatomical types with treatment and outcome. OBSERVATIONS Four distinct types of VoGMs are identified: mural simple (type I), mural complex (type II), choroidal (type III), and choroidal with deep venous drainage (type IV). Seven patients presented with mural simple VoGMs with a “single hole” fistula supplied by only one large feeder. These patients were treated electively at >6 months; development was normal. Fifteen patients presented with complex mural VoGMs. Multiple large feeders joined a single fistulous point within the wall of the varix. Patients typically presented with congestive heart failure (CHF) and required emergent transarterial intervention. Mortality was 7.7% with less than two-thirds developing normally. Twenty-five patients presented with choroidal VoGMs. Multiple large arterial feeders joined at multiple fistulous sites. Severe CHF in most patients required emergent transarterial and sometimes transvenous intervention. Mortality was 9.5%; two-thirds of the patients had a normal development. Three babies presented with choroidal VoGMs with deep intraventricular venous drainage. This phenomenon caused fatal “melting brain syndrome” in all three patients. LESSONS Recognition of the specific VoGM type determines treatment options and sets outcome expectations.