Objective: Vein of Galen malformation (VGAM) in neonates presents a complex management challenge. Measurement of superior vena cava (SVC) blood flow may provide insights into the haemodynamics of VGAM and the effects of therapeutic intervention.
Methods: SVC flow was assessed in 15 neonates with VGAM. SVC flow results, Bicêtre scores (clinical assessment), echocardiographic assessment and clinical outcomes are presented.
Results: SVC flows (166-581 ml/kg/min) were significantly elevated at presentation (p<0.001; normal range 55-111 ml/kg/min). Endovascular intervention was undertaken in 12 cases, with nine survivors. SVC flows decreased sequentially with each embolisation, with a median SVC flow at discharge of 124 ml/kg/min (IQR 79-155 ml/kg/min). All cases with SVC flow >400 ml/kg/min (n=5) had an adverse outcome (death or profound neurological damage). Cases with SVC flow <400 ml/kg (n=10) required embolisation before discharge at a median age of 6 days. There were no survivors with Bicêtre scores <8 (n=2) but the predictive value of early Bicêtre score was poor.
Conclusions: SVC flow measurements provide insight into the haemodynamic challenges of VGAM and provide additional useful prognostic information.