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Superior vena cava flow and management of neonates with vein of Galen malformation

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.