Aims Vein of Galen malformation (VGAM) presenting in the neonatal period presents a complex management challenge. Measurement of superior vena cava (SVC) blood flow, reflecting venous return from the head and neck, may provide insight into both the haemodynamics of VGAM and the effects of therapeutic intervention.
Methods SVC flow was assessed at presentation and following endovascular intervention in 15 neonates with VGAM. SVC flow results, Bicetre scores (clinical assessment), standard echocardiographic assessment and clinical outcomes are presented.
Results SVC flows (166–1858 ml/kg/min) were significantly elevated (p<0.01) (normal range 55–111 ml/kg/min) at presentation. Endovascular intervention was undertaken in 12 cases with 8 survivors. SVC flows fell sequentially with embolisation with a median SVC flow at discharge of 113 ml/kg (79–160). There were significant associations between survival and SVC flow (p<0.01), SVC diameter (p<0.01) and retrograde flow patterns in the aorta at presentation (p<0.01). Cases with SVC flows >400 ml/kg (n=5) were either refused intervention, or died post intervention, because of intractable multiorgan failure. All cases with SVC flow 300–400 ml/kg (n=10) required urgent intervention; deaths (n=2) were related to peri embolisation intracranial haemorrhage. Cases with SVC flow <300 ml/kg (n=2) survived. There were no survivors with either SVC diameter >8 mm at presentation or continuous retrograde flow in the aorta. Bicetre scores at initial presentation did not predict survival although a Bicetre score below 8 was associated with poor outcome.
Conclusions SVC flow measurements provide insight into the haemodynamic challenges of this condition and provide additional useful prognostic information.
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