Transvenous Pressure Monitoring Guides Endovascular Treatment of Vein of Galen Malformation: A Technical Note

Abstract

<b><i>Introduction:</i></b> Vein of Galen malformations (VGMs) are complex congenital arteriovenous malformations that generally require serial endovascular treatment sessions to slowly correct the high-flow fistulous connections that cause increased venous pressures and ultimately lead to the classic presentations of heart failure, hydrocephalus, and intracranial hemorrhages. Despite the advances in endovascular technology and embolic materials, the resolution of embolization is often limited to the subjective view of diminished flow on angiograms. <b><i>Case Report:</i></b> An 8-month-old patient with a VGM developed clinical signs of heart failure and growing head circumference with ventriculomegaly. The patient was treated endovascularly with a transvenous approach for coil embolization while undergoing continuous monitoring of the post-malformation venous pressures. The arterial and venous systolic blood pressures (SBP) were collected at serial time points and used to measure estimated 95% confidence interval bounds for arteriovenous SBP gradients and determine when sufficient coil embolization and flow reduction was thought to be achieved. <b><i>Conclusion:</i></b> The transvenous pressure monitoring demonstrated progressively increasing pressure gradients between the arterial and venous systems that correlated with the degree of flow reduction on angiographic runs. The patient underwent successful coil embolization of the VGM and had improvement of heart failure and ventricular size in follow-up at 8-month post-op. This provides a novel technique to introduce an objective measurement that can guide the embolization of a VGM.

Doppler Ultrasound Flow Reversal in the Superior Sagittal Sinus to Detect Cerebral Venous Congestion in Vein of Galen Malformation

“…The proportion of pathologic FPs in the SSS decreased after partial superselective embolization, likely due to effective shunt reduction with concomitant pressure reduction in the cerebral venous system. 5 In 1 patient, retrograde arterialized flow in the SSS occurred before discharge. Retrospectively, the increasing flow reversal resembled a venous outflow occlusion caused by bilateral stenosis of the jugular bulb.…”

“…Arterialization of the MPV and the cerebral sinus causes a massive increase in cerebral venous pressure, with subsequent functional outflow obstruction, leading to congestion, venous ischemia, and hydrocephalus malresorptivus. [5][6][7][8] Cerebral venous hypertension can be exacerbated by high venous return due to excessive shunt volumes with cardiac volume overload and backward failure of the right heart. 9 Furthermore, a maturation disorder of the sinu-jugular connection due to flow stress in the vessel wall may lead to bilateral stenosis of the jugular bulb, further increasing cerebral venous pressure.…”

Vein of Galen malformations in the newborn: case series

“…VoGMs are exceptionally rare with an incidence of only 1 in 3 million. [36][37][38][39][40][41][42][43][44][45][46][47][48][49][50][51] Therefore, we elected to supplement our case series with additional cases from the literature to further characterize VoGMs subtypes I-IV in greater detail. This constitutes the second major limitation, namely selection bias, because authors generally tend to report their better cases only.…”