C100 Vein of galen malformation: the importance of early diagnosis and treatment in preventing high-output cardiac failure

<h3>Introduction</h3> The Vein of Galen Malformation (VGM) is a rare intracranial vascular malformation in which arteriovenous connections between primary choroidal vessels and the median prosencephalic vein arise between the 6^th and 12^th week of foetal development. The foetus shows no cardiovascular symptoms as the low resistance of the AVM is balanced by the low vascular resistance in the placenta. After birth up to 70% of cardiac output is directed through the low-resistance VGM leading to volume load of the right heart and consecutive heart failure (Frawley <i>et al.</i>, 2002). Untreated the cause is fatal. Therapy of choice is endovascular embolization. <h3>Case Description</h3> A vein of Galen malformation was diagnosed prenatally (SSW 41) in an intrauterine MRI with no prior abnormalities detected during pregnancy. Following diagnosis, the caesarean section was scheduled for the next day with immediate postnatal embolization strategically planned in advance. Initial postnatal echocardiography showed right heart dilation (RV-RA gradient: 40 mmHg). Cerebral DSA confirmed the VGM with multiple macro- and microshunts, mainly from the left pericallosal artery and right posterior choroidal artery (<b>fig. 1</b>). Embolization of the largest macroshunt from the pericallosal artery was performed, reducing shunt volume resulting in reduced fistula flow and hemodynamic improvement (RV-RA gradient post-intervention: 36 mmHg). Clinical and hemodynamic controls followed with plans for staged consecutive embolization in infancy. <h3>Conclusions</h3> Ultraearly embolization therapy of VGMs as well as pre-procedural planning are essential to minimise radiation exposure, reduce high-output heart failure and thus improving the prognosis of affected newborns. <h3>Conflict of Interest</h3> No