Repair of sinus venosus defect in a patient with untreated vein of Galen aneurysmal malformation

Abstract

The vein of Galen aneurysmal malformation (VGAM) is a rare cerebral arteriovenous shunt, which may be associated with a congenital cardiac defect. Embolisation of the VGAM may be undertaken in the neonatal period if necessary, but is safer in infancy. Recent advances in neuroradiology have changed the prognosis for this group with many patients achieving survival with normal development. This case report describes a patient with a sinus venosus defect (SVD) and a VGAM and considers both the optimal timing of treatment of the two malformations and the conduct of anaesthesia for open repair of the SVD in the presence of an untreated VGAM.

Repair of Sinus Venosus Defect and Anomalous Pulmonary Veins Associated With Vein of Galen Malformation

“…However, this gradually improved to 50% during the procedure and stabilized to prebypass levels following surgery. 8 The patient subsequently had an uneventful postoperative recovery and no documented neurologic sequelae during two years of follow-up.…”

An Uncommon Presentation of a Rare Disorder - Ruptured Vein of Galen Malformation with Atrial Septal Defect

“…The VOG also develops around the same period between 6-11 weeks. In some cases the superior vena cava can override the septal defect promoting direct drainage of venous return into the left atrium, thus facilitating paradoxical embolization [11]. VAGMs are most commonly detected in utero or after birth because exclusion of the low resistance placental flow causes an abrupt increase in flow across the fistula and almost 80% of cardiac output is diverted to brain in severe cases.…”

“…VAGMs are most commonly detected in utero or after birth because exclusion of the low resistance placental flow causes an abrupt increase in flow across the fistula and almost 80% of cardiac output is diverted to brain in severe cases. This causes a compensatory increase in cardiac output and blood volume to maintain systemic perfusion leading to its most common presentation, high output cardiac failure [10,11].…”

A cardiopulmonary bypass strategy to support a patient with vein of Galen malformation

“…Patients with a combination of vein of Galen malformation (VGM) and congenital cardiac disease present a challenge in the setting of cardiopulmonary bypass (CPB) and has been reported only once before. 1 Other authors describe poor outcome without intervention or a hybrid 'peratrial' approach with the intent of avoiding CPB. 2,3 A previous case series recommended 'conservative management' until the VGM had been successfully eradicated.…”