Vein of Galen aneurysmal malformations (VGAMs) in infancy have a poor natural history if left untreated. Their high-flow nature can preclude safe and accurate therapeutic vessel occlusion and the risk of inadvertent pulmonary embolism is predominant. The authors describe the technique of rapid cardiac ventricular pacing for inducing transient hypotension to facilitate the controlled embolization of VGAMs.
Initial transjugular venous access was obtained for placing temporary pacing leads for rapid cardiac ventricular pacing immediately prior to embolization. Definitive transarterial embolization procedures for the VGAMs were then performed in the same setting in which liquid embolic agents or coils were used.
Beginning in 2010, a total of five procedures were performed in three infants. Transvenous rapid cardiac ventricular pacing was successfully achieved to induce systemic transient flow arrest in all but two attempts, and facilitated partial embolization with n-butyl cyanoacrylate (n-BCA) and coils in all procedures. Ventricular fibrillation occurred twice in one patient and was successfully reversed with defibrillation on both occasions. One patient failed to improve and died from refractory heart failure. Two patients stabilized following staged embolization.
Rapid transvenous cardiac ventricular pacing can be considered to induce transient hypotension and facilitate controlled embolization in challenging high-flow VGAMs.
ABBREVIATIONSAVM = arteriovenous malformation; MAP = mean arterial pressure; n-BCA = n-butyl cyanoacrylate; NICU = neonatal intensive care unit; RVOT = right ventricle outflow tract; VGAM = vein of Galen aneurysmal malformation.
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