7/5/2016. Neonatal high-output cardiac failure. Case 1 POSTNATAL STRATEGIES FOR CEREBRAL ATERIOVENOUS MALFORMATIONS

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1 John Deveikis, M.D. POSTNATAL STRATEGIES FOR CEREBRAL ATERIOVENOUS MALFORMATIONS JULY, 2016 Neonatal high-output cardiac failure Tachypnea, tachycardia, hypotension, failure to thrive When congenital heart disease is excluded: Suspect an arteriovenous fistula Case 1 Newborn male 3 kg in size Prenatal ultrasound suggested large heart and big vessels in the head. 1

2 Case 1 Case 1: chest x-ray Case 1: MRI at day 1 2

3 Case 1: MRA day 1 Case 1 Echocardiogram showed high output cardiac failure. Has evidence of an arteriovenous malformation in the brain. Echo showed retrograde flow in the aorta during diastole. AV shunt in the brain was stealing blood from the rest of the body. AVF treatment options Medical management of cardiac compromise Medical management of seizures Direct surgical treatment of AVF Endovascular therapy of AVF 3

4 Case 1 Urgent treatment of the AV malformation needed to manage cardiac failure. Invasive procedures in newborns are high risk. Contrast dose in this 3 kg child should not exceed 12 ml. Vascular structures are small and very fragile. Arterial access in newborn Umbilical artery catheterization. Avoids trauma to tiny femoral artery. Standard UAC can be exchanged for microcatheter using microwire. Sharp curve makes it difficult to use sheath or stiff catheter. Case 1: Angiography 4

5 Case 1: Non-galenic Pial AVF Single hole, or multiple hole sub pial high flow arteriovenous fistula without an intervening nidus. Rare. Represent 7% of pediatric brain arteriovenous lesions (AVM + AVF) in large referral centers. 1 4 Estimated to be present in the general population at a rate of 1/1,000,000 to 1/100, WeonYC, Yoshida Y, Sachet M, et al. Acta Neurochir 2005;147: Yoshida Y, WeonYC, Sachet M, et al. Neuroradiol 2004;46: Berenstein A, Ortiz R, NiimiY, et al. Childs Nerv Syst 2010 Oct;26(10): Hetts SW, Keenan K, Fullerton HJ, et al. Am J Neuroradiol 2012; 33: Pial AVF Mean age of presentation 4.5 years. 1 Can be associated with Rendu Osler Weber syndrome. 2 Like our case, 32% present with high output cardiac failure in early infancy. 1,3 Other presentations: 32% seizures, 24% focal deficit, 16% hemorrhage Hetts SW, Keenan K, Fullerton HJ, et al. Am J Neuroradiol 2012; 33: Oda M, Takahashi JA, Hashimoto N, Koyama T. Surg Neurol 2004 Apr;61(4): Garcia Monaco R, De Victor D, Mann C, et al. Childs Nerv Syst 1991 Feb;7(1):48 52 Case 1: Post-Embolization 5

6 Case 1: Follow-up Embolized a single arterial input with coils. Partial treatment of AV shunt was goal. Patient awoke from anesthesia moving all extremities. On Echo: Cardiac status improved. Post procedure, he did well, had normal feeding, interacted with parents. Planned on further treatment at one year. Case 1: MRI at 4 months Case 1: MRI at 12 months 6

7 Case 1: Angio at 14 months Case 1: Embolization Case 1: Follow up Patient did well and remained neurologically intact. Normal development. Needs long term follow up: 25% develop new dural arteriovenous fistulas in longterm follow up. 1 1.Paramasivam S, Toma N, NiimiY, Berenstein A. J Neurointerv Surg 2013 Jul 1;5(4):

8 Pial AVF: Outcome Series of 16 pial AVF treated with glue+/ coils (as in this case) showed 75% excellent outcome and 19% good outcome. 1 Using mixture of open surgery, embolization, and radiation therapy a series of 25 AVFs had good outcome in 72%. Outcomes were worse in more complex multi hole fistulas and presentation before age 2 years Paramasivam S, Toma N, NiimiY, Berenstein A. J Neurointerv Surg 2013 May;5(3): Hetts SW, Keenan K, Fullerton HJ, et al. Am J Neuroradiol 2012; 33: Case 2:Neonatal high-output failure Tachypnea, tachycardia, hypotension, failure to thrive Transferred at day 3 post natally 2.5 kg strapping young man 8

9 Case 2: High output failure in newborn Classic lesion is vein of Galen malformation(vogm). Subarachnnoid arteriovenous shunts into midline vein (prosencephalic vein of Markowski). Can be fatal when presenting in neonates due to cardiac and/or cerebral compromise Mitchell PJ, Rosenfeld JV, Dargaville P, et al. AJNR Am J Neuroradiol 2001;22: Neonatal AVF: vein of Galen Malformation High flow midline AVF with choroidal vessels draining to a dilated vein of Galen. May present perinatally with high output cardiac failure. Case 2: Management Did not respond to initial medical management with diuretics Cord vessels already dried and necrotic Femoral arterial access much higher risk in neonates under 2.7 kg 1 1. Komiyama M,Terada A, Ishiguro T. Neurol Med Chir (Tokyo) 2016;56:

10 Case 2: Venous-arterial access Neonatal Vein of Galen treatment Survival with primarily transarterial nbca (+/ coils) treatment: 76.9% 1 Transvenous embolization alone associated with worse outcome, and is only used as a last resort Li AH, Armstrong D, ter Brugge. J Neurosug Pediatr. 2011;7: Alvarez H, Garcia Monaco R, Rodesch G, et al. Neurosurgery. 2006;59: Take-away points Careful, staged use of nbca +/- coil embolization is an adjunct to medical Rx of CHF Safe & effective for neonatal AVFs with high expectation of good outcome. Long-term clinical and imaging follow-up is needed. 10