Pediatr Cardiol 14:102-106, 1993 Case Reports Pediatric Cardiology 9 Springer-Verlag New YorkInc. 1993 Congestive Heart Failure in Neonates Due to Intracraniai Arteriovenous Malformation: Endovascular Treatment Michael E. McConnell, I Patricia Aronin, 2 and Jiri J. Vitek 3 Departments of ~Pediatrics, 2 Neurosurgery, and 3 Radiology, The University of Alabama at Birmingham, Birmingham, Alabama, USA SUMMARY. Newborns with intracraniai arteriovenous malformations and congestive heart failure have an extremely poor prognosis. This report describes two infants with intraeranial arteriovenous malformations and severe congestive heart failure successfully treated in the newborn period with endovascular embolization. Both infants had immediate improvement in symptoms and both had second embolization procedures performed. Ventriculoperitoneal shunting was necessary in both children because of progressive hydrocephalus. At follow-up of more than 4 years, neither patient has any sign of congestive heart failure, and one is develop- mentally normal. KEY WORDS: Congestive heart failure--Intraeranial arteriovenous malformations Neonates Intracranial arteriovenous malformations causing congestive heart failure in the neonatal period are rare, and survival of these infants is quite poor [7]. Johnston et al. [9], in a comprehensive review of vein of Galen malformations, reported a mortality of 91% for patients presenting in the newborn pe- riod with congestive heart failure secondary to a vein of Galen aneurysm. Intracranial arteriovenous malformations of the epidural vessels are even more rare with few reported cases [1]. In this report we describe the clinical presenta- tion, diagnostic studies, management, and follow- up of two neonates with cranial arteriovenous mal- formations who presented with congestive heart failure in the first week of life. Case Report Case 1 KT was born after an uncomplicated term pregnancy. She was noted on the second day of postnatal life to have tachypnea and a Presented at the meeting, Pediatric Cardiology, Havana, Cuba, November 26-30, 1990. Address offprint requests to: Dr. Michael E. McConnell, Pediat- ric Cardiology, UAB Station, Birmingham, AL 35294-0016, USA. heart murmur. Cardiac evaluation showed markedly increased precordial activity, a normal first heart sound, a loud second heart sound, and a grade 3/6 systolic murmur along the upper left sternal border. The femoral pulses were diminshed. The liver edge was palpable 2 cm below the right costal margin. No cranial bruit was audible. The chest radiograph showed an enlarged car- diothymic silhouette and increased pulmonary vascular mark- ings. The two-dimensional echocardiogram showed generally en- larged chambers, normal ventricular function, and a left aortic arch which was small in the region adjacent to the left subclavian artery. The size of the superior vena cava was not noted. Con- trast echocardiography showed a right-to-left shunt at ductal level. The patient subsequently had a cardiac catheterization performed with a presumptive diagnosis of coarctation of the aorta. During cardiac catheterization saturation of the superior vena cava blood was 93% suggesting an intracranial arterioven- ous malformation or anomalous pulmonary venous connection. A left ventriculogram showed early appearance of contrast in the superior vena cava. A second left ventriculogram with the cam- eras focused on the cranium confirmed the diagnosis. Both ca- rotid and vertebral arteries were significantly enlarged. The arte- riovenous malformation with a significant enlargement of the vein of Galen was supplied through multiple branches of the posterior, anterior, and middle cerebral arteries (Fig. 1A). Be- cause of severe congestive failure (tachycardia, tachypnea, in- creased work of breathing, and hepatomegaly) the patient under- went embolization of the vein of Galen at 11 days of age. Through a small craniectomy over the torcular a short 5F cathe- ter was introduced over a guidewire into the vein of Galen aneu- rysm. Two 15-cm pieces of floppy J guidewire were introduced into the vein of Glen aneurysm. Two 8-mm thrombogenic coil