Iran J Pediatr Case Report Apr 2013; Vol 23 (No 2), Pp: 229-232 Aortic Runoff as a Sign of Intracranial Arteriovenous Malformation: Report of Two Cases Maryam Moradian*, MD; Paridokht Nokhostin-Davari, MD; Mahmood Merajie, MD; Hamid-Reza Pouraliakbar, MD Pediatric Cardiology and Radiology Departments of Rajaie Cardiovascular Medical and Research Center, Tehran University of Medical Sciences, Tehran, Iran May 31, 2011 Dec 29, 2011 Mar 12, 2012 Received: ; Final Revision: ; Accepted: Abstract Background: Intracranial arteriovenous malformation rarely causes pulmonary hypertension and congestive heart failure in the newborn. Its diagnosis is challenging because cardiomegaly may suggest an intra-cardiac structuralCase Presentation lesion. : We present two newborns, one 2-day-old male and the other 11-day-old female, with intracranialConclusion: arteriovenous malformation and misdiagnosis of congenital heart disease. Precise echocardiography revealed the secondary signs of cranial arteriovenous malformation Iranianand hadJournal theof majorPediatrics role, Volume in early23 diagnosis.(Number 2), Apr 2013, Pages: 229-232 Key Words: Dilated brachiocephalic arteries; Congestive heart failure; Doppler echocardiography Introduction rarely result in hemodynamic symptoms. Systemic AVMs with profound hemodynamic effects occur Congenital vascular malformations are structural in the brain, liver, thorax, and extremities. Central abnormalities that result from arrests in normal nervous system AVMs manifest symptoms morphogenetic processes. Arterial malformations according to their hemodynamic effects. Infants have two vascular patterns: arteriovenous presenting with congestive[1] heart failure (CHF) malformations (AVMs) and arteriovenous fistulas typically have large AVFs . (AVFs). AVMs (microfistulas) are multiple arterial AVF is a relatively rare cause of[3] severe feeders joined via a nidus to draining veins. AVFs congestive heart failure in the newborn . If flow (macrofistulas) are direct shunts[1] between large is increased because of shunting from a deep AVM arterial and venous channels . They[2] do not have or by direct arterial communication,[4] the vein of the normal intervening capillary bed . Galen may become massively dilated . Most lesions affect both sexes equally, except An aneurysm of the vein of Galen consists of an cerebral AVMs, which affect males more AVM involving the carotid and or vertebrobasilar frequently than females. Arterial malformations circulation in direct communication with the great * Corresponding Author; Address: Shaheed Rajaie Cardiovascular Medical and Research Center, Valiasr St, Tehran, Iran E-mail: [email protected] © 2012 by Pediatrics Center of Excellence, Children’s MedicalIran Center, J Pediatr; Tehran Vol 23 University (No 2), Apr of2013 Medical Sciences, All rights reserved. Published by: Tehran University of Medical Sciences (http://ijp.tums.ac.ir) 230 Aortic Runoff and Intracranial AVenous Malformation; M Moradian, et al cerebral vein. systolic and diastolic cardiac murmur, loud second An arterial branch may enter the vein directly, heart sound and continuous murmur over skull. or there may be a complex racemose network of Chest X-ray revealed cardiomegaly and ECG non-capillary vessels[5] interposed between the showed right axis deviation. artery and vein . Transthoracic echocardiography showed They may present later in infancy or early enlarged right heart chambers including right childhood with hydrocephalus, seizures, or focal atrium, right ventricle and pulmonary artery. or generalized neurologic signs and symptoms. Superior vena cava was enlarged and Smaller AVMs within the brain parenchyma brachiocephalic arteries were tortuous and frequently present in childhood with signs of dilated. cerebral or subarachnoid hemorrhage, occurring Evaluation via color Doppler showed diastolic from the AVM[1] itself or from a coexisting arterial flow reversal (diastolic run off) in descending and aneurysm . abdominal aorta without aortic valvular regurgitation, coronary artery fistula, aortico- pulmonary window or patent ductus arteriosus. This diastolic flow reversal was confirmed by Case Presentation pulsed Doppler study (Fig 1 and 2). Patient had moderate tricuspid valve regurgitation with 50 mmHg pressure gradient A 2-day–old male newborn was referred to our (pulmonary hypertension) and right to left shunt hospital for cardiac surgery with diagnosis of at foramen ovale and ductal level. Pulmonary Total Anomalous Pulmonary Venous Connection veins had normal connection and drainage to (TAPVC). His medical history revealed that he was posterior left atrial wall and there was no cardiac born after an uneventful normal vaginal delivery anomaly. with normal Apgar score. Gradually during the According to echocardiographic findings and first 24 hours he became cyanotic, his reflexes high suspicion for cranial AVF, brain sonography decreased and the level of consciousness reduced was performed which showed echolucsent cystic and he experienced one episode of seizure. lesion within brain. Brain CT scan showed linear On physical examination the remarkable calcification and atrophic changes of right side findings consisted of tachypnea (78/min), cerebral hemisphere. tachycardia (137/min), decreased oxygen Administration of contrast material demons- saturation (80% with oxygen 100%), reduced trated marked dilation of the vein of Galen asso- consciousness, reduced neonatal reflexes, weak ciated with dilation of transverse sinus and the peripheral pulses, widened carotid pulse pressure, straight sinus (Fig. 3). Fig. 1: Fig. 2: Color Doppler echocardiogram from suprasternal Pulsed Doppler tracing in abdominal aorta view showing reverse flow in descending aorta showing reverse flow throughout diastole Iran J Pediatr; Vol 23 (No 2), Apr 2013 Published by: Tehran University of Medical Sciences (http://ijp.tums.ac.ir) Iran J Pediatr, Vol 22 (No 2); Apr 2012 231 Fig. 4: CXR on age of 11 days showing severe cardiomegaly Fig. 3: embolization of afferent arteries for this patient Multislice CT scan with contrast. Coronal view was successful. Now, after 6 months follow up showing dilated neck arteries and veins and atrophy of baby is in good condition with normal cardiac right hemisphere function and no neurologic complication (Fig. 5). After confirmation of no congenital heart disease and presence of cereberal AV malformation as the cause of congestive heart failure by these modalities, we consulted a neurologist but unfortunately by awareness of poor prognosis the parents refused any more treatmentCase 2 and discharged the baby from hospital. An 11-day-old female newborn was referred to us with the diagnosis of cardiomyopathy. She was born by cesarian section and discharged from nursery in good condition. Four days later she was Fig. 5: visited by pediatrician because of jaundice, he CXR some days after transcatheter embolization of noticed cardiac murmur and severe cardiomegaly cranial AV malformation showing decreased cardiac size on chest x-ray and referred her to our hospital for more cardiac evaluation by initial diagnosis of cardiomyopathy at the age of 11 days (Fig. 4). Discussion On physical examination she had tachypnea (60/min), tachycardia (160/min), and grade 3/6 systolic cardiac murmur, loud second heart sound and continuous murmur over skull. Precise Cerebral AV malformation enlarging the vein of echocardiography revealed right to left shunt via Galen is rare. This defect is the result of an AV PFO and small PDA and reduced cardiac ejection malformation or fistula that increases flow fraction. Brachiocephalic arteries were dilated and through the vein of Galen and deep venous system, there was retrograde aortic runoff in descending causing aneurysmal dilatation. Newborn infants thoracic aorta in diastole. There was no PDA, AP with this disorder often develop symptoms of window and no aortic regurgitation so she was congestive heart failure and cyanosis. Infants with highly suspicious of cranial AV fistula. More severe congestive heart failure caused by an diagnostic evaluation by brain sonography and CT intracranial AV malformation are critically ill, and scanning confirmed the diagnosis. Transcatheter prompt diagnosis is essential. Two-dimensional Iran J Pediatr; Vol 23 (No 2), Apr 2013 Published by: Tehran University of Medical Sciences (http://ijp.tums.ac.ir) 232 Aortic Runoff and Intracranial AVenous Malformation; M Moradian, et al ultrasonography of the heart and brain provides a examination in children which is the cranial rapid, efficient method for detection of auscultation. intracranial AV malformation. Doppler examination of descending aorta shows evidence [6] Conclusion of retrograde diastolic flow . The common causes of reversal of flow in the aortic arch are lesions in which a communication exists between the ascending aorta, the proximal Cranial AV malformation causes volume aortic arch or its branches, and a lower pressure overloading and cyanosis due to persistent fetal chamber or channel (such as pulmonary vessels, circulation and most of these infants are at first ventricles, or systemic veins), that results in considered to have congenital heart disease. "stealing'' of blood from the distal aortic arch and Careful echocardiography, by demonstrating descending aorta (example: aorticopulmonary normal intracardiac anatomy via two-dimentional window, severe aortic valve regurgitation, mode and stealing of blood in cranial region by systemic arteriovenous (A-V) malformation