Congenital hepatic arteriovenous malformations are rare anomalies, which typically present in infancy with congestive heart failure, anemia, and hepatomegaly. Morbidity and mortality is high if the condition is not recognized and treated promptly. Hepatic arteriovenous malformation associated with persistent pulmonary hypertension of the newborn has been reported in two cases in the literature. We report a neonate who was referred for management of persistent pulmonary hypertension and was subsequently diagnosed with a large hepatic arteriovenous malformation. He underwent coil embolization following which pulmonary hypertension resolved.
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Gersony WM . Neonatal pulmonary hypertension: pathophysiology, classification, and etiology. Clin Perinatol 1984; 11 (3): 517–524.
Knudson RP, Alden ER . Symptomatic arteriovenous malformation in infants less than 6 months of age. Pediatrics 1979; 64 (2): 238–241.
Oden J, Cheifetz IM . Neonatal thyrotoxicosis and persistent pulmonary hypertension necessitating extracorporeal life support. Pediatrics 2005; 115 (1): e105–e108.
Long WA, Schall SA, Henry GW . Cerebral arteriovenous malformation presenting as persistent fetal circulation. Diagnosis by cross-sectional echo. Am J Perinatol 1984; 1 (3): 236–241.
Long WA . Structural cardiovascular abnormalities presenting as persistent pulmonary hypertension of the newborn. Clin Perinatol 1984; 11 (3): 601–626.
Eide J, Folling M . Malformation of the great vein of Galen with neonatal heart failure. Report of two cases. Acta Paediatr Scand 1978; 67 (4): 529–532.
Hendson L, Emery DJ, Phillipos EZ, Bhargava R, Olley PM, Lemke RP . Persistent pulmonary hypertension of the newborn presenting as the primary manifestation of intracranial arteriovenous malformation of the Vein of Galen. Am J Perinatol 2000; 17 (8): 405–410.
Dahdah NS, Alesseh H, Dahms B, Saker F . Severe pulmonary hypertensive vascular disease in two newborns with aneurysmal vein of galen. Pediatr Cardiol 2001; 22 (6): 538–541.
Mulrooney DA, Carpenter B, Georgieff M, Angel C, Hunter D, Foker J et al. Hepatic mesenchymal hamartoma in a neonate: a case report and review of the literature. J Pediatr Hematol Oncol 2001; 23 (5): 316–317.
Bamford MF, De Bono D, Pickering D, Keeling JW . An arteriovenous malformation of the liver giving rise to persistent transitional (fetal) circulation. Arch Dis Child 1980; 55 (3): 244–245.
Paley MR, Farrant P, Kane P, Heaton ND, Howard ER, Karani JB . Developmental intrahepatic shunts of childhood: radiological features and management. Eur Radiol 1997; 7 (9): 1377–1382.
Boon LM, Burrows PE, Paltiel HJ, Lund DP, Ezekowitz RA, Folkman J et al. Hepatic vascular anomalies in infancy: a twenty-seven-year experience. J Pediatr 1996; 129 (3): 346–354.
Subramanyan R, Narayan R, Costa DD, Derweesh A, Khusaiby SM . Transcatheter coil occlusion of hepatic arteriovenous malformation in a neonate. Indian Heart J 2001; 53 (6): 782–784.
Gallego C, Miralles M, Marin C, Muyor P, Gonzalez G, Garcia-Hidalgo E . Congenital hepatic shunts. Radiographics 2004; 24 (3): 755–772.
Fellows KE, Hoffer FA, Markowitz RI, O'Neill Jr JA . Multiple collaterals to hepatic infantile hemangioendotheliomas and arteriovenous malformations: effect on embolization. Radiology 1991; 181 (3): 813–818.
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Alexander, C., Sood, B., Zilberman, M. et al. Congenital hepatic arteriovenous malformation: an unusual cause of neonatal persistent pulmonary hypertension. J Perinatol 26, 316–318 (2006). https://doi.org/10.1038/sj.jp.7211493
- pulmonary hypertension
- arterio-venous malformation
- respiratory distress
- coil empolization
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