Abstract
Congenital portosystemic shunts (CPSs) may cause myriad manifestations. They can be detected prenatally, in infancy, or later. They may involute, cause acute symptoms, or remain unrecognized and cause chronic disease. As CPSs can require treatment, early diagnosis allows close monitoring. In symptomatic patients, interventional catheterization embolization of CPSs can be undertaken at any age.
Similar content being viewed by others
References
Abernethy J (1793) Account of two instances of uncommon formation in the viscera of the human body. Philos Trans R Soc 83:59–66
Acherman RJ, Evans WN, Galindo A et al (2007) Diagnosis of absent ductus venosus in a population referred for fetal echocardiography: association with a persistent portosystemic shunt requiring postnatal device occlusion. J Ultrasound Med 26:1077–1082
Araki T, Kamada M, Okamaoto Y, Arai S, Oba O (2003) Coil embolization of a patent ductus venosus in a 52-day-old girl with congenital heart disease. Ann Thorac Surg 75:273–275
Avila LF, Luis AL, Encinas JL, et al. (2006) Congenital portosystemic shunt. The Abernethy malformation. Cir Peditr 19:204–209 (in Spanish)
Bellah RD, Hayek J, Teele RL (1989) Anomalous portal venous connection to the suprahepatic vena cava: sonographic demonstration. Pediatr Radiol 20:115–117
Broere D, van Gemert WG, Kneepkens CM et al (2000) A 6-year-old boy with hyperammonaemia: Partial N-acetylglutamate synthase deficiency or portosystemic encephalopathy? Eur J Pediatr 159:905–907
Champneys F (1871) Communication between the external illiac and portal veins. J Anat Physiol 6:417–419
Eroglu Y, Donaldson J, Sorensen LG et al (2004) Improved neurocognitive function after radiologic closure of congenital portosystemic shunts. J Pediatr Gastroenterol Nutr 39:410–417
Gallego C, Miralles M, Marin C et al (2004) Congenital hepatic shunts. Radiographics 24:755–772
Gillespie MJ, Golden A, Sivarajan VB, Rome JJ (2006) Transcatheter closure of patent ductus venosus with the Amplatzer vascluar plug in twin brothers. Pediatr Cardiol 27:142–145
Ikeda H, Aotsuka H, Nakjima H, Sawada M (2005) Portosystemic shunt with polysplenia and hypoplastic left heart syndrome. Pediatr Cardiol 26:446–448
Ikeda S, Sera Y, Yoshida M et al (1999) Successful coil embolization in an infant with congenital intrahepatic portosystemic shunts. J Pediatr Surg 34:1031–1032
Jacob S, Farr G, De Vun D et al (1999) Hepatic manifestations of familial patent ductus venosus in adults. Gut 45:442–445
Kim IO, Cheon JE, Kim WS et al (2000) Congenital intrahepatic portohepatic venous shunt: treatment with coil embolisation. Pediatr Radiol 30:336–338
Kimura T, Soh H, Hasegawa T et al (2004) Laparoscopic correction of congenital portosystemic shunt in children. Surg Laparosc Endosc Percutan Tech 14:285–288
Kitagawa S, Geason W, Northrup H et al (1992) Symptomatic hyperammonemia caused by a congenital portosystemic shunt. J Pediatr 121:917–919
Lange PA, Stoller JK (1995) The hepatopulmonary syndrome. Ann Intern Med 122:521–529
Lewis AM, Aquino NM (1992) Congenital portohepatic vein fistula that resolved spontaneously in a neonate. AJR Am J Roentgenol 159:837–838
Marx M, Huber WD, Crone J et al (2001) Interventional stent implantation in a child with patent ductus venosus and pulmonary hypertension. Eur J Pediatr 160:501–504
Meyer WW, Lind J (1966) Postnatal changes in the portal circulation. Arch Dis Child 41:606–612
Mitchell IM, Pollock JC, Gibson AA (1991) Patent ductus venosus. Pediatr Cardiol 12:181–183
Murayama K, Nagasaka H, Tate K et al (2006) Significant correlations between the flow volume of patent ductus venosus and early neonatal liver function: possible involvement of patent ductus venosus in postnatal liver function. Arch Dis Child Fetal Neonatal Ed 91:F175–F179
Murray CP, Yoo SJ, Babyn PS (2003) Congenital extraheptic portosystemic shunts. Pediatr Radiol 33:614–620
Nishimura Y, Tajima G, Dwi Bahagia A et al (2004) Differential diagnosis of neonatal mild hypergalactosaemia detected by mass screening: clinical significance of portal vein imaging. J Inherit Metab Dis 27:11–18
Ohno T, Muneuchi J, Ihara K, et al. (2008) Pulmonary hypertension in patients with congenital portosystemic venous shunt: a previously unrecognized association. Pediatrics 121:e892–e899
Pattynama MT, Wils A, van der Linden E, van Dijk LC (2007) Embolization with Amplatzer vascular plug in TIPS patients. CardioVasc Interv Radiol 30:1218–1221
Pipitone S, Garofalo C, Corsello G et al (2003) Abnormalities of the umbilio-portal venous systems in Down syndrome: a report of two patients. Am J Med Genet 120:528–532
Rouveyrol F, Meyer M, Lusson JR et al (2006) Idiopathic intrahepatic portosystemic shunts in 4 children. Arch Pediatr 13:251–255 (in French)
Sagiv-Friedgut K, Witzling M, Dalal I et al (2007) Congenital patent ductus venosus: an association with hyper IgE syndrome. J Pediatr 150:210–212
Satoh M, Yokoya S, Hachiya Y et al (2001) Two hyperandrogenic adolescent girls with congenital portosystemic shunt. Eur J Pediatr 160:307–311
Saxena KS, Kushaljit SS, Arora J et al (2004) Congenital intrahepatic portosystemic venous shunt in an infant with Down syndrome. AJR Am J Roentgenol 183:1783–1784
Schwartz YM, Berkowitz D, Lorber A (1999) Transvenous coil embolization of a patent ductus venosus in a 2-month-old child. Pediatrics 103:1045–1047
Stringer MD (2008) The clinical anatomy of congenital portosystemic venous shunts. Clin Anat 21:147–157
Tanoue S, Kiyosue H, Komatsu E et al (2003) Symptomatic intrahepatic portosystemic venous shunt: embolization with an alternative approach. AJR Am J Roentgenol 181:71–78
Uchino T, Endo F, Ikeda S et al (1996) Three brothers with progressive hepatic dysfunction and severe hepatic steatosis due to a patent ductus venosus. Gastroenterology 110:1964–1968
Uchino T, Matsuda I, Endo F (1999) The long-term prognosis of congenital portosystemic venous shunt. J Pediatr 135:254–256
Watanabe A (2000) Portal-systemic encephalopathy in non-cirrhotic patients: classification of clinical types, diagnosis and treatment. J Gastroenterol Hepatol 15:969–979
Yoon HK, Choo SW, Do YS et al (1998) Congenital intrahepatic portosystemic shunts in the neonate: coil embolization via the umbilical vein. J Vasc Interv Radiol 9:509–511
Yoshimoto Y, Shimizu R, Saeki T et al (2004) Patent ductus venosus in children: a case report and review of the literature. J Pediatr Surg 39:1–5
Acknowledgment
We are grateful to Paul R. Lurie, Senior Consultant to Children’s Heart Center—Nevada and Emeritus Professor of Pediatrics University of Southern California, for suggestions and editorial assistance.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Evans, W.N., Galindo, A., Acherman, R.J. et al. Congenital Portosystemic Shunts and AMPLATZER Vascular Plug Occlusion in Newborns. Pediatr Cardiol 30, 1083–1088 (2009). https://doi.org/10.1007/s00246-009-9501-7
Received:
Revised:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00246-009-9501-7