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Cardiac anomalies in the setting of the Abernethy malformation of the portal vein

Published online by Cambridge University Press:  22 December 2006

Christopher Ratnasamy ,
Amethyst Kurbegov  and
Sethuraman Swaminathan
Christopher Ratnasamy
Affiliation:
Division of Pediatric Cardiology, University of Miami – Miller School of Medicine, Miami, Florida, United States of America
Amethyst Kurbegov
Affiliation:
Division of Pediatric Gastroenterology, University of Miami – Miller School of Medicine, Miami, Florida, United States of America
Sethuraman Swaminathan
Affiliation:
Division of Pediatric Cardiology, University of Miami – Miller School of Medicine, Miami, Florida, United States of America
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Abstract

We describe a child with tricuspid atresia associated with a porto-systemic shunt and complete absence of the portal vein, the latter known as the Abernethy malformation. As far as we are aware, this association has not previously been reported. We review the various cardiac defects that have been reported in children with the Abernethy malformation, emphasizing the interesting clinical presentations of this rare malformation of the portal venous system.

Keywords

Tricuspid atresiaporto-systemic shuntencephalopathy
Type
Brief Report
Information
Cardiology in the Young , Volume 17 , Issue 2 , April 2007 , pp. 212 - 214
Copyright
© 2007 Cambridge University Press

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References

Abernethy J. Account of two instances of uncommon formation in the viscera of the human body. Philos Trans R Soc 1793; 83: 59–66.Google Scholar
Massin M, Verloes A, Jamblin P. Cardiac anomalies associated with congenital absence of the portal vein. Cardiol Young 1999; 9: 522–525.Google Scholar
Pipitone S, Garofalo C, Corsello G, et al. Abnormalities of the umbilico-portal venous system in Down syndrome: a report of two new patients. Am J Med Genet A 2003; 120: 528–532.Google Scholar
Ikeda H, Aotsuka H, Nakajima H, Sawada M. Porto-systemic shunt with polysplenia and hypoplastic left heart syndrome. Pediatr Cardiol 2005; 26: 446–448.Google Scholar
Howard ER, Davenport M. Congenital extrahepatic portocaval shunts – the Abernethy malformation. J Pediatr Surg 1997; 32: 494–497.Google Scholar
Kanmori Y, Hashizume K, Kitano Y, Sugiyama M, Motoi T, Tange T. Congenital extrahepatic portocaval shunt (Abernethy type 2), huge liver mass, and patent ductus arteriosus – a case report of its rare clinical presentation in a young girl. J Pediatr Surg 2003; 38: E15.Google Scholar
Volpe P, Marasini M, Caruso G, et al. Prenatal diagnosis of ductus venosus agenesis and its association with cytogenetic/congenital anomalies. Prenat Diagn 2002; 22: 995–1000.Google Scholar
Hishitani T, Hoshino K, Ogawa K, Koyanagi K, Nakamura Y. Perioperative course in two cases of tetralogy of Fallot with portal venous atresia. Pediatr Cardiol 2002; 23: 545–547.Google Scholar
Murray CP, Yoo SJ, Babyn PS. Congenital extrahepatic porto-systemic shunts. Pediatr Radiol 2003; 33: 614–620.Google Scholar
Ersch J, Banziger O, Braegger C, Arbenz U, Stallmach T. An infant with pulmonary hypertension due to a congenital porto-caval shunt. Eur J Pediatr 2002; 161: 660–662.Google Scholar