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The Abernethy malformation with inferior caval vein hypoplasia: a tailored technique for transcatheter closure and an insight into embryological perspective

Part of: Cardiac Morphology

Published online by Cambridge University Press:  06 July 2018

Ronak Sheth  and
Kothandam Sivakumar
Ronak Sheth
Affiliation:
Department of Pediatric Cardiology, Institute of Cardio Vascular Diseases, Madras Medical Mission, Chennai, India
Kothandam Sivakumar*
Affiliation:
Department of Pediatric Cardiology, Institute of Cardio Vascular Diseases, Madras Medical Mission, Chennai, India
*
Address for correspondence: Dr K. Sivakumar MD, DM, Head of Department of Pediatric Cardiology, Institute of Cardio Vascular Diseases, Madras Medical Mission, 4A, Dr J J Nagar, Mogappair, Chennai 600089, India. Tel: +91 9444449966; Fax +91 44 26565859; E-mail drkumarsiva@hotmail.com
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Abstract

Abernethy malformations manifest as hepatopulmonary syndrome, pulmonary vasculopathy, or encephalopathy. A novel intervention in a child with portosystemic shunt and inferior caval vein hypoplasia led to complete normalisation of hypoxia and relief of obstruction in the inferior caval vein. Embryological explanations of venous anomalies may indicate that inferior caval vein anomalies are frequent but under-recognised in patients with Abernethy malformation.

Keywords

Abernethy malformationportosystemic shunthepatopulmonary syndromeinferior caval vein hypoplasiacovered stent
Type
Brief Report
Information
Cardiology in the Young , Volume 28 , Issue 9 , September 2018 , pp. 1169 - 1171
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Copyright
© Cambridge University Press 2018 

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Footnotes

Cite this article: Sheth R, Sivakumar K. (2018) The Abernethy malformation with inferior caval vein hypoplasia: a tailored technique for transcatheter closure and an insight into embryological perspective. Cardiology in the Young28: 1169–1171. doi: 10.1017/S1047951118000884

References

1. Howard, ER, Davenport, M. Congenital extrahepatic portocaval shunts – the Abernethy malformation. J Pediatr Surg 1997; 32: 494–497.Google Scholar
2. Yoshidome, H, Edwards, M. An embryological perspective on congenital portacaval shunt: a rare anomaly in a patient with hepatocellular carcinoma. Am J Gastroenterol 1999; 94: 2537–2539.Google Scholar
3. Soni, N, Ambesh, P, Sunil, K, Jena, M. Abernethy malformation with inferior vena cava stenosis: a cryptic cause of cyanosis. Indian Heart J 2015; 67: 543–545.Google Scholar
4. Gupta, NA, Abramowsky, C, Pillen, T, et al. Pediatric hepatopulmonary syndrome is seen with polysplenia/interrupted inferior vena cava and without cirrhosis. Liver Transpl 2007; 13: 680–686.Google Scholar
5. Bercoff, E, Colin, R, Benozio, M, Janvresse, A, Berland, J, Fillastre, JP. Infrahepatic interruption of the inferior vena cava with portal continuation. Radiology 1985; 154: 771–772.Google Scholar
6. Le Borgne, J, Paineau, J, Hamy, A, et al. Interruption of the inferior vena cava with azygos termination associated with congenital absence of portal vein. Surg Radiol Anat 2000; 22: 197–202.CrossRefGoogle ScholarPubMed
7. Bartram, U, Fischer, G, Kramer, H. Congenitally interrupted inferior vena cava without other features of the heterotaxy syndrome: report of five cases and characterization of a rare entity. Pediatr Dev Pathol 2008; 11: 266–273.Google Scholar
8. Doraiswamy, V, Sivakumar, K. Impact of Abernathy malformation on pulmonary circulatory hemodynamics in a univentricular heart. Ann Pediatr Cardiol 2017; 10: 90–91.CrossRefGoogle Scholar
9. Bernard, O, Franchi-Abella, S, Branchereau, S, et al. Congenital portosystemic shunts in children: recognition, evaluation and management. Semin Liver Dis 2012; 32: 273–287.Google Scholar
10. Kuo, MD, Miller, FJ, Lavine, JE, et al. Exploiting phenotypic plasticity for the treatment of hepatopulmonary shunting in Abernethy malformation. J Vasc Interv Radiol 2010; 21: 917–922.Google Scholar

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