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Persistent primitive hepatic venous plexus with Scimitar syndrome: description of a case and review of the literature

Published online by Cambridge University Press:  24 September 2014

M. Santiago Restrepo ,
Osamah Aldoss  and
Benton Ng
M. Santiago Restrepo
Affiliation:
Department of Pediatrics, Division of Pediatric Cardiology, University of Iowa Children’s Hospital, Carver College of Medicine, Iowa City, Iowa, United States of America
Osamah Aldoss
Affiliation:
Department of Pediatrics, Division of Pediatric Cardiology, University of Iowa Children’s Hospital, Carver College of Medicine, Iowa City, Iowa, United States of America
Benton Ng*
Affiliation:
Department of Pediatrics, Division of Pediatric Cardiology, University of Iowa Children’s Hospital, Carver College of Medicine, Iowa City, Iowa, United States of America
*
Correspondence to: Dr B. Ng, MD, Clinical Assistant Professor, Department of Pediatrics, Division of Pediatric Cardiology, University of Iowa Children’s Hospital, Carver College of Medicine, 200 Hawkins Drive, 2801 JPP Iowa City, IA 52242, United States of America. E-mail: Benton-ng@uiowa.edu
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Abstract

Persistent primitive hepatic venous plexus is an anomaly of the systemic venous return characterised by postnatal persistence of the foetal intrahepatic venous drainage. Scimitar syndrome is a condition that consists of partial anomalous pulmonary venous return of the right pulmonary venous drainage into the systemic veins, associated with pulmonary artery hypoplasia with the underdeveloped right lung, pulmonary sequestration, and cardiac malposition. Both conditions are rare and together have been rarely described in the literature. We report the first case of this combination of lesions imaged by cardiac magnetic resonance imaging with a three-dimensional reconstruction and reviewed the literature to characterise this uncommon combination.

Keywords

Persistent primitive hepatic venous plexuspartial anomalous pulmonary venous returnScimitar syndrome
Type
Brief Reports
Information
Cardiology in the Young , Volume 25 , Issue 5 , June 2015 , pp. 1009 - 1011
Copyright
© Cambridge University Press 2014 

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