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End-organ consequences of the Fontan operation: liver fibrosis, protein-losing enteropathy and plastic bronchitis

Published online by Cambridge University Press:  09 January 2014

Jack Rychik ,
David Goldberg ,
Elizabeth Rand ,
Edisio Semeao ,
Pierre Russo ,
Yoav Dori  and
Kathryn Dodds
Jack Rychik*
Affiliation:
Division of Cardiology, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania, United States of America Department of Pediatrics, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania, United States of America
David Goldberg
Affiliation:
Division of Cardiology, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania, United States of America Department of Pediatrics, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania, United States of America
Elizabeth Rand
Affiliation:
Division of Gastroenterology, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania, United States of America Department of Pediatrics, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania, United States of America
Edisio Semeao
Affiliation:
Division of Gastroenterology, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania, United States of America Department of Pediatrics, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania, United States of America
Pierre Russo
Affiliation:
Department of Pediatrics, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania, United States of America Department of Pathology, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania, United States of America
Yoav Dori
Affiliation:
Division of Cardiology, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania, United States of America Department of Pathology and Laboratory Medicine at The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, United States of America
Kathryn Dodds
Affiliation:
Division of Cardiology, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania, United States of America
*
Correspondence to: J. Rychik, MD, The Children's Hospital of Philadelphia, 34th Street and Civic Center Boulevard, Philadelphia, PA 19104, United States of America. Tel: 215.590.2192; Fax: 267.426.5082; E-mail: Rychik@email.chop.edu
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Abstract

The Fontan operation, although part of a life-saving surgical strategy, manifests a variety of end-organ complications and unique morbidities that are being recognised with increasing frequency as patients survive into their second and third decades of life and beyond. Liver fibrosis, protein-losing enteropathy and plastic bronchitis are consequences of a complex physiology involving circulatory insufficiency, inflammation and lymphatic derangement. These conditions are manifest in a chronic, indolent state. Management strategies are emerging, which shed some light on the origins of these complications. A better characterisation of the end-organ consequences of the Fontan circulation is necessary, which can then allow for development of specific methods for treatment. Ideally, the goal is to establish systematic strategies that might reduce or eliminate the development of these potentially life-threatening challenges.

Keywords

Fontan operationliver fibrosisprotein-losing enteropathyplastic bronchitis
Type
Original Article
Information
Cardiology in the Young , Volume 23 , Issue 6: HeartWeek 2013 , December 2013 , pp. 831 - 840
Copyright
Copyright © Cambridge University Press 2013 

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