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Rare problems associated with the Fontan circulation

Published online by Cambridge University Press:  01 December 2010

David J. Goldberg ,
Kathryn Dodds  and
Jack Rychik
David J. Goldberg*
Affiliation:
Division of Cardiology, The Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania, United States of America
Kathryn Dodds
Affiliation:
Division of Cardiology, The Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania, United States of America
Jack Rychik
Affiliation:
Division of Cardiology, The Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania, United States of America
*
Correspondence to: D. J. Goldberg, MD, Division of Cardiology, The Children’s Hospital of Philadelphia, 34th and Civic Center Boulevard, Philadelphia, Pennsylvania 19104, United States of America. Tel: 267 426 3058; Fax: 215 590 3267; E-mail: goldbergda@email.chop.edu
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Abstract

The Fontan operation, originally described for the surgical management of tricuspid atresia, is now the final surgery in the strategy of staged palliation for a number of different forms of congenital cardiac disease with a functionally univentricular heart. Despite the improved technical outcomes of the Fontan operation, staged palliation does not recreate a normal physiology. Without a pumping chamber delivering blood to the lungs, the cardiovascular system is less efficient; cardiac output is generally diminished, and the systemic venous pressure is increased. As a result, patients with “Fontan physiology” may face a number of rare but potentially life-threatening complications including hepatic dysfunction, abnormalities of coagulation, protein-losing enteropathy, and plastic bronchitis. Despite the staged palliation resulting in remarkable survival, the possible complications for this group of patients are complex, involve multiple organ systems, and can be life threatening. Identifying the mechanisms associated with each of the rare complications, and developing strategies to treat them, requires the work of many people at many institutions. Continued collaboration between sub-specialists and between institutions will be required to optimise the care for this group of survivors with functionally univentricular hearts.

Keywords

Congenital heart diseasepaediatric cardiac diseasehepatic dysfunctionabnormalities of coagulationprotein-losing enteropathyplastic bronchitissingle ventriclefunctionally univentricular heart
Type
Original Article
Information
Cardiology in the Young , Volume 20 , Issue S3: Rare and Challenging Congenital Cardiac Lesions: an Interdisciplinary Approach , December 2010 , pp. 113 - 119
Copyright
Copyright © Cambridge University Press 2010

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