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New concepts: development of a survivorship programme for patients with a functionally univentricular heart

Published online by Cambridge University Press:  13 December 2011

David J. Goldberg ,
Kathryn Dodds  and
Jack Rychik
David J. Goldberg*
Affiliation:
The Cardiac Center, The Children's Hospital of Philadelphia, Pennsylvania, United States of America Department of Pediatrics, The Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, United States of America
Kathryn Dodds
Affiliation:
The Cardiac Center, The Children's Hospital of Philadelphia, Pennsylvania, United States of America
Jack Rychik
Affiliation:
The Cardiac Center, The Children's Hospital of Philadelphia, Pennsylvania, United States of America Department of Pediatrics, The Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, United States of America
*
Correspondence to: Dr D. J. Goldberg, Division of Cardiology, The Children's Hospital of Philadelphia, 34th Street and Civic Center Boulevard, Philadelphia, Pennsylvania 19146, United States of America. Tel: +1 267 426 8143; Fax: +1 267 425 6108; E-mail: goldbergda@email.chop.edu
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Abstract

Children with functionally univentricular hearts are now surviving into their third and fourth decades of life. Although survival alone is a remarkable achievement, a lot must still be done to improve the quality and duration of life after the Fontan operation. Challenges that may be faced by these patients include the impact of the Fontan operation on the liver and the density of bone, protein-losing enteropathy, and plastic bronchitis. Paediatric cardiologists are familiar with the haemodynamic issues inherent in Fontan physiology; however, training in cardiology is often not sufficient to give us a complete understanding of the pathophysiology of the complications or of the options for treatment. Collaboration with other subspecialists including gastroenterologists, endocrinologists, and pulmonologists is essential in order to provide the rigorous and nuanced care that our patients need and deserve. A clinic in which a patient can see multiple subspecialists, and in which the subspecialists, as a group, can discuss each patient, can provide a unique and valuable service for patients with a functionally univentricular heart.

Keywords

Fontansingle ventriclefunctionally univentricular heartfollow-up
Type
Original Article
Information
Cardiology in the Young , Volume 21 , Issue S2: A Holistic Approach to Hypoplastic Left Heart Syndrome and other evolving challenges in Pediatric and Congenital Cardiac Disease , 13 December 2011 , pp. 77 - 79
Copyright
Copyright © Cambridge University Press 2011

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