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Pediatric Cardiology
Published in: Pediatric Cardiology 4/2014

01-04-2014 | Review Article

Beyond a Broken Heart: Circulatory Dysfunction in the Failing Fontan

Authors: Makoto Mori, Alfredo J. Aguirre, Robert W. Elder, Ali Kashkouli, Alton Brad Farris, Ryan M. Ford, Wendy M. Book

Published in: Pediatric Cardiology | Issue 4/2014

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Abstract

The role of ventricular dysfunction in late morbidity and mortality of univentricular hearts has been described previously. However, a significant proportion of adult Fontan patients who die or require heart transplantation do so with preserved ventricular function. The clinical deterioration in patients who have undergone Fontan palliation requires a broader view of circulatory dysfunction, one that takes into account the complex interaction of regulatory systems affecting hepatic, renal, and pulmonary blood flow, in addition to cardiac function. This review focuses primarily on the pathophysiology of multiple organ involvement in this circulatory dysfunction, with particular focus on the consequences of hepatic dysfunction and portal hypertension. The authors discuss hepatic perfusion, both in health and disease, and review the current understanding of liver histopathology and liver disease in adult Fontan patients and similar clinicopathologic states. They compare and contrast features of postsinusoidal portal hypertension with more typical adult cirrhotic disease. Finally, they delineate the related effects of portal hypertensive physiology on the systemic and pulmonary vasculature, the kidney, and the heart itself and discuss how these changes affect the care of the adult Fontan patient.
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Metadata
Title
Beyond a Broken Heart: Circulatory Dysfunction in the Failing Fontan
Authors
Makoto Mori
Alfredo J. Aguirre
Robert W. Elder
Ali Kashkouli
Alton Brad Farris
Ryan M. Ford
Wendy M. Book
Publication date
01-04-2014
Publisher
Springer US
Published in
Pediatric Cardiology / Issue 4/2014
Print ISSN: 0172-0643
Electronic ISSN: 1432-1971
DOI
https://doi.org/10.1007/s00246-014-0881-y

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