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Beyond a Broken Heart: Circulatory Dysfunction in the Failing Fontan

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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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Acknowledgments

We thank Matthew Certain, MD, and Nicholas Wozmak, MD, for their contributions to the artwork.

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Authors and Affiliations

  1. Division of Cardiology, Emory University School of Medicine, 1365 Clifton Road, NE, Bldg A – Suite 2447, Atlanta, GA, 30322, USA

    Makoto Mori, Alfredo J. Aguirre & Wendy M. Book

  2. Section of Cardiology, Yale University School of Medicine, New Haven, CT, USA

    Robert W. Elder

  3. Division of Nephrology, Emory University School of Medicine, Atlanta, GA, USA

    Ali Kashkouli

  4. Department of Pathology, Emory University School of Medicine, Atlanta, GA, USA

    Alton Brad Farris

  5. Transplant Hepatology, Emory University School of Medicine, Atlanta, GA, USA

    Ryan M. Ford

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  1. Makoto Mori
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Correspondence to Wendy M. Book.

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Mori, M., Aguirre, A.J., Elder, R.W. et al. Beyond a Broken Heart: Circulatory Dysfunction in the Failing Fontan. Pediatr Cardiol 35, 569–579 (2014). https://doi.org/10.1007/s00246-014-0881-y

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