title: Beyond a broken heart: circulatory dysfunction in the failing Fontan.
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.
authors: Division of Cardiology, Emory University School of Medicine, 1365 Clifton Road, NE, Bldg A - Suite 2447, Atlanta, GA, 30322, USA.
authors: Makoto
authors: M
authors: Mori
authors: Alfredo J
authors: AJ
authors: Aguirre
authors: Robert W
authors: RW
authors: Elder
authors: Ali
authors: A
authors: Kashkouli
authors: Alton Brad
authors: AB
authors: Farris
authors: Ryan M
authors: RM
authors: Ford
authors: Wendy M
authors: WM
authors: Book
mesh_terms: D000328:Adult; D018729:Fontan Procedure / Q000009:adverse effects*; D014943:Global Health; D006330:Heart Defects, Congenital / Q000503:physiopathology / Q000601:surgery*; D006352:Heart Ventricles / Q000002:abnormalities / Q000503:physiopathology / Q000601:surgery; D006439:Hemodynamics / Q000502:physiology*; D006801:Humans; D006975:Hypertension, Portal / Q000453:epidemiology / Q000209:etiology / Q000503:physiopathology; D015994:Incidence; D015996:Survival Rate / Q000639:trends; D017211:Treatment Failure