Fontan-associated liver disease: Implications for heart transplantation

JOURNAL OF HEART AND LUNG TRANSPLANTATION

卷:35

Article

Greenway, Steven C.^1,2,3,4  Crossland, David S.⁵  Hudson, Mark⁶  Martin, Steven R.^1,2  Myers, Robert P.⁷  Prieur, Tim^3,4  Hasan, Asif⁵  Kirk, Richard⁵ 

[1] Univ Calgary, Dept Paediat, Calgary, AB T2N 1N4, Canada

[2] Univ Calgary, Alberta Childrens Hosp Res Inst, Calgary, AB, Canada

[3] Univ Calgary, Dept Cardiac Sci, Calgary, AB, Canada

[4] Univ Calgary, Libin Cardiovasc Inst Alberta, Calgary, AB, Canada

[5] Freeman Rd Hosp, Dept Paediat Cardiol & Cardiothorac Surg, Newcastle Upon Tyne, Tyne & Wear, England

[6] Freeman Rd Hosp, Liver Unit, Newcastle Upon Tyne, Tyne & Wear, England

[7] Univ Calgary, Div Gastroenterol, Dept Med, Liver Unit, Calgary, AB, Canada

关键词: Fontan;    heart transplantation;    liver disease;    combined heart-liver transplantation;    liver fibrosis;   

DOI  :  10.1016/j.healun.2015.10.015

来源: Elsevier

【 摘 要 】

Chronic liver diseases are associated with multiple complications, including cirrhosis, portal hypertension, ascites, synthetic dysfunction and hepatocellular carcinoma, and these processes are increasingly recognized in post-Fontan patients. Fontan-associated liver disease (FALD) can be defined as abnormalities in liver structure and function that result from the Fontan circulation and are not related to another disease process. FALD arises due to chronic congestion of the liver created by the elevated venous pressure and low cardiac output of the Fontan circulation, which may be superimposed on previous liver injury. Pathology studies have generally shown that FALD worsens as time post-Fontan increases, but the prevalence of FALD is not well defined because the majority of Fontan patients, even those with significant hepatic fibrosis, appear to be asymptomatic and biochemical or functional hepatic abnormalities are usually subtle or absent. Alternate non-invasive investigations, derived from the study of other chronic liver diseases, have been tested in small series of pediatric and adult Fontan patients, but they have been confounded by congestion and do not correlate well with liver biopsy findings. Liver disease can complicate Fontan circulatory failure and may even be significant enough to be considered a contraindication to heart transplantation or require combined heart liver transplantation. The search for the optimal management strategy continues in the setting of increasing numbers of Fontan patients surviving to adulthood and being referred for heart transplantation. Thus, in this review we attempt to define the scope and significance of FALD and address transplant-related assessment and management of this challenging disorder. (C) 2016 International Society for Heart and Lung Transplantation. All rights reserved.

【 授权许可】

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