The Precarious State of the Liver After a Fontan Operation: Summary of a Multidisciplinary Symposium

Division of Cardiology, Single Ventricle Survivorship Program, The Cardiac Center at the Children's Hospital of Philadelphia, 34th Street and Civic Center Boulevard, Philadelphia, PA, 19104, USA, .
Pediatric Cardiology (Impact Factor: 1.31). 04/2012; 33(7):1001-12. DOI: 10.1007/s00246-012-0315-7
Source: PubMed


As the cohort of survivors with the single-ventricle type of congenital heart disease grows, it becomes increasingly evident that the state of chronically elevated venous pressure and decreased cardiac output inherent in the Fontan circulation provides the substrate for a progressive decline in functional status. One organ at great risk is the liver. Wedged between two capillary beds, with the pulmonary venous bed downstream, which typically has no pulsatile energy added in the absence of a functional right ventricle, and the splanchnic bed upstream, which may have compromised inflow due to inherent cardiac output restriction characteristic of the Fontan circulation, the liver exists in a precarious state. This review summarizes a consensus view achieved at a multidisciplinary symposium held at The Children's Hospital of Philadelphia in June 2011. The discussion includes current knowledge concerning the hemodynamic foundations of liver problems, the diagnostic tools available, the unique histopathology of the liver after the Fontan operation, and proposed mechanisms for hepatic fibrosis at the cellular level. At the completion of the symposium, a consensus recommendation was made by the authors' group to pursue a new prospective protocol for clinical evaluation of the liver for all patients in our practice 10 years after the Fontan operation.

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    • "Liver is the organ at great risk after the Fontan operation [29]. Hepatic dysfunction caused by volume overload and low cardiac output may result from a coexistence of passive venous congestion of the liver, hypoxia, or concomitant pulmonary disease [30]. "
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    ABSTRACT: The Fontan circulation is used as palliative surgery in the functionally univentricular heart. Due to the advances made in prenatal diagnosis, cardiac surgery, technology, and monitoring in complex congenital heart disease, survival rates have improved significantly. For this group of patients, the physiology and pathology remain challenging and eluding, even more so in the long-term follow-up. In the last decade pulmonary vascular resistance has been shown to be of utmost importance regarding cardiac output both at rest and during exercise. The aim of this article is to review the most recently published data, namely new concepts and our current understanding regarding the physiology; performance during exercise; the value of imaging modalities; and chronic liver pathology in the Fontan circulation.
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