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.

Download full-text


Available from: Karen Krok
  • Source
    • "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]. "
    [Show abstract] [Hide abstract]
    ABSTRACT: Objectives: Increased arterial stiffness is a risk factor of atherosclerosis and cardio-vascular complications. The aim of the study was to determine whether peripheral vascular function might be an early marker of impaired health status in patients with a single ventricle after Fontan procedure.Methods and results: Twenty five consecutive adults (11 women and 14 men) aged 24.7 +/- 6.2 years after the Fontan procedure and 25 sex, age and BMI match healthy volunteers underwent physical examination, blood analysis, transthoracic echocardiography and noninvasive assessment of aortic stiffness. Augmented pressure and Augmentation Index (AI) were both significantly elevated in Fontan when compared to the controls (6,08 +/- 0,7 vs. 2,0 +/- 3,7; p = 0.002 and 17,01 +/- 3,3 vs. 6,05 +/- 11; p < 0.001, respectively). There were no differences in pulse wave velocity (PWV), Mean blood pressure (BP), brachial pulse pressure (PP), central: systolic BP, diastolic BP and PP. In Fontan group we find negative correlation between PWV and SatO2 (r = -0.68; p = 0.04) and positive correlation with WBC (0.72; p = 0.72; p = 0.013), INR (0.81; p = 0.008), TNFalpha (r = 0.45; p = 0.04), and postoperative time (r = 0.77; p = 0.02). AI correlates positively only with age at surgery (r = 0.45; p = 0.04). Bilirubin level correlates positively with brachial PP (r = 0.71; p = 0.02) and central PP (r = 0.68; p = 0.03).The multivariate model showed that SatO2 (beta = -0.44, p = 0.04) was the only independent predictor of PWV (R2 = 0.32, p = 0.03). Adult Fontan patients have an increased arterial stiffness assessed by a noninvasive technique. Low arterial oxygen saturation postoperative time, age at surgery, white blood cells, TNFalpha and bilirubin level are associated with arterial stiffening in these patients. The combination of blood parameters of the hepatic function and noninvasive measurements of arterial stiffness could be helpful in comprehensive care of patients with Fontan circulation.
    Full-text · Article · Apr 2014 · Cardiovascular Ultrasound
  • Source
    [Show abstract] [Hide abstract]
    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.
    Full-text · Article · Jun 2013
  • Source
    [Show abstract] [Hide abstract]
    ABSTRACT: This Almanac highlights recent papers on congenital heart disease in the major cardiac journals. Over 100 articles are cited. Subheadings are used to group relevant papers and allow readers to focus on their areas of interest, but are not meant to be comprehensive for all aspects of congenital cardiac disease.
    Preview · Article · Sep 2012 · Hellenic journal of cardiology: HJC = Hellēnikē kardiologikē epitheōrēsē
Show more