Arterialised hepatic nodules in the Fontan circulation: Hepatico-cardiac interactions
ABSTRACT Hypervascular nodules occur commonly when there is hepatic venous outlet obstruction. Their nature and determinants in the Fontan circulation is poorly understood. We reviewed the records of 27 consecutive Fontan patients who had computerized tomography scan (CT) over a 4 year period for arterialised nodules and alterations in hepatic flow patterns during contrast enhanced CT scans and related these findings to cardiac characteristics. Mean patient age was 24 ± 5.8 years, (range 16.7-39.8) and mean Fontan duration was 16.8 ± 4.8 years (range 7.3-28.7). Twenty-two patients demonstrated a reticular pattern of enhancement, 4 a zonal pattern and only 1 demonstrated normal enhancement pattern. Seven (26%) patients had a median of 4 (range 1-22) arterialised nodules, mean size 1.8 cm (range 0.5 to 3.2 cm). All nodules were located in the liver periphery, their outer aspect lying within 2 cm of the liver margin. Patients with nodules had higher mean RA pressures (18 mmHg ± 5.6 vs. 13 mmHg ± 4, p=0.025), whereas their mixed venous saturation and aortic saturation was not significantly different (70% ± 11 vs. 67% ± 9 and 92% ± 10 vs. 94% ± 4, p>0.05). Post-mortem histology suggests focal nodular hyperplasia is the underlying pathology. Conclusions
Abnormalities of hepatic blood flow and the presence of arterialised nodules are common in the failing Fontan circulation. They occur especially when central venous pressures are high, and very likely indicate arterialisation of hepatic blood flow and reciprocal portal venous deprivation. The underlying pathology is most likely focal nodular hyperplasia.
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- "Recently, the presence of arterialized nodules in the Fontan liver were described . These occur with relatively high frequency. "
ABSTRACT: 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.Pediatric Cardiology 04/2012; 33(7):1001-12. DOI:10.1007/s00246-012-0315-7 · 1.31 Impact Factor
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ABSTRACT: The purpose of this study was to evaluate whether ferumoxides-enhanced MR imaging of focal hepatic lesions provides distinctive signal intensity and lesion-to-liver contrast changes for benign and malignant lesions, helping to further characterize and differentiate these lesions. Data analysis was performed on 70 patients, with previously identified focal hepatic lesions, who underwent MR imaging of the liver before and after IV administration of ferumoxides (10 micromol Fe/kg). Lesions analyzed with pathologically proven diagnoses included metastases (n = 40), hepatocellular carcinoma (n = 11), cholangiocarcinoma (n = 6), hemangioma (n = 4), focal nodular hyperplasia (n = 6), and hepatocellular adenoma (n = 3). Response variables measured and statistically compared included the percentage of signal-intensity change and lesion-to-liver contrast. Focal nodular hyperplasia showed significant signal intensity loss on ferumoxides-enhanced T2-weighted images (mean, -43%+/-6.7%, p < 0.01). All other lesion groups showed no statistically significant change in signal intensity on ferumoxides-enhanced T2-weighted images, although signal intensity loss was seen in some individual hepatocellular adenomas (mean, -6.6%+/-24.0%) and hepatocellular carcinomas (mean, -3.3%+/-10.3%). All lesions, with the exception of hepatocellular carcinoma, had a marked increase in lesion-to-liver contrast on ferumoxides-enhanced T2-weighted images, which was statistically significant for metastases and hemangioma (p < 0.02). Focal nodular hyperplasia shows significant decrease in signal intensity on ferumoxides-enhanced T2-weighted images, which may aid in the differentiation of focal nodular hyperplasia from other focal hepatic lesions. Other lesions, namely, hepatocellular adenoma and carcinoma, can have reticuloendothelial uptake, but usually to a lesser degree than that of focal nodular hyperplasia.American Journal of Roentgenology 08/2000; 175(1):159-63. DOI:10.2214/ajr.175.1.1750159 · 2.73 Impact Factor
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ABSTRACT: The Fontan procedure has undergone many modifications since first being performed on a patient with tricuspid valve atresia in 1968. It is now the procedure of choice for individuals born with single-ventricle physiology or for those in whom a biventricular repair is not feasible. Forty years of experience with the Fontan procedure have gradually revealed the shortfalls of such a circulatory arrangement. Sequelae related to the underlying congenital anomaly or to the altered physiology of passive, nonpulsatile flow through the pulmonary arterial bed can result in failure of the Fontan circulation over time. Liver abnormalities including abnormalities in the clotting cascade have been well documented in Fontan patients. The clinical significance of these findings, however, has remained poorly understood. As Fontan survivors have increased in age and number, we have begun to better recognize subclinical hepatic dysfunction and the contribution of liver disease to adverse outcomes in this population. The purpose of this review is to summarize the existing data pertaining to liver disease in the Fontan population and to identify some questions that have yet to be answered.Congenital Heart Disease 03/2011; 6(3):190-201. DOI:10.1111/j.1747-0803.2011.00504.x · 1.08 Impact Factor