Portal venule central vein distension. Perisinusoidal fibrosis - progresses to centrilobular fibrosis and then diffuse fibrosis. Dilation of sinusoids in all zone III areas - key feature. Cardiac congestive hepatopathy, wherein outflow obstruction occurs secondary to backflow from a failing heart, and Budd Chiari syndrome, wherein obstruction of outflow occurs secondary to obstruction, usually thrombotic, have overlapping features, including sinusoidal dilation with emphasis upon the central vein, space of Disse erythrocytes, centrilobular inflammation, hemorrhage, bile ductules, and hemosiderin, and portal inflammation, fibrosis and bile ductular reaction. In end stage liver, fibrosis for cardiac hepatopathy comprises stellate centrizonal fibrous with haphazard spread, as opposed to the more frequent nodular cirrhosis in Budd-Chiari.
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Pathology Abstract Chronic right heart failure predisposes to hepatic passive congestion and centrizonal necrosis that may lead to hepatic fibrosis cardiac sclerosis. Although there have been several studies on the histologic features of congestive hepatopathy, there is no available grading system. In this study we developed a novel grading system for congestive hepatic fibrosis. Liver biopsies were examined in patients with chronic heart failure of various etiologies including congenital heart disease, idiopathic cardiomyopathy, ischemic heart disease, and valvular heart disease.
After exclusion, 42 cases were included in the study. Other histopathologic features include sinusoidal dilatation and centrizonal hepatocyte atrophy. In summary, although sinusoidal dilatation and centrizonal fibrosis are the hallmarks of hepatic passive congestion, the presence of portal fibrosis is suggestive of more advanced disease, as it correlates with more severe impairment of right heart function, regardless of the etiologies of right heart failure.
Congestive hepatic fibrosis score is a useful indicator of clinical severity. Download PDF Main Liver pathology in patients with congestive heart failure has been recognized for decades.
Several studies have since confirmed and further characterized these histologic findings in post-mortem liver specimens from patients with various cardiac diseases or shock, including centrilobular congestion, with or without necrosis, inflammation, sclerosis, and regenerative hyperplasia. In one such analysis, Myers et al 5 reported that elevated right atrial pressure and hepatic venous pressure were associated with the presence of centrilobular necrosis, inflammation, and sinusoidal dilatation, but surprisingly, not with hepatic fibrosis.
Because the latter observations raise concerns about the reproducibility of histopathologic observations in cardiac hepatopathy, we believe that ongoing investigations of this condition, including meta-analyses of existing studies, would be aided by a robust histologic scoring system.
In the current study, we propose a simplified histologic scoring system for congestive hepatopathy—what we will refer to as the congestive hepatic fibrosis score—that may serve as a clinically useful indicator of the severity of congestive hepatopathy.
Our findings demonstrate that the congestive hepatic fibrosis score correlates well with echocardiographic and hemodynamic parameters. A total of 54 patients met the inclusion criteria. There were 42 patients available for final analysis. Clinical, Echocardiographic, and Cardiac Catheterization Data All clinical, laboratory, echocardiographic, and cardiac catheterization data analyzed in this study were obtained from electronic medical records of our institution.
Echocardiographic data included left ventricular ejection fraction, echocardiographic diagnosis, estimated right atrial pressure, and the degree of right atrial and right ventricular dilatation. For cardiac catheterization, we included the mean right atrial pressure data from right heart catheterization studies performed within 4 months of the echocardiography.
Congestive Hepatic Fibrosis Score and Histopathologic Examination We designed a simplified congestive hepatic fibrosis score based on prior literature review 2 , 3 , 5 and 9 index autopsy cases — that met the aforementioned inclusion and exclusion criteria. Based on the pattern of fibrosis, scores of 0, 1, 2A, 2B, 3, and 4 may be assigned as follows: score 0, no fibrosis; score 1, central fibrosis; score 2A, central zone and portal fibrosis, with accentuation of fibrosis in the central zone; score 2B, moderate portal fibrosis and central zone fibrosis, with accentuation of fibrosis in the portal zone; score 3, bridging fibrosis; score 4, cirrhosis see also Table 1 and Figure 1.
Figure 2 presents examples of the congestive hepatic fibrosis score using a Masson trichrome stain. Table 1 Proposed scoring system for congestive hepatic fibrosis Full size table Figure 1 Diagram of the proposed congestive hepatic fibrosis score: score 0, no fibrosis; score 1, central zone fibrosis only; score 2A, central zone and portal fibrosis, with accentuation of fibrosis in the central zone; score 2B, moderate portal fibrosis and central zone fibrosis, with accentuation of fibrosis in the portal zone; score 3, bridging fibrosis; and score 4, cirrhosis.
Full size image Figure 2 Examples of the congestive hepatic fibrosis score using Masson trichrome stain: a Centrizonal fibrosis without portal fibrosis score 1 in a background of sinusoidal dilatation, b centrizonal accentuated fibrosis with mild portal fibrosis score 2A , c portal accentuated fibrosis and central zone fibrosis score 2B and sinusoidal dilatation, and d bridging fibrosis score 3.
Full size image For histopathologic data collection, archived hematoxylin and eosin and Masson trichrome-stained sections of liver biopsy samples from each patient were evaluated.
The adequacy of the biopsy specimen is defined as biopsy length of at least 1. All cases that were analyzed meet these criteria. Biopsies were evaluated for sinusoidal dilatation and hepatocyte atrophy, and were assigned a consensus congestive hepatic fibrosis score by two pathologists MMY and PES who were blinded to clinical and outcome data. Nonparametric test for trend was applied to investigate the relationship between the congestive hepatic fibrosis score and the right atrial pressure. Results Liver biopsies from 42 patients with heart diseases met the inclusion and exclusion criteria and were assigned a congestive hepatic fibrosis score.
The demographic data and heart disease status of our study population stratified by the congestive hepatic fibrosis score are summarized in Table 2. The mean age was There was no significant difference in age or gender across different congestive hepatic fibrosis score groups.
Similarly, the indication for liver biopsy preheart transplant evaluation, abnormal liver tests, and abnormal liver imaging suspected cirrhosis by ultrasound, CT, or MRI was not significantly different across congestive hepatic fibrosis scores. The left ventricular systolic function as indicated by LV ejection fraction overall mean LV ejection fraction of Table 2 The demographic data and heart disease status Full size table Histologic Findings and Congestive Hepatic Fibrosis Score Overall, tissue preservation was adequate in all biopsies for histologic evaluation.
The most striking finding was the frequency of sinusoidal dilatation and centrizonal hepatocyte atrophy in the study population Figure 3.
Figure 3 Moderate sinusoidal dilatation and hepatocyte atrophy in a patient with elevated right atrial pressure but no significant fibrosis congestive hepatic fibrosis score 0. Full size image The correlation of congestive hepatic fibrosis score with other histopathologic findings is shown in Table 3.
Table 3 Congestive hepatic fibrosis score and frequencies of histologic findings Full size table Figure 4 illustrates the correlation between congestive hepatic fibrosis score and the severity of right heart dysfunction. Furthermore, patients with portal fibrosis scores 2—3 have significantly higher right atrial pressure than those with no fibrosis score 0 or those with central fibrosis only score 1, Figure 4b , suggesting that portal fibrosis is an indicator of more advanced disease.
Because chronic right heart pressure or volume overload induces chamber dilatation, we assessed the relationship between the congestive hepatic fibrosis score and the severity of right atrial and ventricular dilation.
As shown in Figures 5a and b , increasing congestive hepatic fibrosis score is significantly associated with more severe right atrial and right ventricular dilatation. Full size image Figure 5 Proportions of various degrees of a right atrial dilatation and b right ventricular dilatation stratified by congestive hepatic fibrosis score.
Full size image As right heart catheterization is the gold standard for direct measurement of right atrial pressure, we compared the mean right atrial pressure obtained by right heart catheterization and pressure estimation by echocardiography using data available from 18 patients.
There was no significant difference between RA pressure by right heart catheter measurement and RA pressure estimation by echocardiography, with a mean difference of 0. Discussion In this study we introduce a histologic fibrosis grading system for patients with congestive hepatopathy. We observe that sinusoidal dilatation, centrilobular hepatocyte atrophy, centrilobular fibrosis, and portal fibrosis correlate with right atrial pressure, whereas higher congestive hepatic fibrosis scores are associated with right atrial and right ventricular dilation.
Congestive hepatopathy is a common condition associated with chronic elevation of right heart pressure, commonly seen in end-stage heart failure or after Fontan operation.
In patients with severe heart failure, particularly right heart failure, hepatic injury results from chronic congestion, with or without superimposed decline in hepatic arterial perfusion. In this study, we have proposed a simple scoring system—the congestive hepatic fibrosis score—and have demonstrated that this score correlates well with echocardiography-derived right atrial pressure as well as both right atrial and right ventricular dilatation.
Furthermore, the presence of portal fibrosis is associated with significantly higher right atrial pressure when compared with those without portal fibrosis Figure 4b. As shown in Figure 1 , the congestive hepatic fibrosis score is simple and straightforward and the above findings support the validity of this score as a useful indicator of the severity of congestive hepatopathy. The application of the congestive hepatic fibrosis score in this study was also a test of interobserver agreement.
The initial independent evaluation of test cases by two pathologists assessed the degree of agreement in this setting, After review of representative images including those used in Figures 1 and 2 and a discussion of scoring criteria in the context of representative histologic material, the interobserver agreement reached This emphasizes the role of appropriate training before employing this or any other histologic scoring system for prospective or retrospective studies.
Several classical autopsy studies have reported common histologic findings in post-mortem liver specimens from patients with cardiac diseases, including centrilobular congestion, centrilobular necrosis, inflammation, sclerosis, and regenerative hyperplasia. A recent study by Myers et al 5 reported the clinical, hemodynamic, and histological features of liver biopsies from 83 patients with acute, acute on chronic, or chronic heart failure. They reported an association between free hepatic venous pressure and the presence of centrilobular necrosis, inflammation, periportal necrosis, and stainable hepatic iron, but not centrilobular fibrosis.
Sinusoidal dilatation was also associated with higher right atrial and free hepatic venous pressures. In a large autopsy series of subjects with cardiac dysfunction, Arcidi et al 3 reported that hepatic centrilobular necrosis often occurred in the setting of shock, whereas elevated central venous pressure often manifested as centrilobular congestion.
In patients with complex congenital heart disease results in a single functional ventricle including hypoplastic left or right heart syndrome, and tricuspid or pulmonary atresia , the Fontan procedure bypasses the right ventricle via either a right atrium or central venous to pulmonary artery anastomosis. Given the growing number of hepatocellular carcinoma cases in patients with congestive hepatopathy, a good scoring system is necessary for reporting consistency.
Furthermore, congestive hepatic fibrosis score might be helpful to identify high-risk patients who need closer follow-up, screening for hepatocellular carcinoma, and consideration of heart versus heart and liver dual organ transplantation. Congestive hepatic fibrosis increases the risk of postsurgical mortality in heart transplant recipients. Emerging evidence suggests that hepatic fibrosis in chronic liver disease may regress following removal of these insults.
Previous reports on pathology of congestive hepatopathy have emphasized rheumatic heart disease and ischemic cardiomyopathy. It is important to note that neither the various etiologies of heart failure nor the left ventricular ejection fraction seem to correlate with the congestive hepatic fibrosis score, although this study may not have adequate sample size to determine whether different etiologies of heart failure correlate with the congestive hepatic fibrosis score.
Nevertheless, our histopathologic observation demonstrates that sinusoidal dilatation, centrilobular hepatocyte atrophy, centrilobular fibrosis, and portal fibrosis more likely result from increased right atrial pressure because of the various causes of right heart failure.
A limitation of this study is the lack of disease-free survival analysis, an element that limits the utility of the congestive hepatic fibrosis score for prognosis. This was not performed for two reasons: first, the progression of congestive hepatopathy to advanced cardiac cirrhosis is relatively rare, despite frequent manifestation of centilobular fibrosis, 2 , 5 as discussed above; second, many of the patients in our study died of end-stage heart failure before the development of cardiac cirrhosis.
With the strong confounding effect of heart failure and the lack of proper end point, the prognostic significance of the congestive hepatic fibrosis thus remains unknown.
Despite this limitation, our data support the utilization of congestive hepatic fibrosis score as an indicator of disease severity. In conclusion, our study has introduced a novel and simple histologic grading of hepatic fibrosis based on trichrome staining. This congestive hepatic fibrosis score is well correlated with the hemodynamic and echocardiographic parameters that reflect the severity of heart disease, supporting its utility as a valuable indicator of disease severity.
References 1 Sherlock S. The liver in heart failure; relation of anatomical, functional, and circulatory changes. Br Heart J ;—
CONGESTIVE HEPATOPATHY PDF
Vudozilkree Unsourced or poorly sourced material may be challenged and removed. This review will summarize the pathophysiologic mechanisms of congestive hepatopathy and provide both description and examples of its multimodality imaging findings. True nutmeg liver is usually secondary to left-sided heart failure cojgestive congestive right heart failure, so treatment options are limited. Gastrointestinal catarrh is usually present, and vomiting of blood may occur. From Wikipedia, the free encyclopedia.
Pathology Abstract Chronic right heart failure predisposes to hepatic passive congestion and centrizonal necrosis that may lead to hepatic fibrosis cardiac sclerosis. Although there have been several studies on the histologic features of congestive hepatopathy, there is no available grading system. In this study we developed a novel grading system for congestive hepatic fibrosis. Liver biopsies were examined in patients with chronic heart failure of various etiologies including congenital heart disease, idiopathic cardiomyopathy, ischemic heart disease, and valvular heart disease. After exclusion, 42 cases were included in the study.
In addition to the heart or lung symptoms, there will be a sense of fullness and tenderness in the right hypochondriac region. Gastrointestinal catarrh is usually present, and vomiting of blood may occur. There is usually more or less jaundice. Owing to portal obstruction, ascites occurs, followed later by generalised oedema. The stools are light or clay-colored, and the urine is colored by bile.