Help
Subscribe


All of GastroHep is now free access! - Click here to register Read For FREE - Our full range of review articles
GastroHep.com - the global online resource for all aspects of gastroenterology, hepatology and endoscopy
GastroHep.com - the global online resource for all aspects of gastroenterology, hepatology and endoscopy Profile of Guido Tytgat Profile of Pete Peterson Profile of Peter Cotton Profile of Roy Pounder

Home

News
Journals
Review Articles
Slide Atlas
Video Clips
Online Books
Advanced Digestive Endoscopy
Classical Cases  
Conference Diary
PubMed
International GH Links
USA GH Links
National GH Links
National GI Societies
Other Useful Links




Emails on Gastroenterology and Hepatology
the National AIDS Treatment Advocacy Project
Visit the gastroenterology section of the EUMS

Classical Case Studies

Click a heading in the right-hand column to see the selected articles in that subject area

See any comments for this case

SK Patel, S Barton and A Quaglia An unusual case of cirrhosis
SK Patel, S Barton and A Quaglia, 13 February 2013

 

Summary

Cardiac cirrhosis is an increasingly rare disorder. In this case a 74-year-old man developed cardiac cirrhosis secondary to constrictive pericarditis. In 2006, a liver biopsy showed a pattern of fibrosis that is observed following long-standing venous outflow block.

Previous research has demonstrated that patients with cardiac cirrhosis present with symptoms of liver congestion – hepatomegaly, splenomegaly, esophageal varices and ascites. However, in this case the patient had very few typical signs and symptoms. This demonstrates the difficulty of diagnosing cardiac cirrhosis clinically and the importance of histology plays in providing a definitive diagnosis.

Introduction

Cardiac cirrhosis is an increasingly rare disorder that develops after long-standing right-sided congestive heart failure most commonly secondary to constrictive pericarditis or tricuspid insufficiency (Brown et al. 1997).

The pathogenesis is generally thought to be due to a reaction of the hepatic stroma to hypoxia, pressure, or hepatocellular necrosis (Kotelsky et al. 1930).

Other etiologies for cardiac failure leading to cardiac cirrhosis include ischemic heart disease, ischemic cardiomyopathy, valvular heart disease, restrictive lung disease and pericardial disease.

The morphological changes seen may vary from focal lesions in the liver to disperse changes that affect the complete liver, thus resulting in the development of complete cirrhosis (Naschitz et al. 2000).

In this case we describe a case of this rare disease.

 

Case Report

History

A 74-year-old man developed cardiac cirrhosis secondary to constrictive pericarditis. His constrictive pericarditis developed at the age of 14 secondary to tuberculosis.

Fig. 1  Chest x-ray demonstrating a calcified pericardial sac and a raised right hemi diaphragm with the colon beneath.

A 63-year-old man presented in 2000 in clinic. He was experiencing episodic abdominal bloating and a sensation of ‘fullness’ in his neck veins which eased after frequent urination. These symptoms had worsened since his recent diagnosis of atrial fibrillation.

Past history included tuberculosis age 14 which required a pericardial resection and a phrenic crush as part of his treatment (Figure 1).  He was diagnosed with biopsy-proven coeliac disease in 2000.  In addition, his past medical history included type 2 diabetes, controlled by oral hypoglycaemics, paroxysmal atrial fibrillation, duodenal ulcer, gastric angiodysplasia, diabetic retinopathy and cataract.

He had a past history of significant alcohol consumption but had been abstinent for 2 years.

Investigation

Blood tests showed a mildly thrombocytopenic picture. Liver function tests (LFTs) were mildly abnormal (alkaline phosphatase 145, gamma GT 117, transaminases normal). Serology testing showed that he was anti-nuclear antibody, smooth muscle antibody and anti-mitochondrial antibody negative, Hepatitis B surface antigen (HbsAg) ELISA negative and Hepatitis C antibody negative. Serum ferritin and alpha 1 antitrypsin level were in the normal range.

Liver biopsy in 2000 showed normal architecture.  Most of the portal tracts were normal but showed mild inflammatory cell infiltrates consisting of lymphocytes and neutrophils. There was an associated expansion with bile duct damage and bile duct proliferation. The lobules showed a grade I siderosis consistent with a history of past alcoholism. There was no distinct cholestasis. The conclusion was non-specific portal inflammation.

He continued to be monitored in gastroenterology out-patients.

Because of worsening LFT’s, Magnetic Resonance Cholangiopancreatography (MRCP) in 2003 showed an incidental finding of Chiladiti’s syndrome with the hepatic flexure of the colon high under the right hemi diaphragm normal (Figure 1). The pancreatic duct was uniformly a little prominent at around 5 mm indicating previous chronic pancreatitis. No other abnormalities were seen.

Repeat MRCP in 2006 showed that the liver had a lobulated appearance in keeping with cirrhosis, the bile duct was dilated at 8 mm but no filling defect was noted and the pancreatic duct appeared mildly dilated (approximately 4 mm).

 

Fig. 2  A liver biopsy histopathology slide demonstrating classical features of cardiac cirrhosis.

In 2006 a further liver biopsy was taken (Figure 2) and showed marked sinusoidal dilatation and fibrous septa sparing portal tracts and with a distribution suggestive of central-central bridging. This pattern of fibrosis is observed following long standing venous outflow block as can be observed in patients with blockage of the hepatic veins, vena cava or right cardiac failure.

 

Outcome

In 2009 the patient started to show signs of hepatic encephalopathy with serum ammonia of 150. For the next few years he had frequent admissions with increasing confusion and jaundice which he was treated for hepatic encephalopathy. In 2012 he developed extra pyramidal symptoms which were thought to be likely due to his portal encephalopathy. The patient died shortly after.

 

Discussion

Previous research has demonstrated that patients with cardiac cirrhosis present with symptoms of liver congestion and hepatomegaly in approximately 90%, and splenomegaly in approximately 20%, of patients. Esophageal varices are seen in approximately 10% of patients. Ascites develops in approximately 30% of patients. Ascitic protein concentration is typically above 25 g/l and serum/ascitic albumin gradient is above 11 g/l, testifying to the transudative nature of the peritoneal effusion due to the presence of portal hypertension (Dunn et al. 1974).

In view of the clinical background of constrictive pericarditis the histological findings in the most recent biopsy were consistent with cardiac cirrhosis (Figure 2), however our patient had very few typical signs and symptoms. This demonstrates the difficulty of diagnosing cardiac cirrhosis clinically, previous research has suggested that it should be based upon the triad of right-sided heart failure, hepatomegaly and ascites with a high protein content and high serum ascites albumin gradient, however it should be noted that histology plays an important role in the definitive diagnosis.

The histological pattern of injury develops as a consequence of outflow blockage and affects initially the part of the lobule around the hepatic venule, with sinusoidal dilatation and congestion, haemorrhage and atrophy of hepatocyte plates, with sparing of the portal tracts. When fibrosis develops, it tends to affect the centrilobular regions which start to link up generating a pattern of central-central bridging giving the impression of reverse lobulation in which residual portal tracts are placed in the centre, and the central-central bridges at the periphery of a hypothetically “reversed” lobular structure.

Despite research into cardiac cirrhosis, it is still unknown as to why congestive fibrosis develops in some patients and not others despite similar levels of cardiac decompensation and why the degree of fibrosis is variable from one region of the liver to the next. A number of studies have taken place in an attempt to discover why congestive fibrosis develops.

A possible explanation by Kotelsky et al. 1944 is that the lack of uniformity of the disease may be attributed to variability in the degree of stasis in the radicals of the hepatic vein (Kotelsky et al. 1944).   An alternative explanation is suggested by a small autopsy study of cardiac cirrhosis patients conducted by Wanless et al. 1995 which discovered 4 out of 25 livers had an acute thrombus in the sinusoids, suggesting that cardiac cirrhosis is a response to intrahepatic thrombosis.

 

Conclusion

Cardiac cirrhosis remains a rare but important cause of cirrhosis.

 

References

Brown J, Naylor M & Yagan N. Imaging of Hepatic Cirrhosis. Radiology.1997 Volume 202 Number 1.

Conn HO, Atterbury CE & Schiff L. Cirrhosis: Diseases of the Liver. 7th Edition Philadelphia, Lipencott. 1993 pp. 875-934.

Dunn GD, Hayes P, Breen KJ, Schenker S. The liver in congestive heart failure: a review.1973 Am. J. Med. Sci. 265, 174–189.

Kazin HM, Waller JV and Blumgart HL. “Cardiac Cirrhosis” of the liver. A clinical and pathological study. Arch Intern Med.1939 Vol 64 pages 457-470.

Kotelsky S and Barnebee JH. “Cardiac” or congestive cirrhosis. Pathological and Clinical Aspects. Am J Med Sci. 1944 Vol 207 pp. 421-430.

Naschitz JE, Slobodin G, Lewis RJ, Zuckerman E, Yeshurun D. Heart diseases affecting the liver and liver diseases affecting the heart. Am. Heart J.  2000 140, 111–120.

Wanless IR, Liu JJ and; Butany J. Role of thrombosis in the pathogenesis of congestive hepatic fibrosis (cardiac cirrhosis) Hepatology. 1995 Vol 21 N

 
Go to top of page

YourComments

Go to top of page Email this page Email this page to a colleague

 31 October 2014

Advanced search
Close folder Gastroenterology
Mouth
Esophagus
Stomach & duodenum
Helicobacter pylori
Small intestine
Colo-rectum and anus
Pancreas
Inflammatory bowel disease
Functional bowel disorders
Nutrition
Symptoms/signs of gastrointestinal disease
Basic science
Close folder Hepatology
Biliary tract
Viral hepatitis
Liver diseases
Cirrhosis and portal hypertension
Liver transplantation
Liver & other diseases
Pediatric hepatology
Basic science
Close folder Endoscopy
Upper endoscopy
ERCP
Colonoscopy
Endoscopic ultrasound
Technology
Practice issues

Blackwell Publishing


GastroHep.com is a Blackwell Publishing registered trademark
© 2014 Wiley-Blackwell and GastroHep.com and contributors
Privacy Statement
Disclaimer
About Us