Cardiac cirrhosis (congestive hepatopathy) includes a spectrum of hepatic

derangements that occur in the setting of right-sided heart failure. Clinically, the
signs and symptoms of congestive heart failure (CHF) dominate the disorder. Unlike
cirrhosis caused by chronic alcohol use or viral hepatitis, the effect of cardiac
cirrhosis on overall prognosis has not been clearly established. As a result, treatment
is aimed at managing the underlying heart failure. [1, 2]
Distinguishing cardiac cirrhosis from ischemic hepatitis is important. The latter
condition may involve massive hepatocellular necrosis caused by sudden
cardiogenic shock or other hemodynamic collapse. Typically, sudden and dramatic
serum hepatic transaminase elevations lead to its discovery. Although cardiac
cirrhosis and ischemic hepatitis arise from distinct underlying cardiac lesions (right-
sided heart failure in the former and left-sided failure in the latter), in clinical practice
they may present together.
Despite its name, cardiac cirrhosis (which usually implies congestive hepatopathy
that results in liver fibrosis) rarely satisfies strict pathologic criteria for cirrhosis. The
terms congestive hepatopathy and chronic passive liver congestion are more
accurate, but the name cardiac cirrhosis has become convention. For the remainder
of this chapter, the term cardiac cirrhosis will be used to mean congestive
hepatopathy with or without liver fibrosis.

Cardiac cirrhosis.
Congestive hepatopathy with large renal vein.
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 Cardiac cirrhosis.
Congestive hepatopathy with large inferior vena cava.

Pathophysiology
Decompensated right ventricular or biventricular heart failure causes
transmission of elevated right atrial pressure to the liver via the inferior
vena cava and hepatic veins. At a cellular level, venous congestion
impedes efficient drainage of sinusoidal blood flow into terminal hepatic
venules. Sinusoidal stasis results in accumulation of deoxygenated blood,
parenchymal atrophy, necrosis, collagen deposition, and, ultimately,
fibrosis.
A separate theory proposes that cardiac cirrhosis is not simply a response
to chronically increased pressure and sinusoidal stasis. Because
intrahepatic vascular lesions are confined to areas of the liver with higher
fibrotic burden, cardiac cirrhosis may require a higher grade of vascular
obstruction, such as intrahepatic thrombosis, for its development. Thus,
thrombosis of sinusoids and terminal hepatic venules propagates to
medium-sized hepatic veins and to portal vein branches, resulting in
parenchymal extinction and fibrosis.

Epidemiology
Frequency
United States
Cardiac cirrhosis rarely occurs in the United States. Its true prevalence is
difficult to estimate, since the disease typically remains subclinical and
undiagnosed. The incidence of cardiac cirrhosis at autopsy has decreased
significantly over the past several decades. This may be due to lower rates
of uncorrected rheumatic heart disease and constrictive pericardial
disease.
Mortality/Morbidity
The effect of cardiac cirrhosis on mortality and morbidity rates is unknown.
The severity of the patient's underlying cardiac disease, which is typically
advanced and chronic, is the major determinant of overall outcome.
Sex
Comparative sex data for cardiac cirrhosis do not exist. However, because
CHF is more common in men than women in the United States, the same is
likely for cardiac cirrhosis. [3]
Age
No published data exist. However, the prevalence of cardiac cirrhosis in the
United States, like that of CHF, almost certainly increases with age.
History
Symptoms of CHF almost always mask gastrointestinal symptoms.
Symptoms typically progress insidiously but may present suddenly and
dramatically in cases of constrictive pericarditis or acute right ventricular
decompensation. Patients may present with asymptomatic liver enzyme
abnormalities, jaundice, and right upper quadrant discomfort. Case reports
of fulminant hepatic failure have also been reported.
In addition to CHF, a patient's past medical history is likely to include one
or more of the following:
 Coronary artery disease
 Myocardial infarction
 Hypertension
 Dilated cardiomyopathy
 Valvular heart disease
 Chronic alcohol abuse
 Chronic obstructive pulmonary disease (COPD)
 Cor pulmonale
 Pulmonary hypertension
 Constrictive pericarditis
 Rheumatic heart disease
Symptoms may be divided into those that accompany right ventricular heart
failure and the additional findings of biventricular failure.
Symptoms associated with isolated right-sided heart failure are as follows:
 Dependent edema and weight gain
 Increased abdominal girth
 Right upper quadrant abdominal pain
 Nocturia
 Progressive fatigue
 Anorexia, nausea, and vomiting
Symptoms associated with biventricular heart failure are as follows:
 Progressive dyspnea
  Orthopnea
 Paroxysmal nocturnal dyspnea
 Wheezing and/or cough (ie, cardiac asthma)
 Anxiety: Multifactorial causes include dyspnea, palpitations, and
increased sympathetic tone.
Physical
Signs of heart failure dominate the physical examination findings.
Edema
Edema typically occurs in the lower extremities and dependent regions,
which may progress to anasarca in cases of advanced and untreated heart
failure. Chronic edema may be associated with lower extremity
pigmentation, induration, and cellulitis.
Jugular venous pressure
Jugular venous pressure is elevated. Further distention of neck veins may
be elicited with application of pressure over the right upper quadrant for as
long as 1 minute (ie, hepatojugular reflux).
Paradoxical rise in jugular venous pressure during inspiration (ie, Kussmaul
sign) may indicate constrictive pericarditis, right ventricular heart failure,
tricuspid stenosis, or cor pulmonale.
Right atrial pressure recordings reveal large a waves, indicating elevated
right atrial pressure that may appear as presystolic liver pulsations.
Prominent v waves with rapid y descent indicate tricuspid regurgitation.
Progression to a systolic, or c-v, wave occurs in severe tricuspid
insufficiency and may appear as systolic liver pulsations. [4]
Rales
Rales on lung examination indicate biventricular CHF. Decreased basilar
breath sounds from pleural effusion also are common.
Cardiac abnormalities
Cardiac examination may reveal abnormalities related to right ventricular
failure, tricuspid regurgitation, or both.
Abnormal systolic sternal or left parasternal lift signifies both pulmonary
and right ventricular hypertension.
Right ventricular third and fourth heart sounds commonly are appreciated
at the lower left sternal border of the sternum or over the xiphoid. Right
ventricular S 3suggests right ventricular failure. Right ventricular S 4 results
from right atrial contraction into a noncompliant right ventricle. Inspiration
increases the intensity of both extra heart sounds.
The holosystolic, high-pitched, blowing murmur of tricuspid insufficiency
often accompanies severe right ventricular dilation and failure. The murmur
is best heard at the lower left sternal border. But in cases of severe right
ventricular enlargement, the murmur may be displaced as far laterally as
the left midclavicular line. The murmur intensifies with inspiration and
decreases with expiration.
Signs of pulmonary hypertension include a closely split S 2 with a loud
pulmonic component. The Graham Steell murmur of pulmonary
hypertensive pulmonic regurgitation is a high-pitched, blowing diastolic
murmur beginning with a loud P2 and continuing through most of diastole.
Hepatomegaly
Hepatomegaly is common, usually presenting as a firm, hard liver.
Elevated hydrostatic pressure within the hepatic veins and the peritoneal
venous drainage system causes cardiac ascites. Protein-losing enteropathy
with subsequent reduction of plasma oncotic pressure also may exacerbate
ascites.
Splenomegaly may be found.
Fewer than 10% of patients exhibit jaundice.
Hepatic encephalopathy is rare.
Cachexia
Anorexia, weight loss, and malnutrition (ie, cardiac cachexia) indicate
advanced underlying heart disease.
Causes
Causes of cardiac cirrhosis mirror the many etiologies of right-sided CHF,
including congenital heart disease. Although inferior vena caval thrombosis
and Budd-Chiari syndrome exhibit similar pathophysiology, they are
categorized separately and are not included as causes of cardiac cirrhosis.
The most frequent causes of cardiac cirrhosis are the following:
 Ischemic heart disease (31%)
 Cardiomyopathy (23%)
 Valvular heart disease (23%)
 Primary lung disease (15%)
 Pericardial disease (8%)
A study by Yoo et al suggested that the Fontan procedure is a significant
risk factor for cardiac cirrhosis. The study included 46 patients with Fontan
circulation, as well as 26 patients with right-sided heart failure and hepatic
congestion. The Fontan patients were found, via transient elastography, to
have a significantly higher liver-stiffness value than did the patients with
right-sided heart failure. Moreover, a significant association was seen
between liver stiffness in the Fontan patients and total bilirubin and albumin
levels, white blood cell counts, and aspartate aminotransferase-to-platelet
ratio indexes. The investigators found the age at which the Fontan
procedure was completed and the total bilirubin level to be independent risk
factors for hepatopathy. [5]
Diagnostic Considerations
Important considerations include the following:
 Diagnose a correctable underlying cause of cardiac cirrhosis . For example,
patients with alcoholism and cardiac disease who present with ascites may be
misdiagnosed by attributing liver derangements to alcoholic cirrhosis.
 Search for concomitant hepatobiliary disease in patients with either significantly
or persistently elevated hepatic transaminases, alkaline phosphatase, or total
bilirubin levels. For example, collecting a detailed social history may lead to the
diagnosis of acute hepatitis B infection and prevent progression to end-stage
liver failure.
 Do not perform unnecessary liver biopsies.
 Transjugular intrahepatic portosystemic shunt (TIPSS) is contraindicated in
cardiac cirrhosis. It may precipitate acute right heart failure from an acute
increase in pulmonary arterial pressure.
 Adjust hepatically cleared medications.
Other conditions to consider in patients with cardiac cirrhosis and congestive
hepatopathy include the following:
 Biventricular congestive heart failure
 Rright ventricular congestive heart failure
 Hepatic veno-occlusive disease
 Ischemic hepatitis
Differential Diagnoses
 Alcoholic Cardiomyopathy
 Alcoholism
 Benign Cardiac Tumors
 Budd-Chiari Syndrome
 Cardiogenic Shock
 Cirrhosis
 Cocaine-Related Cardiomyopathy
 Constrictive Pericarditis
 Cor Pulmonale
 Dilated Cardiomyopathy
 Effusive-Constrictive Pericarditis
 Hemochromatosis
 Hypertrophic Cardiomyopathy
 Inferior Vena Caval Thrombosis
 Peripartum Cardiomyopathy
 Portal Hypertension
 Portal Vein Obstruction
 Primary Cardiac Neoplasms
 Idiopathic Pulmonary Arterial Hypertension
 Pulmonary Regurgitation (Pulmonic Regurgitation)
 Pulmonic Stenosis
 Restrictive Cardiomyopathy
 Pulmonary Arterial Hypertension
 Tricuspid Atresia
 Tricuspid Regurgitation
 Tricuspid Stenosis
Laboratory Studies
Liver function tests
Evaluate severity of hepatic failure with liver function tests (LFTs), including
hepatic transaminases, alkaline phosphatase, total bilirubin, and albumin.
The most common liver enzyme abnormality is an elevation of serum
bilirubin. Patients with cardiac cirrhosis may exhibit modest elevations in
aspartate aminotransferase (AST), alanine aminotransferase (ALT),
alkaline phosphatase, and total bilirubin, as well as mild decreases in
albumin.
Abnormal values are more common in patients with mean right atrial
pressures in excess of 10 mm Hg and cardiac indices less than 1.5
L/min/m2.
Abnormalities typically remain clinically silent and resolve with
compensation of heart failure.
Extreme elevations of AST and ALT should alert the clinician to other
causes of liver failure, including ischemic, toxic, and viral hepatitis.
Prothrombin time
A study from the 1960s showed prothrombin time (PT) to be abnormal in as
many as 80% of patients with acute or chronic right-sided heart failure.
Other laboratory studies
Evaluate serial cardiac enzymes, CBC count, urinalysis, and routine serum
electrolytes in a patient with cardiac cirrhosis in the setting of new-onset
heart failure.
Search for evidence of reversible causes of CHF. For example, obtain
levels of serum iron, total iron-binding capacity, and ferritin in an evaluation
for hemochromatosis when cardiac cirrhosis presents with significant or
persistent LFT abnormalities. Thyroid-stimulating hormone (TSH) levels are
indicated in patients with unexplained cardiac cirrhosis and atrial fibrillation.
Chest Radiography
Radiographic images may show cardiomegaly, pulmonary venous
hypertension, interstitial or pulmonary edema, or pleural effusion. Pleural
effusions typically are larger on the right.
Echocardiography
Transthoracic echocardiogram with Doppler
An echocardiogram may diagnose the underlying cause of cardiac
cirrhosis. Evaluation of biventricular size, mass, function, wall motion, and
valves are indicated.
Because restrictive cardiomyopathy and pericardial constriction can lead to
cardiac cirrhosis, specific attention should be paid to diastolic function
parameters such as mitral inflow, pulmonary vein flow, mitral annular flow,
and their responses to respiration.
Lack of inferior vena cava (IVC) respiratory variation (normally greater than
or equal to 50% narrowing during inspiration) or IVC diameter greater than
or equal to 2.3 cm suggest right-sided cardiac disease with increased right
atrial filling pressures.
Subcostal Doppler view of hepatic veins demonstrating systolic flow
reversal is highly specific for clinically significant tricuspid regurgitation.
Radionuclide imaging
Radionuclide imaging with thallium or technetium is a noninvasive means
to identify reversible cardiac ischemia in patients with cardiac cirrhosis in
the setting of new or decompensated heart failure. Technetium-labeled
agents and positron-emission tomography (PET) identify dilated
cardiomyopathy and determine myocardial function.
Abdominal Doppler Ultrasonography
Consider abdominal Doppler US in the setting of ascites, right upper
quadrant abdominal pain, jaundice, and/or abnormal serum LFTs that are
refractory to effective treatment of underlying heart failure. The test is
performed to search for an alternative diagnosis, such as Budd-Chiari
syndrome.
Computed Tomography Scanning and Magnetic
Resonance Imaging
CT scan and MRI help to diagnose restrictive and constrictive pericardial
disease. These studies also may identify enlarged chamber size,
ventricular hypertrophy, diffuse cardiomyopathy, valvular disease, and
other structural abnormalities such as arrhythmogenic dysplasia of the right
ventricle. Both can measure ejection fraction and effectively rule out
cardiac cirrhosis. Body imaging may reveal evidence of cardiac cirrhosis,
including hepatomegaly, hepatic congestion, IVC enlargement, and
splenomegaly (see following images).

Cardiac cirrhosis.
Congestive hepatopathy with large renal vein.
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Cardiac cirrhosis.
Congestive hepatopathy with large inferior vena cava.
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Medical Care
No prospective studies have been performed to evaluate the medical
treatment of cardiac cirrhosis. Because no data suggest that the presence
of cardiac cirrhosis worsens mortality or morbidity rates, direct treatment at
the underlying source of elevated right-sided heart pressure and hepatic
venous congestion.
Note the following:
 Initiate treatment in an inpatient setting, both to rule out ischemic heart
disease and to administer IV diuretics.
 In most cases, diuresis is the cornerstone of initial medical therapy for
symptomatic relief.
 Once the patient is euvolemic, beta-blockers and ace inhibitors should
be added if the underlying cause is left ventricular dysfunction.
 Spironolactone should be considered, especially if there is New York
Heart Association class III or IV heart failure.
Consult with specialists in cardiology, gastroenterology, and diet and/or
nutrition.
Surgical Care
Definitive treatment of cardiac cirrhosis sometimes requires surgical
intervention, particularly when the underlying structural or anatomic lesion
remains symptomatic despite maximal medical therapy.
Examples of surgical intervention include the following:
 Coronary artery bypass surgery or percutaneous transluminal coronary
angioplasty for ischemic cardiomyopathy
 Tricuspid valve repair or replacement for tricuspid regurgitation or
tricuspid stenosis
  Pericardiectomy (cardiac decortication) for constrictive pericarditis
 Peritoneovenous shunt not indicated to treat cardiac ascites
 Transjugular intrahepatic portosystemic shunt (TIPSS): This is
generally contraindicated because of the risk of acute right-sided
decompensation from increased venous return. One recent case report
illustrated the use of TIPSS procedure in a patient with cardiac
cirrhosis after heart transplant that resulted in a successful outcome. [9]
 Cardiac transplantation can be considered for end-stage
cardiomyopathy. The presence of cardiac cirrhosis with significant liver
fibrosis is considered a contraindication to transplantation. Although
standard transplant criteria applies, several caveats should be
considered. First, right-heart failure can be accompanied by significant
pulmonary hypertension, which may necessitate combined heart-lung
transplant. Second, synthetic liver function may be affected, leading to
bleeding complications associated with transplantation. However, a
study examining the reversibility of cardiac cirrhosis in patients
undergoing heart transplant showed that synthetic function significantly
improved within 3 months after transplant. [10]
Diet
Sodium restriction is a fundamental component of long-term management.
The sodium intake goal is less than 2 g/d.
Activity
A sensible exercise program is appropriate for most patients with cardiac
cirrhosis after medical control of their underlying heart failure.
Medication Summary
With few exceptions (eg, acute right ventricular myocardial infarction),
diuresis is the cornerstone of initial management of cardiac cirrhosis. As
cardiac cirrhosis is a direct complication of elevated central venous
pressures, effective diuresis should improve hepatic derangements. Lack of
improvement should prompt a search for primary hepatic disease.
Beyond diuretics, medical therapy should be directed at treating underlying
heart failure and correcting the source of elevated right-sided heart
pressures.
Diuretics
Class Summary
Initial treatment of cardiac cirrhosis usually requires a loop diuretic (eg,
furosemide). Spironolactone may provide additional diuresis through its
aldosterone antagonism effects.

Furosemide (Lasix)
 View full drug information
Increases excretion of water by interfering with chloride-binding cotransport
system, which in turn inhibits sodium and chloride reabsorption in
ascending loop of Henle and distal renal tubule.
Initial administration should be IV to avoid poor bowel absorption through
edematous bowel mucosa. Start dosing low and increase to achieve
desired diuresis and clinical effect. Useful clinical target is return to patient's
baseline weight.
Rising serum BUN and creatinine levels are indicators of prerenal azotemia
and suggest maximal diuresis has been achieved. Once determined,
administer effective dose qd or bid.

Spironolactone (Aldactone)
 View full drug information
For management of edema resulting from excessive aldosterone levels
secondary to hepatic cirrhosis or CHF. Competes with aldosterone for
receptor sites in distal renal tubules, increasing water excretion while
retaining potassium and hydrogen ions.
Further Outpatient Care
Instruct patients to maintain a diary of their daily weights. Specific
instructions may be issued to increase the patient's oral diuretic dose, as
well as to return for immediate medical evaluation when certain weight
increases are exceeded (eg, 2 lb/d or 5 lb/wk).
Schedule periodic follow-up. Monitor symptoms, preferably using well-
defined activities (eg, walking 100 ft on ground level, climbing 1-2 flights of
stairs).
Follow serum levels of potassium, BUN, creatinine, AST, ALT, alkaline
phosphatase, and total bilirubin. All should normalize with attainment of
heart failure compensation. Failure of levels to resolve despite heart failure
resolution should prompt evaluation of noncardiac sources of liver disease.
Electrocardiography
Evidence of prior myocardial infarction, ventricular hypertrophy, and right
atrial enlargement is common.
Right ventricular hypertrophy, right axis deviation, and right bundle-branch
block may suggest chronic right ventricular pressure overload.
Paracentesis
Diagnostic paracentesis may distinguish between cardiac and other
etiologies of ascites. The information is useful especially in patients with
chronic alcoholism and uncharacterized cardiac disease. Evaluate fluid for
cell count and differential, albumin, total protein, and cytology.
Typically, cardiac ascites will reveal a high serum-ascites albumin gradient
(SAAG) greater than 1.1 g/dL and a high ascitic fluid total protein greater
than 2.5 g/dL. Patients with cirrhotic ascites also have a high SAAG value,
but ascitic fluid total protein will be greater than 2.5 g/dL only 10% of the
time. [6] See the Ascites Albumin Gradient calculator.
Employ therapeutic paracentesis for ascites refractory to diuretic treatment.
Because hepatic albumin synthetic function usually is preserved in cardiac
cirrhosis, parenteral albumin supplementation after paracentesis is not
indicated.
Cardiac Catheterization and Coronary Angiography
The procedure may be indicated in patients with cardiac cirrhosis and heart
failure in the context of known or suspected coronary artery disease. The
study is employed primarily to evaluate coronary arterial anatomy and the
need for revascularization.
Perform right heart catheterization to diagnose pulmonary hypertension in
the setting of suggestive physical examination or echocardiographic
findings.
In less than 1% of patients with chronic liver failure, pulmonary
hypertension occurs in the absence of underlying pulmonary or cardiac
disease. This entity, known as portopulmonary hypertension, may progress
to right ventricular failure and present a diagnostic challenge to determine
whether liver failure or heart disease is the primary lesion.
Biopsy
Needle liver biopsy
The procedure is not indicated routinely. Needle biopsy is indicated in heart
transplant candidates with ascites to rule out cirrhosis.
Endomyocardial biopsy
The procedure may be indicated in patients with cardiac cirrhosis with
deteriorating clinical condition and a strong clinical suspicion for
myocarditis. It also may be indicated in the presence of a systemic disease
with possible cardiac involvement, such as hemochromatosis or sarcoid.
Histologic Findings
Cardiac cirrhosis is associated with characteristic histologic changes. The
presence of centrilobular parenchymal atrophy, sinusoidal and terminal
hepatic venular distention, and perisinusoidal collagen deposition
establishes chronic passive hepatic congestion (CPC).
In more severe cases, centrilobular fibrosis develops and eventually may
include diffuse fibrous septa [7] and regenerative nodules characteristic of
true cirrhosis.
Histologic findings are bland, with an absence of inflammatory cells.
Exposure of the liver to venous hypertension alone has not been
demonstrated to cause centrilobular necrosis (CLN); in practice, however,
histologic features of both CPC and CLN frequently occur together. CPC
and CLN form a morphological continuum reflecting degrees of preexisting
hepatic congestion and acute liver hypoperfusion. The synergistic
combination of CPC and CLN is known as centrilobular hemorrhagic
necrosis, referred to more commonly as nutmeg liver. [8]
The liver's mottled gross appearance results from the contrast of red-brown
centrilobular regions suffused with blood against viable, if somewhat fatty,
periportal tissue.