Cardiac Cirrhosis

Article Author:
Mary Rodriguez Ziccardi
Article Editor:
Steve Bhimji
Updated:
10/27/2018 12:31:27 PM
PubMed Link:
Cardiac Cirrhosis

Introduction

[Epidemiology: Please read for clarity. Uses the word than without a clear comparison."In the situation where cardiac cirrhosis is present, the mortality is related to the underlying cardiac disease than the hepatic congestion and injury."]

Cardiac cirrhosis is a term used to include the spectrum of hepatic disorders that occur secondary to passive hepatic congestion due to cardiac dysfunction, especially the right heart chambers. Cardiac cirrhosis can be caused by any right sided pathology that can generate right heart failure that causes an increase in venous congestion and increase of pressure in the hepatic sinusoid. Cardiac abnormalities that can cause cardiac cirrhosis due to hepatic congestion are (1) valvular disease, (2) severe pulmonary hypertension, (3) cor pulmonale, (4) biventricular heart failure or pericardial diseases as cardiac tamponade, and (5) constrictive pericarditis.

Etiology

Cardiac cirrhosis is the consequence of the hemodynamic rearrangements caused by a failing heart. For this, heart failure is the first event that precipitates hepatic congestion and liver injury. 

Right-sided heart failure secondary to left-sided heart failure, valvular diseases (tricuspid regurgitation, mitral stenosis) or decreases in relaxation or filling of the right sided heart as in the setting of constrictive pericarditis, can produce an increase in the preload and backflow to the hepatic system producing hepatic congestion.

Epidemiology

The prevalence of cardiac cirrhosis is hard to determine, especially because this condition can be asymptomatic or the diagnosis missed because of other causes of liver injury or confused with an ischemic hepatic injury. In the situation where cardiac cirrhosis is present, the mortality is related more to the underlying cardiac disease than the hepatic congestion and injury. 

Cardiac cirrhosis age of presentation is unclear but is most likely to be related to the onset or decompensation of heart failure that also has an increased risk of development of heart disease with aging. Similar to the incidence per age, there is no specific sex preference for the development of cardiac cirrhosis, but it is more likely to be present in males because they also have an increased risk of developing heart failure and consecutive cardiac cirrhosis than females.

Pathophysiology

The development of cardiac cirrhosis is dependent upon an underlying cardiac condition. The primary pathophysiology causing hepatic dysfunction secondary to congestion due to heart dysfunction is caused by either increase in cardiac filling pressures or low cardiac output and impaired perfusion.

There are several mechanisms which create unbalance in the circulatory system and will cause hepatic injury. The main mechanism causing hepatic dysfunction secondary to congestion due to heart dysfunction is either an increase in cardiac filling pressures or low cardiac output and impaired perfusion. An increase in the preload or central venous pressure due to right ventricular dysfunction may cause direct liver damage due to increasing retrograde pressure to the venous and capillaries into the liver. This generates an elevation in liver enzymes that may be due to the elevated pressure transmitted from the right heart chambers (right ventricle and atrium) to the hepatic veins and sinusoids leading to intrahepatic edema, decreased perfusion and oxygen diffusion as well as hemorrhagic injury and modification on the hepatocyte architecture and atrophy with associated collagen deposition, and fibrosis to the hepatic veins and sinusoids.

Any cause of right-sided heart failure can generate hepatic congestion, including (1) mitral stenosis, (2) tricuspid regurgitation, (3) constrictive pericarditis, (4) right and left sided heart failure, and (5) cor pulmonale. Tricuspid regurgitation is recognized as one of the factors for severe hepatic congestion due to direct transmission of pressure from the right ventricle to the hepatic vasculature.

Another mechanism that can cause hepatic injury, especially when the left side of the heart is affected, is impaired perfusion and tissue hypoxia from decreased cardiac output. This may be associated with acute hepatocellular necrosis with marked elevation of serum alanine aminotransferase (ALT), aspartate aminotransferase (AST), lactic dehydrogenase (LDH), and prolongation of coagulation studies as thrombin and prothrombin time.

History and Physical

Patients with cardiac cirrhosis are usually asymptomatic, and the condition is suggested from abnormal liver function tests. When symptoms are present, these are typically progressive but may present suddenly and dramatically in cases of acute right ventricular decompensation, constrictive pericarditis or acute decompensation of a valve disease.

Symptomatic patients typically present with abdominal pain that can be localized in right upper quadrant (secondary to hepatic capsule dilation), nausea and vomiting, jaundice, hepatomegaly, abdominal distention, and ascites. These findings are easily confused with biliary disease. Peripheral edema and jugular venous distention are common findings in right side heart failure. Jaundice is not commonly reported.

Liver injury can present in the setting of heart failure exacerbation with liver enzymes that are massively elevated after 24 to 72 hours after the cardiac decompensation. 

Evaluation

At the moment of presentation, the evaluation of the cause of liver injury, as well as cardiac decompensation, must be determined. A careful history should be taken to evaluate possible causes and risk factors as medications and infection that might be causing liver injury.

An initial evaluation of liver function should be done. Within the different laboratory abnormalities, the most common finding is the elevation of the total bilirubin mostly due to increasing indirect bilirubin. This finding has been found in 70% of patients with cardiac cirrhosis, and the bilirubin levels are rarely greater than 3 mg/dl. Alteration of the synthetic function of the liver is also common with prolongation of the coagulation times, despite this, increases in the international normalized ratio are not that common but can be elevated in severe cases of ischemic injury. Serum albumin levels can be decreased in a 40% of patients. Severe elevation in LDH and ALT/LDH ratio of less than 1.5 helps to differentiate perfusion injury from other causes of acute hepatitis.

Evaluation of causes that can explain exacerbation of heart failure is important. Cardiac enzymes and brain peptide natriuretic peptide (BNP) may be elevated. An electrocardiogram can demonstrate arrhythmias and ischemic changes as well as suggest right ventricular hypertrophy. Echocardiography is also an important test to determine valvular disease, and functional status of the left and right sided heart.

Due to common signs and symptoms of the biliary disease, imaging evaluation should be considered. Abdominal ultrasound for evaluation of the biliary tract and gallbladder can rule out acute obstructive disease or hepatic vein thrombosis. 

An echocardiogram is an important test to evaluate the underlying cause of congestive hepatopathy. Evaluate for valvular disease, wall motion abnormalities, pulmonary artery systolic pressure, mitral inflow, atrial and ventricular size, and evaluation for pericardial effusion or constrictive pericarditis. Inferior vena cava (IVC) evaluation would help to define volume status and pressure between the right atrium and the hepatic circulation. IVC respiratory variation (normally greater than or equal to 50% narrowing during inspiration) or IVC diameter greater than or equal to 2.3 cm suggests right-sided cardiac disease with increased right atrial filling pressures. CT and MRI evaluations of cardiac and hepatic conditions also can be used to determine etiology of the clinical presentation.

In the presence of ascites, paracentesis should be performed. This fluid typically reveals a high protein content, commonly > 2.5g/dl. This increased in protein can be related to hepatic lymphatic ruptured with leakage of protein-rich fluid. Serum to ascites albumin gradient (SAAG) greater than 1.1 reflects portal hypertension.

Treatment / Management

The treatment of cardiac cirrhosis is based on management of the underlying cardiac condition causing hemodynamic unbalance. Determination of the volume status and adequate management of fluids for these patients is important. Clinical features of cardiac cirrhosis may improve dramatically with adequate diuresis.  Close monitoring of cardiac output is important to prevent ischemic hepatitis. Use of chronotropic agents or supportive pump can be considered.