Cardiac Cirrhosis and Congestive Hepatopathy Workup

• 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 (PT): One study from the 1960s showed prothrombin time to be abnormal in as many as 80% of patients with acute or chronic right-sided heart failure.
• 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.

  • Serum iron, total iron-binding capacity, and ferritin: An evaluation for hemochromatosis is indicated when cardiac cirrhosis presents with significant or persistent LFT abnormalities.
  • Thyroid-stimulating hormone (TSH): TSH level is indicated in patients with unexplained cardiac cirrhosis and atrial fibrillation.

• Chest radiography: Images may show cardiomegaly, pulmonary venous hypertension, interstitial or pulmonary edema, or pleural effusion. Pleural effusions typically are larger on the right.
• 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 (US): 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.
• CT scan and MRI: CT scan and MRI 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. Cardiac cirrhosis. Congestive hepatopathy with large inferior vena cava.

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.^[3]
  • 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/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.
• 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.

• 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^[4] 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.^[5]
• 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.