Cardiac Cirrhosis and Congestive Hepatopathy Clinical Presentation

Updated: Dec 22, 2014
  • Author: Xiushui (Mike) Ren, MD; Chief Editor: Henry H Ooi, MD, MRCPI  more...
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Presentation

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:

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.
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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 3 suggests 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.

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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]

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