eMedicine Specialties > Cardiology > Myocardial Disease and Cardiomyopathies

Cardiac Cirrhosis

Author: Xiushui (Mike) Ren, MD, Clinical Cardiology Fellow, Division of Cardiology, Kanbar Cardiac Center, California Pacific Medical Center
Coauthor(s): Allan Hiroshi Andrews, MD, Clinical Assistant Professor, Department of Internal Medicine, Medical College of Georgia; Staff Gastroenterologist, Department of Internal Medicine, Eisenhower Army Medical Center; Kent C Holtzmuller, MD, Consulting Staff, Department of Medicine, Division of Hepatology, Mecklenburg Medical Group
Contributor Information and Disclosures

Updated: Mar 16, 2008

Introduction

Background

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 is unknown. Because of this, treatment is aimed at managing the patient's underlying heart failure.

Distinguish cardiac cirrhosis from ischemic hepatitis. 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 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.

Pathophysiology

Decompensated right ventricular or biventricular heart failure causes transmission of elevated central venous pressures directly 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. That intrahepatic vascular lesions are confined to areas of the liver with higher fibrotic burden suggests that cardiac cirrhosis requires a higher grade of vascular obstruction, such as intrahepatic thrombosis, for its development. The theory proposes that thrombosis of sinusoids and terminal hepatic venules propagates to medium-sized hepatic veins and to portal vein branches, resulting in parenchymal extinction and fibrosis.

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.¹

Age

No published data exist. However, the prevalence of cardiac cirrhosis in the United States, like that of CHF, almost certainly increases with age.

Clinical

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
    • 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
    • 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 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 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.²
• Rales on lung examination indicate biventricular CHF. Decreased basilar breath sounds from pleural effusion also are common.
• 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 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.
• 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. 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%)

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