Congestive hepatopathy is usually secondary to right-sided heart failure, which causes passive congestion of the liver leading to hepatic sinusoidal dilation and edema. Sinusoidal thrombosis and mechanical stress cause atrophy of centrilobular (zone 3) hepatocytes. Leakage of blood and protein-rich fluid into the space of Disse can activate hepatic stellate cells and promote centrilobular fibrosis. As this disease process progresses, fibrosis may bridge between liver lobules. Progressive congestive hepatopathy can cause portal hypertension leading to ascites and compression of bile canaliculi. inhibiting normal bile flow. Hepatic synthetic function may also be affected. Case reports suggest that fibrosis may improve with proper management of the underlying condition causing the hepatic congestion.
Most patients with congestive hepatopathy are older adults with right-sided heart failure; however, this entity may also be present in pediatric patients who have had certain surgical procedures for congenital heart disease. This phenomenon has been best characterized in patients who have had a Fontan procedure for an anatomic or functional single ventricle. After a Fontan, systemic venous pressures are 3-4 times greater than normal, and these patients develop progressive hepatic fibrosis.
Congestive hepatopathy is often asymptomatic, or the symptoms are masked by those of right heart failure. When symptomatic, patients often have a dull right upper quadrant pain. The liver may be enlarged and firm, and patients may experience malaise, nausea, vomiting, anorexia, and early satiety. Signs of right-sided heart failure will likely be present and include jugular venous distension, hepatojugular reflux, and lower extremity edema; about 25% of patients will have ascites. In the case of right heart failure due to tricuspid regurgitation, patients may have a pulsatile liver early in the disease process, which will diminish over time with progressive fibrosis. Jaundice, spider angiomata, and splenomegaly are only rarely present. Encephalopathy may occur in these patients secondary to cerebral hypoperfusion rather than hepatic encephalopathy. Esophageal varices are not present due to lack of a pressure gradient between the hepatic and portal venous systems.
K76.1 – Chronic passive congestion of liver
74669004 – Cardiac Cirrhosis
Differential Diagnosis & Pitfalls
- Dilated cardiomyopathy
- Alcoholic cardiomyopathy
- Constrictive pericarditis (see pericarditis)
- Cardiogenic shock
- Pulmonary hypertension
- Restrictive cardiomyopathy
- Tricuspid regurgitation
- Pulmonic stenosis
- Pulmonary embolism
- Chronic obstructive pulmonary disease / cor pulmonale
- Congestive heart failure
- Myocardial infarction
- Mitral stenosis