Congestive Hepatopathy (Passive Hepatic Congestion)

Physiology

  • Liver has Complex Vascular Supply and High Metabolic Rate: makes it vulnerable to circulatory dysfunction from a variety of etiologies
  • Mechanism of Hyperbilirubinemia: unknown
    • Possible Contributors
      • Canalicular Obstruction Due to Distended Hepatic Veins
      • Hemolysis
      • Hepatocellular Dysfunction
      • Medications
      • Pulmonary Infarction
      • Co-Existing Sepsis
  • “Nutmeg Liver”: classical pathologic term used to describe the gross appearance of a congested liver
    • Reddish central areas: sinusoidal congestion and bleeding into atrophic regions surrounding enlarged hepatic veins
    • Contrasting yellowish areas: represent either normal liver tissue or fatty liver
    • Hepatomegaly with a purple hue and prominent hepatic veins

Etiology

Right-Sided Congestive Heart Failure (CHF) (see Congestive Heart Failure, Congestive Heart Failure)

Other


Diagnosis

Serum/Urine Testing

  • Testing for Viral Hepatitis: to rule out viral hepatitis
  • Ferritin: to rule out hemochromatosis
  • Ceruloplasmin: to rule out Wilson’s disease
  • Serum Copper: to rule out Wilson’s disease
  • Urinary Copper: to rule out Wilson’s disease
  • Alpha-1 Antitrypsin Level: to rule out alpha-1 antitrypsin deficiency
  • Testing for Celiac Disease: to rule out celiac disease
  • Thyroid Function Tests (TFT’s): to rule out thyroid disease

Radiographic Imaging

  • Cardiac MRI: useful to define pericardial disease
  • Chest CT: useful to define pericardial disease
  • Echocardiogram: to evaluate for congestive heart failure with/without vavlular heart disease
  • RUQ U/S with Dopplers: to evaluate for possible hepatic disease

Liver Biopsy

  • Indications: may be helpful in establishing the diagnosis in equivocal cases (especially in the setting of cirrhosis)
  • Contraindications : avoid in the setting of ascites
  • Pathology in Passive Hepatic Congestion
    • Sinusoidal engorgement, degeneration, and variable hemorrhagic necrosis in zone 3 of the hepatic acinus
    • Fatty change
    • Variable degrees of cholestasis with occasional bile thrombi in the canaliculi (bile thrombi are more commonly seen in patients with severe jaundice)
  • Pathology in Cardiac Cirrhosis
    • Reticulin and collagen accumulate in zone 3 -> fibrous bands extend outward from the central veins, occasionally linking with portal tracts to produce called cardiac sclerosis that resembles micronodular cirrhosis
    • Progressive fibrosis may lead to bridging between adjacent hepatic venules, producing rings of fibrosis around the spared portal regions that characterizes “cardiac cirrhosis” (or, more accurately, cardiac fibrosis)
    • Histologic pattern is distinct from other forms of cirrhosis where fibrous bands tend to link adjacent portal areas
    • Fibrosis can also involve terminal hepatic venules, causing phlebosclerosis
    • Regeneration of periportal hepatocytes may result in focal nodular hyperplasia pattern

Endomyocardial Biopsy

  • Indication: suspicion of myocarditis or infiltrative cardiac disease (hemochromatosis, sarcoidosis)

Clinical

General Comments

  • Asymptomatic: in many cases (abnormal LFT’s may be the only finding in these cases)

Gastrointestinal Manifestations

Abnormal Liver Function Tests (LFT’s)

  • Hyperbilirubinemia with Jaundice (see Hyperbilirubinemia, Hyperbilirubinemia): hyperbilirubinemia is the most common isolated abnormality in congestive hepatopathy (occurs in 70% of cases)
    • Mechanism: unknown
    • Usually <3 mg/dL (and mostly unconjugated = indirect)
    • However, marked elevation may be seen in the setting of acute right-sided congestive heart failure
    • Serum bilirubin correlates with right atrial pressures, but not with cardiac output
    • Serum bilirubin may predict morbidity/mortality
    • Jaundice/hyperbiliruinemia is often absent in cases due to constrictive pericarditis (for unclear reasons)
  • Transaminitis: present in 33% of cases
    • Usually <2-3x upper limit of normal
    • However, may be more significantly elevated in cases with associated hypoxic/ischemic hepatitis due to decreased cardiac output (in such cases, the degree of transaminitis correlates with the amount of zone 3 necrosis on liver biopsy): in these cases, diagnostic confusion with acute viral hepatitis may occur
  • Alkaline Phosphatase: usually normal-slightly elevated, even in the presence of jaundice -> aids in distinguishing jaundice due to congestive hepatopathy from biliary obstruction
  • Hypoalbuminemia (see Hypoalbuminemia, Hypoalbuminemia): present in 30-50% of cases
    • Mechanism: likely caused by malnutrition and protein-losing gastroenteropathy due to increased intestinal lymphatic pressure
    • Rarely <2.5 g/dL
    • Degree of hypoalbuminemia does not correlate with the degree of histologic liver damage

Ascites (see Ascites, Ascites)

  • Diagnostic
    • High Total Protein: usually greater than 2.5 g/dL, reflecting the preserved serum albumin levels and the contribution of “hepatic lymph” to the ascites (presumably due to rupture of hepatic lymphatics, which are rich in protein)
    • Serum/Ascites Albumin Gradient (SAAG): >1.1 (suggetsing portal hypertension)

“Cardiac Cirrhosis” (see End-Stage Liver Disease, End-Stage Liver Disease)

  • Epidemiology: may occur in cases with chronic passive congestion that is long-standing over months-years
    • Cirrhosis due to chronic passive congestion may particularly occur in constrictive pericarditis, mitral valve disease, and pulmonary hypertension (due to pulmonary vascular disease
  • Clinical: cirrhosis due to chronic passive congestion uncommonly results in complications such as esophageal variceal bleeding [MEDLINE]

Fulminant Hepatic Failure (FHF) (see Fulminant Hepatic Failure, Fulminant Hepatic Failure)

  • Epidemiology: cases reported have been typically in the setting of superimposed shock and hepatic ischemia rather than passive congestion alone

Hepatic Encephalopathy (see Hepatic Encephalopathy, Hepatic Encephalopathy)

  • Epidemiology: congestive hepatopathy rarely causes hepatic encephalopathy

Hepatic Pulsatility

  • Epidemiology: may be seen in cases associated with tricuspid regurgitation (presystolic pulsations corresponding to prominent “v waves” on a right atrial pressure tracing)
  • Clinical: loss of hepatic pulsatility in a patient with long-standing congestive hepatopathy suggests progression to cardiac cirrhosis

Hepatojugular Reflux (see Hepatojugular Reflux, Hepatojugular Reflux)

  • Epidemiology: usually present
  • Clinical: useful to differentiate passive hepatic congestion from Budd-Chiari Syndrome and primary liver disease

Hepatomegaly (see Hepatomegaly, Hepatomegaly)

  • Clinical
    • May be massive in some cases
    • Liver edge is usually firm, smooth, and may be tender
    • Splenomegaly is uncommon (but if it occurs, it is due to transmitted elevated central venous pressure)

Hyperammonemia

  • Epidemiology: may occur occasionally
  • Mechanism: probably related to congestive heart failure itself, rather than being due to liver disease

RUQ Abdominal Pain (see Abdominal Pain, Abdominal Pain)

  • Mechanism: due to distention of hepatic capsule

Other Manifestations

  • Coagulopathy/Elevated INR: usually mildly elevated
    • Mechanism: probably due (in part) to impaired hepatic synthesis of coagulation factors II, V, VII, IX, and X
      • However, INR may not correct completely with administration of vitamin K, suggesting that other coagulation defects (such as disseminated intravascular coagulation) may contribute
  • Elevated Serum N-Terminal Pro-BNP (NT Pro-BNP) Level (see Brain Natriuretic Peptide, Brain Natriuretic Peptide): may help distinguish ascites due to congestive heart failure from ascites due to cirrhosis

Treatment

  • Treatment of Underlying Congestive Heart Failure (see Congestive Heart Failure, Congestive Heart Failure): aim to optimize cardiac output
    • Diuretics: may significantly improve jaundice and ascites
      • However, over-diuresis may precipitate hepatic necrosis [MEDLINE]
    • Improvement in LFT’s with treatment of congestive heart failure -> supports diagnosis
  • Cautious Use of Coumadin (see Coumadin, Coumadin): hepatic congestion may increase sensitivity to coumadin
  • Cautious Use of Medications with Hepatic Metabolism: hepatic congestion may impair their clearance
  • Cautious Use of Transjugular intrahepatic Portosystemic Shunt (TIPSS): usually contraindicated, due to risk of worsening right-sided congestive heart failure

References

  • The liver in heart failure; relation of anatomical, functional, and circulatory changes. Br Heart J 1951; 13:273
  • The blood ammonia in congestive heart failure. Am Heart J 1955; 50:715
  • Case Records of the Massachusetts General Hospital; case 44212. N Engl J Med 1958; 258:1058
  • Alterations in indices of liver function in congestive heart failure with particular reference to serum enzymes. Am J Med 1961; 30:211
  • Cardiac failure simulating viral hepatitis. Three cases with serum transaminase levels above 1,000. Ann Intern Med 1962; 56:784
  • The liver in congestive heart failure: a review. Am J Med Sci 1973; 265:174
  • Fulminant hepatic failure secondary to congestive heart failure. Am J Dig Dis 1976; 21:895 [MEDLINE]
  • Left-sided heart failure presenting as hepatitis. Gastroenterology 1978; 74:583
  • Ischemic hepatitis. Dig Dis Sci 1979; 24:129
  • Fulminant hepatic failure due to transient circulatory failure in patients with chronic heart disease. Dig Dis Sci 1980; 25:49
  • Severe hyperbilirubinemia and coma in chronic congestive heart failure. Dig Dis Sci 1982; 27:175
  • Constrictive pericarditis mimicking Budd-Chiari syndrome. Am J Med 1986; 80:113
  • Liver function abnormalities in chronic heart failure. Influence of systemic hemodynamics. Arch Intern Med. Jul 1987;147(7):1227-30
  • Cardiac ascites: a characterization. J Clin Gastroenterol 1988; 10:410
  • Constrictive pericarditis masquerading as Budd-Chiari syndrome. J Clin Gastroenterol 1991; 13:178
  • Hypercoagulability in heart failure. Semin Thromb Hemost 1997; 23:543
  • Heart diseases affecting the liver and liver diseases affecting the heart. Am Heart J 2000; 140:111 [MEDLINE]
  • The liver in heart failure. Clin Liver Dis 2002; 6:947
  • Acute hypoxic hepatitis (‘liver shock’): still a frequently overlooked cardiological diagnosis. Eur J Heart Fail 2004; 6:561
  • Cardiac hepatopathy before and after heart transplantation. Transpl Int. Jun 2005;18(6):697-702
  • Liver function abnormalities and outcome in patients with chronic heart failure: data from the Candesartan in Heart Failure: Assessment of Reduction in Mortality and Morbidity (CHARM) program. Eur J Heart Fail 2009; 11:170
  • Cardiac cirrhosis: a rare manifestation of an uncorrected primum atrial septal defect. J Cardiovasc Med (Hagerstown). Dec 3 2009
  • The value of hepatic diffusion-weighted MR imaging in demonstrating hepatic congestion secondary to pulmonary hypertension. Cardiovasc Ultrasound 2010; 8:28
  • Usefulness of serum N-terminal-ProBNP in distinguishing ascites due to cirrhosis from ascites due to heart failure. J Clin Gastroenterol 2010; 44:e23
  • Congenital heart disease and the liver. Hepatology 2012; 56:1160