Liver has Complex Vascular Supply and High Metabolic Rate: makes it vulnerable to circulatory dysfunction from a variety of etiologies
Congestive Hepatopathy: passive hepatic congestion, usually attributable to right-sided congestive heart failure (with or without decreased cardiac output
Ischemic Cardiomyopathy (approximately 31% of cases)
Pericardial Disease (approximately 8% of cases)
Constrictive Pericarditis (see Constrictive Pericarditis, Constrictive Pericarditis): hepatic vein pressures are typically higher in constrictive pericarditis than those in other etiologies of right-sided congestive heart failure -> increasing probablity of developing hepatic necrosis (and ultimately “cardiac cirrhosis”)
Valvular Heart Disease (approximately 23% of cases)
Fontan Procedure (see Fontan Procedure, Fontan Procedure): directs systemic venous return to the pulmonary artery with bypass of the right ventricle
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
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)
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]
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
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
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
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