congestive hepatopathy

Congestive hepatopathy includes a spectrum of hepatic derangements that can occur in the setting of right-sided heart failure (and its underlying causes). If there is subsequent hepatic fibrosis the term cardiac cirrhosis may be used. The condition can rarely occur as a result of non-cardiac causes (e.g. renal arteriovenous malformation).

Pathology

Etiology

Congestive hepatopathy resulting from passive hepatic congestion is caused by stasis of blood within the liver parenchyma due to compromise of hepatic venous drainage. Prolonged exposure to elevated hepatic venous pressure may lead to liver fibrosis and cirrhosis.

Underlying conditions include :

• pulmonary hypertension
• tricuspid regurgitation
• constrictive pericarditis
• restrictive cardiomyopathy
• congenital heart disease, especially those treated with a Fontan procedure

Markers

If paracentesis is performed, the serum ascites albumin gradient (SAAG) will be equal to or above 1.1 g/dL, which is consistent with ascites due to portal hypertension. Elevation of total serum bilirubin level can occur up to 70% of patients with congestive hepatopathy .

Radiographic features

The majority (80%) of patients will have hepatomegaly, and in severe cases have peripheral edema, ascites and/or pleural effusions .

CT

• reticular enhancement pattern, similar to nutmeg liver in Budd-Chiari syndrome
• zonal enhancement pattern 
• arterial-enhancing (i.e. hypervascular) nodules, which may represent focal nodular hyperplasia (more commonly) or hepatocellular carcinoma (especially in the setting of cirrhosis)
• Reflux of intravenous contrast into the IVC and hepatic veins

Ultrasound

Sonographic findings are generally non-specific when used in isolation, but may contribute to a clinical picture when putative cause and effect (i.e. heart failure and liver disease) are shown to co-exist. 2D/B-mode ultrasound may reveal the following features :

• cirrhosis with portal hypertension
  • decreased hepatic size with nodularity and altered echogenicity
  • portosystemic shunts
    • color flow Doppler of the portal vein extending to the hepatic edge may suggest recanalization of the umbilical vein
  • venous dilation
    • left renal vein diameter >1.5 cm
    • portal vein diameter >1.3 cm
    • superior mesenteric vein >1.0 cm
  • splenomegaly
  • ascites
• cardiac chamber enlargement and/or dysfunction
  • left ventricular systolic or diastolic dysfunction
    • decreased cardiac index
  • right ventricular dysfunction
  • right and/or left atrial enlargement
  • pleural effusion
  • valvular stenosis or regurgitation
• sonographic pulmonary edema
• dilated (>2.1 cm) inferior vena cava
  • more specific for elevated filling pressures when lacking respiratory variation (<50%)
  • elevated filling pressures in the absence of structural cardiac disease may (rarely) imply constrictive pericarditis

Doppler studies may demonstrate:

• increased hepatic arterial resistance (RI >0.7)
  • resistive index (RI) normally between 0.55 and 0.7
  • this change may be obfuscated by portal hypertension related shunting, which classically results in a decreased RI
• pulsatility of the hepatic venous Doppler waveform
  • prominent a wave and v wave
  • tricuspid regurgitation may diminish or reverse the S wave
  • pure right ventricular dysfunction will have a preserved S/D relationship with the amplitude of the S wave > D wave
• elevated cardiac filling pressures
  • pulsed wave Doppler of the tricuspid regurgitant jet showing an elevated right ventricular systolic pressure (RVSP)
  • pulsed wave doppler of the right upper pulmonary vein showing high velocity AR (atrial reversal) waves with low S/D ratio
  • pulsed wave doppler of the mitral valve inflow showing restrictive filling

Differential diagnosis

For CT appearances of a nutmeg liver, consider:

• Budd-Chiari syndrome
• hepatic veno-occlusive disease (sinusoidal obstruction syndrome)
• portal venous emboli
• shunting through vascular malformations - e.g. hereditary hemorrhagic telangiectasia

Siehe auch:

• Regeneratknoten der Leber
• Pericarditis constrictiva
• hepatic veno-occlusive disease
• Rechtsherzbelastung
• Radiologische Zeichen der Rechtsherzinsuffizienz
• Budd-Chiari-Syndrom
• Cirrhose cardiaque
• Muskatnussleber

und weiter:

• pathological conditions of hepatic vascularization
• inhomogene Leberperfusion
• erweiterte Lebervenen
• CT signs of right heart strain
• Leberperfusion
• Kontrastmittelrückstau Rechtsherzinsuffizienz