Bayraktar UD, Seren S, Bayraktar Y. Hepatic venous outflow obstruction: Three similar syndromes. World J Gastroenterol 2007; 13(13): 1912-1927 [PMID: 17461490 DOI: 10.3748/wjg.v13.i13.1912]
Corresponding Author of This Article
Ulas Darda Bayraktar, MD, Department of Internal Medicine, Interfaith Medical Center, 229 Parkville Ave Apt# 4B, Brooklyn, NY 11230, United States. bayraktarulas@yahoo.com
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World J Gastroenterol. Apr 7, 2007; 13(13): 1912-1927 Published online Apr 7, 2007. doi: 10.3748/wjg.v13.i13.1912
Table 1 Definition, etiology and histology of VOD, BCS, and CH
VOD
BCS
CH
Site of venous obstruction
Hepatic sinusoids and terminal venules
From hepatic veins to the superior end of IVC
Heart
Etiology
Sinusoidal endothelial injury due to HSCT, chemotherapy, abdominal radiotherapy, and pyrrolizidine alkaloids
Hepatic vein thrombosis, IVC webs, compression of hepatic veins or IVC by tumor, cyst, or abscess
Increased right atrial pressure due to CHF (CAD, cardiomyopathies, valve abnormalities), cor pulmonale (COPD, ILD, pulmonary HTN), and pericardial disease (constrictive pericarditis, pericardial tamponade)
Histology
Changes predominantly in perivenular areas
Predominantly in perivenular areas except in presence of concomitant PVT.
Predominantly in perivenular areas Sinusoidal congestion and hepatocellular necrosis
Gaps in SEC barrier leading to subendothelial edema
Sinusoidal congestion followed by ischemic cell necrosis and bridging fibrosis between central veins
Bridging fibrosis between central veins leading to cardiac fibrosis in chronic cases
Narrowing of central veins and sinusoids with sinusoidal congestion and hepatocellular necrosis
Caudate lobe hypertrophy, with fibrosis and atrophy in the rest of liver
Collagen accumulation in sinusoids and veins leading to bridging fibrosis between central veins
Table 2 Radiological findings, treatment, and prognosis in VOD, BCS, and CH
VOD
BCS
CH
Radiological findings
Ultrasonography to rule out other liver disorders
Doppler: Abnormal flow in a hepatic vein; large intrahepatic collateral vessels; e nlarged, stenotic, or tortuous hepatic veins
Dilatation of all three hepatic veins on sonogram
Doppler may show reverse blood flow in the portal vein
MRI: Large intrahepatic comma shaped c ollaterals. Hepatic venography: Spider web venous network pattern
ECHO: Increased pulmonary artery pressure, dilatation of right side of heart, TR, abnormal diastolic ventricular filling due to pericardial disease
Treatment
(1) Prevention: UDCA, heparin, LMWH, and defibrotide
(1) Prevention of thrombus extension: Anticoagulation with heparin and warfarin
Treatment of the underlying heart disease
(2) Treatment: Symptomatic care, defibrotide, tPA, AT-III concentrate
(2) Restoration of blood flow: Thrombolytic therapy, percutaneous, angioplasty, TIPS, or shunt surgery
Pericardiectomy in constrictive pericarditis
(3)TIPS and liver transplantation in selected cases
(3) Liver transplantation
Prognosis
Mortality rate between 9% to 98% depending on the severity
Five-year survival rate 42% to 89% in hepatic vein thrombosis and 25% in IVC obstruction
Liver disease rarely contributes to mortality in these patients
Table 3 Diagnostic criteria for veno-occlusive disease
Seattle criteria
Development of at least 2 of the following 3 clinical features before d 30 after transplantation
Jaundice
Hepatomegaly with right upper quadrant pain
Ascites and/or unexplained weight gain
Baltimore criteria
Development of hyperbilirubinemia with serum bilirubin > 2 mg/dL within 21 d after transplantation and at least 2 of the following clinical signs and symptoms
Hepatomegaly, which may be painful
Weight gain > 5% from baseline
Ascites
Table 4 Differential diagnosis of veno-occlusive disease
Citation: Bayraktar UD, Seren S, Bayraktar Y. Hepatic venous outflow obstruction: Three similar syndromes. World J Gastroenterol 2007; 13(13): 1912-1927