Prognosis

Untreated Budd-Chiari syndrome (BCS) has a reported mortality rate of 80%.[13] However, the prognosis has dramatically improved in the past few decades, due to advances in the diagnosis and treatment of BCS.[78]​ With appropriate management, survival at 5 years now exceeds 80%.[79]​

Spontaneous resolution has been occasionally reported, and up to 25% of patients remain asymptomatic.[15][80]

Determinants of survival

Encephalopathy, ascites, prothrombin time, and bilirubin are independent determinants of survival. A prognostic classification combining these factors identified 3 classes of patients (classes I-III):[9]

  • Ascites and hepatic encephalopathy are scored as present (1) or absent (0)

  • Prothrombin time as higher (1) or lower (0) than 2.3 INR

  • Bilirubin is included as a continuous variable for which the risk increased with 0.004 per micromol/L.

Class I represented a total score between 0 and 1.1, class II between 1.1 and 1.5, and class III a total score of 1.5 and higher.

The 5-year survival rate was 89% for class I (good), 74% for class II (intermediate), and 42% for class III (poor).

Associated portal vein thrombosis (PVT)

PVT has been associated with poor prognosis in patients with BCS.[81][82] The mean survival rate is 1 month in patients with PVT compared to 6.3 years in those without PVT.[81]

Paroxysmal nocturnal hemoglobinuria (PNH)

Patients with BCS and underlying PNH were found to have high mortality.[83][84] The cause for this is not known.

Postspecific therapies

Medical therapy

  • One retrospective population-based study from the US found no difference in mortality between patients managed medically with early initiation of anticoagulation, and those who underwent interventional or surgical procedures.[78]

  • Treating the cause of the disease, particularly for myeloproliferative neoplasms and paroxysmal nocturnal hemoglobinuria, has a better outcome than anticoagulation alone.[79]

Hepatic veins and/or inferior vena cava (IVC) angioplasty

  • If IVC or hepatic vein obstruction dominates, as in Asian countries, angioplasty is successful in over 70% of patients.[79]

Transjugular intrahepatic portosystemic shunts (TIPS)

  • One-, 5-, and 10-year survival rates post-TIPS were 90%, 84%, and 80% respectively.[85]

  • Reintervention procedure in the form of restenting or dilation is reported in 36% to 72% of patients who underwent TIPS.[86]

Surgical shunting

  • Five-year survival rate after surgical shunting ranges between 75% and 94% with higher range in patients with patent IVC.[86]

  • Better surgical outcomes are seen in patients with Child-Pugh class A and with an underlying cause that has a favorable long-term outcome, such as essential thrombocythemia.[42]

Liver transplant

  • Five-year survival rates of patients with BCS undergoing liver transplantation range from 50% to 95%.[87] In one 2005 European cohort, the 5-year survival was 71.4% with orthotopic liver transplantation.[88]

  • Postoperative complications of liver transplantation include portal vein and hepatic artery thrombosis, which occur in 12% of patients.[89] Bleeding complications related to anticoagulant therapy are reported in 40% of patients.[90]

  • BCS was reported to recur in 2 out of 7 transplant recipients despite anticoagulant therapy.[90]

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