Long-term Outcome of Budd-Chiari Syndrome: A Single Center Experience
Neda Nozari1, Hassan Vossoghinia2, Fatemeh Malekzadeh1
1Digestive Diseases Research Center, Shariati Hospital, Tehran University of Medical Sciences, Tehran Iran.
Insights
Budd-Chiari syndrome (BCS), a hepatic venous outflow obstruction, is a serious liver condition. Anticoagulant therapy is effective, but long-term prognosis remains guarded, with many patients requiring liver transplant or succumbing to end-stage liver disease.
Area of Science:
- Hepatology
- Vascular Medicine
- Internal Medicine
Background:
- Budd-Chiari syndrome (BCS) is a rare and life-threatening liver disorder characterized by hepatic venous outflow obstruction (HVOO).
- This study focuses on the clinical presentation, etiological factors, and long-term outcomes of BCS in Iran.
Purpose of the Study:
- To describe the clinical and etiological characteristics of Budd-Chiari syndrome.
- To evaluate the long-term outcomes of BCS patients treated at a referral center.
Main Methods:
- Retrospective review of 55 patients diagnosed with BCS between 1989 and 2012 at Shariati Hospital, Tehran.
- Diagnosis confirmed by at least two imaging techniques.
- Comprehensive analysis of clinical, paraclinical, etiological data, and long-term outcomes.
Main Results:
- Congenital thrombophilia (50%) was the leading etiology, including protein C deficiency, protein S deficiency, antithrombin deficiency, and factor V Leiden mutation.
- Common clinical presentations included abdominal pain (60%), abdominal distention (38.2%), and jaundice (18%). Ascites (76.4%) was the most frequent sign.
- During the 22-year follow-up, 30% of patients died. 40% required liver transplantation or died from end-stage liver disease.
Conclusions:
- Budd-Chiari syndrome presents a significant challenge in Iran, despite its rarity.
- Anticoagulation and supportive care are effective medical therapies, but prognosis is guarded.
- Long-term management requires careful monitoring, with a substantial proportion of patients needing advanced interventions like liver transplantation.
Background:
Budd-Chiari syndrome (BCS) is defined as hepatic venous outflow obstruction(HVOO). BCS is an uncommon, life-threatening liver disorder. This studydescribes the clinical and etiological characteristics in addition to the longtermoutcome of BCS in a single referral center in Tehran, Iran.
Methods:
We reviewed long-term outcome of patients who were diagnosed with BCSbetween 1989 and 2012 at Shariati Hospital, a tertiary hospital affiliated withTehran University of Medical Sciences, Tehran, Iran. The diagnosis was confirmedby at least two imaging techniques. A comprehensive analysis of theclinical and paraclinical manifestations, etiology and long-term outcome of thedisease was conducted.
Results:
Seventy one patients (43 female) with a diagnosis of Budd-Chiari syndromewere identified during the 22 year period of study. The age were ranged from17 to 64 years (median: 29 years). We excluded 16 patients because of incompleteinformation or follow up. The remaining 55 cases were the subjects ofthis study. Underlying etiologies consisted of congenital thrombophilia factorsin 50% (28 cases) which was defined as protein C deficiency (12 cases),protein S deficiency (3 cases), antithrombin deficiency (3 cases) and factor VLeiden mutation (10 cases). Etiology was unknown in 18% (10 cases). Acquiredcauses of thrombophilia were observed in 25% (14 cases) that consistedof 9 cases of myeloproliferative disease and 5 cases of autoimmune diseases.In 3 cases pregnancy was the only etiology. The main clinical presentationswere abdominal pain in 33 (60%), abdominal distention in 21 (38.2%), andjaundice in 10 (18%) cases. The main clinical signs were ascites (76.4%), splenomegaly(34%), hepatomegaly (25.5%) and deep vein thrombosis (1.8%). All55 patients were treated with anticoagulants (heparin followed by warfarin)and supportive care. Two cases underwent mesocaval shunt surgery, 2 patientsrequired transjugular portosystemic shunt (TIPS) and 5 were referred for livertransplantation. A total of 17 (30%) patients died during 22 years of follow up.
Conclusion:
BCS, although uncommon in Iran, is a challenging liver disease with an importantburden. Medical therapy that includes anticoagulation seems to be effectivein most cases although the prognosis is guarded. In long-term followup, 40% of cases will need liver transplant or die from end stage liver disease.
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