Bleeding in patients with Budd-Chiari syndrome.
OBJECTIVE
Anticoagulation therapy is recommended for patients with Budd-Chiari syndrome (BCS). This study aimed to assess the incidence, severity, and risk factors of major bleeding in patients with Budd-Chiari syndrome (BCS) receiving anticoagulation therapy.


METHODS
We evaluated 94 consecutive BCS patients. Major bleeding required hospitalization, and/or transfusion of ≥ 2 red blood cell units, and/or was located intracranially, and/or retroperitoneally, and/or was fatal.


RESULTS
After a median follow-up of 43 months, 47 patients had 92 major bleeding episodes (22.8 per 100 patient-years). Forty episodes were related to invasive therapy for BCS. The origin of the 52 other episodes was gastrointestinal in 26 (including 15 related to portal hypertension) and genital in 10; 26 were spontaneous and 26 provoked. Excess anticoagulation was identified in 13 (27%) out of 49 documented episodes. Bleeding was managed by interrupting or reducing anticoagulation in 34 episodes, surgery in 18, endoscopy in 12, and radiological intervention in 8. The presence of esophageal varices was an independent predictor of bleeding unrelated to invasive therapy for BCS. Bleeding contributed to death in five patients and caused neurological complications in two. These poor outcomes were associated with more severe liver disease at baseline.


CONCLUSIONS
Major bleeding is common in BCS patients receiving anticoagulation therapy. Invasive procedures and portal hypertension are major factors, while excess anticoagulation plays a secondary role. Baseline BCS severity is the main determinant of prognosis at bleeding. Reducing anticoagulation intensity during invasive therapy and reinforced prophylaxis for portal hypertension could improve the benefit-risk ratio of anticoagulation.
Rautou
Pierre-Emmanuel
PE
Pôle des Maladies de l'Appareil Digestif, Service d'Hépatologie, Hôpital Beaujon, AP-HP, Clichy, France.
Douarin
Ludivine
L
Denninger
Marie-Hélène
MH
Escolano
Sylvie
S
Lebrec
Didier
D
Moreau
Richard
R
Vidaud
Michel
M
Itzykson
Raphaël
R
Moucari
Rami
R
Bezeaud
Annie
A
Valla
Dominique
D
Plessier
Aurélie
A
D000328:Adult; D000925:Anticoagulants / Q000009:adverse effects*; D006502:Budd-Chiari Syndrome / Q000150:complications* / Q000188:drug therapy* / Q000628:therapy; D004932:Esophageal and Gastric Varices / Q000150:complications; D005260:Female; D006471:Gastrointestinal Hemorrhage / Q000209:etiology; D006470:Hemorrhage / Q000209:etiology*; D006801:Humans; D006975:Hypertension, Portal / Q000150:complications; D016031:Liver Transplantation / Q000009:adverse effects; D008297:Male; D008875:Middle Aged; D019106:Postoperative Hemorrhage / Q000209:etiology; D011379:Prognosis; D012307:Risk Factors; D016896:Treatment Outcome
D000925:Anticoagulants