Gastrointestinal Bleeding in Patients with Budd-Chiari Syndrome: a Prospective Evaluation
Jildou Hoekstra1, Sarwa Darwish Murad1, Aurelie Plessier2, Manuel Hernandez-Guerra3, Elwyn
Elias4, Massimo Primignani5, Matthias J. Bahr6, Joerg Heller7, Antoine Hadengue8, Philippe
Langlet9, Helena Miranda10, Juan-Carlos Garcia-Pagan3, Dominique-Charles Valla2, Harry L.A.
Janssen1 for the European Network for Vascular Disorders of the Liver (EN-Vie); 1 Department
of Gastroenterology and Hepatology, Erasmus University Medical Center, Rotterdam, The
Netherlands; 2 Department of Hepatology, Hopital Beaujon, AP-HP, INSERM-U773 & University
Paris-7, Clichy, France; 3 Hepatic Hemodynamic Laboratory, Liver Unit, Hospital Clinic, IDIBAPS
and Ciberehd, Barcelona, Spain; 4 Liver Unit, Queen Elisabeth Hospital Birmingham, Birmingham,
United Kingdom; 5 Gastroenterology and Gastrointestinal Endoscopy Unit, Ospedale Policlinico,
Mangiagalli and Regina Elena Foundation, Milan, Italy; 6 Department of Gastroenterology,
Hepatology, and Endocrinology, Hannover Medical School, Hannover, Germany; 7 Department of
Internal Medicine I, University Hospital of Bonn, Bonn, Germany; 8 Division of Gastroenterology and
Hepatology, Geneva University Hospitals, Geneva, Switzerland; 9 Department of
Hepatogastroenterology, Centre Hospitalier Universitaire Brugmann, Bruxelles, Belgium; 10 Liver
Transplantation Unit, Hospital General Santo Antonio, Porto, Portugal.
Obstruction of the hepatic veins or inferior vena cava, defined as Budd-Chiari Syndrome (BCS), often
leads to portal hypertension. Bleeding from gastroesophageal varices is an important complication of
portal hypertension. However, little is known about the characteristics of gastrointestinal (GI) bleeding
in patients with BCS. Therefore, the aim of this study was to evaluate the type, frequency and outcome
of GI-bleeding in these patients. We studied 163 BCS-patients that were prospectively included in the
EN-Vie Study, a multi-center European study. At baseline and during follow-up, data was collected on
clinical characteristics, episodes of GI-bleeding, complications and survival. Median age at diagnosis
of BCS was 38 years (range 16-83), 43% of the patients was male and median follow-up period was
17 months (range 0.1-31). During the study period, a total of 40 bleeding episodes occurred in 24
patients (15%). In 8 patients (5%), GI-bleeding was present at diagnosis. Of the other patients, a first
bleeding event occurred within the first year of follow-up in 12 patients and after 1 year in 4 patients.
Nine patients (38%) experienced recurrent bleeding, of which five patients had more than two events.
Compared to patients without bleeding (n=139), patients that experienced an episode of bleeding were
more often males (63% vs. 40%, p=0.036) and had a higher frequency of concurrent portal vein
thrombosis (32% vs. 14%, p=0.042). Of all events, 50% was caused by bleeding from
gastroesophageal varices or portal hypertensive gastropathy. Other causes of bleeding were ischemic
enterocolitis, ulcerative colitis and gastric antral vascular ectasia. All patients that bled from
esophageal varices (n=9) were treated with endoscopic band ligation or sclerotherapy. In one of these
patients, failure to control bleeding was an indication for a transjugular intrahepatic portosystemic
shunt (TIPS). Two patients (8%) died as a result of GI-bleeding. Baseline Rotterdam prognostic score
did not differ significantly between patients with and without GI-bleeding (1.29 vs. 1.26, p=0.868).
Conclusions. GI-bleeding occurs as a presenting symptom in 5% of BCS-patients and in another 10%
during short-term follow-up. Patients with additional portal vein thrombosis appear to be more prone
to bleeding complications. Most cases of bleeding in BCS are related to portal hypertension but other
causes should also be explored, especially since many patients will use anticoagulation.