TY - JOUR
T1 - TIPS for Budd-Chiari Syndrome
T2 - Long-Term Results and Prognostics Factors in 124 Patients
AU - Garcia-Pagán, Juan Carlos
AU - Heydtmann, Mathis
AU - Raffa, Sebastian
AU - Plessier, Aurélie
AU - Murad, Sarwa
AU - Fabris, Federica
AU - Vizzini, Giovanni
AU - Abraldes, Juan Gonzales
AU - Olliff, Simon
AU - Nicolini, Antonio
AU - Luca, Angelo
AU - Primignani, Massimo
AU - Janssen, Harry L A
AU - Valla, Dominique
AU - Elias, Elwyn
AU - Bosch, Jaume
PY - 2008/9
Y1 - 2008/9
N2 - Background & Aims: Budd-Chiari syndrome (BCS) is a rare and life-threatening disorder secondary to hepatic venous outflow obstruction. Small series of BCS patients indicate that transjugular intrahepatic portosystemic shunt (TIPS) may be useful. However, the influence of TIPS on patient survival and factors that predict the outcome of TIPS in BCS patients remain unknown. Methods: One hundred twenty-four consecutive BCS patients treated with TIPS in 6 European centers between July 1993 and March 2006 were followed until death, orthotopic liver transplantation (OLT), or last clinical evaluation. Results: Prior to treatment with TIPS, BCS patients had a high Model of End Stage Liver Disease and high Rotterdam BCS prognostic index (98% of patients at intermediate or high risk) indicating severity of liver dysfunction. However, 1- and 5-year OLT-free survival were 88% and 78%, respectively. In the high-risk patients, 5-year OLT-free survival was much better than that estimated by the Rotterdam BCS index (71% vs 42%, respectively). In the whole population, bilirubin, age, and international normalized ratio for prothrombin time independently predicted 1-year OLT-free survival. A prognostic score with a good discriminative capacity (area under the curve, 0.86) was developed from these variables. Seven out of 8 patients with a score >7 died or underwent transplantation vs 5 out of 114 patients with a score
AB - Background & Aims: Budd-Chiari syndrome (BCS) is a rare and life-threatening disorder secondary to hepatic venous outflow obstruction. Small series of BCS patients indicate that transjugular intrahepatic portosystemic shunt (TIPS) may be useful. However, the influence of TIPS on patient survival and factors that predict the outcome of TIPS in BCS patients remain unknown. Methods: One hundred twenty-four consecutive BCS patients treated with TIPS in 6 European centers between July 1993 and March 2006 were followed until death, orthotopic liver transplantation (OLT), or last clinical evaluation. Results: Prior to treatment with TIPS, BCS patients had a high Model of End Stage Liver Disease and high Rotterdam BCS prognostic index (98% of patients at intermediate or high risk) indicating severity of liver dysfunction. However, 1- and 5-year OLT-free survival were 88% and 78%, respectively. In the high-risk patients, 5-year OLT-free survival was much better than that estimated by the Rotterdam BCS index (71% vs 42%, respectively). In the whole population, bilirubin, age, and international normalized ratio for prothrombin time independently predicted 1-year OLT-free survival. A prognostic score with a good discriminative capacity (area under the curve, 0.86) was developed from these variables. Seven out of 8 patients with a score >7 died or underwent transplantation vs 5 out of 114 patients with a score
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U2 - 10.1053/j.gastro.2008.05.051
DO - 10.1053/j.gastro.2008.05.051
M3 - Article
C2 - 18621047
AN - SCOPUS:51449107456
VL - 135
SP - 808
EP - 815
JO - Gastroenterology
JF - Gastroenterology
SN - 0016-5085
IS - 3
ER -