0). The remaining 131 patients were followed until death (n = 36; 23%; median time to death: 10 months [range, 0.1-41.0]) or study closure (n = 95; 61%; median follow-up: 57 months [range, 43-74]). Table 1 describes the baseline characteristics.

Median age at diagnosis of BSC was 37 years (range, 16-83), and 90 patients (57.3%) were female. Supporting Table 1 describes the etiology for the total study population. With reference to the Sirolimus original EN-Vie study, we found additional causal factors in 12 patients: myeloproliferative neoplasms in 7; celiac disease in 2; and antiphospholipid syndrome, factor V Leiden mutation, and hyperhomocysteinemia in 1 each. One hundred and thirty-nine patients (88.5%) received long-term anticoagulation. Twenty-eight bleeding complications occurred in 24 patients (17%) during the study. Main causes of bleeding were portal hypertension (PH) related (n = 14; 2 died), intracranial hemorrhage (n = 3; 1 died), and abdominal wall bleeding (n = 2), genital bleeding (n = 2), bronchial bleeding (n = 1), and peptic ulcer (n = 1; all alive). Figure JQ1 1 shows the flowchart of treatments received by patients. Twenty-two patients underwent angioplasty (n = 13), thrombolysis (n = 7), or both (n = 2) as first invasive treatment. In 6 of these 22 patients, a vascular stent was placed at the time of angioplasty. After this initial intervention, 14 patients (64%) required further treatment with either TIPS (N = 12) or

OLT (N = 2) after a median time of 1.5 months (range, 0.2-19.0) (Fig. 1). The remaining 8 patients were only treated with angioplasty/thrombolysis (in 2 patients more than

once). Seven of them are alive and free 上海皓元 of ascites with a median follow-up of 47 months (range, 32-61), but 1 died 6 months later as a result of liver failure. Sixty-two patients underwent TIPS (39.5%). Main indications were refractory ascites (69%), liver failure (13%), and variceal bleeding (7%). Four of these (6.45%) had rescue OLT a median of 1.8 months after TIPS (range, 0.03-13.0) for the following reasons: HE (n = 1); fulminant liver failure (N = 1); and TIPS thrombosis with refractory ascites (N = 2). Three of these four patients died a median of 35 months after OLT (range, 7-45) as a result of liver failure (N = 2) and extrahepatic malignancy (N = 1). Of the remaining 58 patients, 10 (17%) died within 5.8 months (range, 0.2-39) and 48 (83%) were alive after a median follow-up of 51 months (range, 0.3-69.0). Thus, overall, 13 patients died, 9 of them resulting from a liver-related cause. One, 3-, and 5-year actuarial survival and OLT-free survival of patients treated with TIPS was 88%, 83%, and 72% and 85%, 78%, and 72%, respectively (Fig. 2). Similar results were found if deaths clearly unrelated to liver disease were removed from the analysis or considering the date of TIPS as time zero (data not shown). Median time from diagnosis to TIPS was 1 month (range, 0-38).