Khuroo Mohammad Sultan, Al-Suhabani Hamad, Al-Sebayel Mohammad, Al Ashgar Hamad, Dahab Saleim, Khan Mohammad Qaseem, Khalaf Hatem Ali
Section of Gastroenterology, Department of Medicine, King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia.
J Gastroenterol Hepatol. 2005 Oct;20(10):1494-502. doi: 10.1111/j.1440-1746.2005.03878.x.
The long-term outcome of Budd-Chiari syndrome (BCS) with transjugular intrahepatic portosystemic shunts (TIPS) is not well studied. To address this, the records of 47 consecutive patients with BCS evaluated in one center from January 1989 to April 2004, were analyzed.
Seven patients with liver tumors were excluded from analyses. Eleven patients had Bechet's disease, 14 had thrombophiliac disorders, four had myeloproliferative diseases and 11 patients had other or unknown causes. The site of block was hepatic vein in 16 patients, in the suprhepatic inferior vena cava in 19 and not known in five. The majority of patients (21/40; 52.5%) presented with subacute disease with massive ascites and abdominal pain as the dominant manifestations. Eight patients with membranes or segemental block were treated with transluminal angiopalsty, and six were treated with clinical and biochemical recovery. The TIPS was placed through a transcaval puncture in eight patients with progressive liver disease who were on medical therapy and had thrombosis limited to hepatic veins. One patient bled from portal vein puncture, which was managed by placing stent across the punctured site. The TIPS was very effective in decreasing portal pressure gradient, improving synthetic functions, reducing transaminase levels and controlling ascites. Five patients had TIPS dysfunction needing revision. In two patients it was difficult to maintain TIPS patency due to repeated TIPS dysfunction. However, both these patients were asymptomatic with normal liver function tests. Long-term follow up revealed that patients with TIPS had significantly better survival than those treated with medical therapy alone (log-rank test, P = 0.04). In a multivariate Cox-model analysis four variables, namely, more florid presentation, male sex, no treatment with TIPS and increasing Child-Pugh-Turcotte score, adversely affected the survival.
Budd-Chiari syndrome needs an individualized multidisciplinary approach and TIPS is indicated in a subgroup of patients with progressive liver disease. It is safe, feasible and improves survival.
布加综合征（BCS）行经颈静脉肝内门体分流术（TIPS）的长期疗效尚未得到充分研究。为解决这一问题，我们分析了1989年1月至2004年4月在一个中心连续评估的47例BCS患者的记录。
7例肝脏肿瘤患者被排除在分析之外。11例患者患有白塞病，14例有血栓形成倾向疾病，4例有骨髓增殖性疾病，11例患者病因不明或为其他原因。阻塞部位为肝静脉的有16例，肝上下腔静脉的有19例，5例不明。大多数患者（21/40；52.5%）表现为亚急性疾病，以大量腹水和腹痛为主要表现。8例膜性或节段性阻塞患者接受了腔内血管成形术治疗，6例患者临床和生化指标恢复。8例接受药物治疗且血栓仅限于肝静脉的进行性肝病患者通过经腔静脉穿刺放置了TIPS。1例患者门静脉穿刺出血，通过在穿刺部位放置支架进行处理。TIPS在降低门静脉压力梯度、改善肝功能、降低转氨酶水平和控制腹水方面非常有效。5例患者出现TIPS功能障碍需要修复。2例患者由于TIPS反复功能障碍难以维持其通畅性。然而，这2例患者均无症状，肝功能检查正常。长期随访显示，接受TIPS治疗的患者生存率明显高于单纯接受药物治疗的患者（对数秩检验，P = 0.04）。在多变量Cox模型分析中，四个变量，即病情更严重、男性、未接受TIPS治疗和Child-Pugh-Turcotte评分增加，对生存率有不利影响。
布加综合征需要个体化的多学科方法，TIPS适用于一部分进行性肝病患者。它安全、可行且能提高生存率。
