Study of portal vein thrombosis in patients with idiopathic portal hypertension in Japan.

BACKGROUND/AIMS: The aim of this study was to elucidate the incidence and clinical manifestations of portal vein thrombosis (PVT) in patients with idiopathic portal hypertension (IPH) in Japan during long-term follow-up. PATIENTS AND METHODS: Twenty-two patients with IPH were examined for PVT by sonography during a follow-up of 12+/-6 years. Clinical manifestations and patient outcome related to PVT were studied. Seventy patients with liver cirrhosis were examined by sonography as an incidence control of thrombosis. RESULTS: Nine IPH patients had portal thrombosis (9/22, 41%), a higher incidence than in liver cirrhosis patients (7/70, 10%). Those with thrombosis showed ascites, marked hypersplenism, and low serum albumin. Four patients with thrombosis died. Patients without thrombosis showed less clinical problems after long-term follow-up. Plasma antithrombin III and protein C activity decreased in almost half of the patients. However, there were no differences in these parameters between patients with and without thrombosis. CONCLUSIONS: In Japan, IPH patients had a high incidence of portal thrombosis, a significant factor for poor prognosis. Whether the management of PVT contributes to an improvement of a clinical course of IPH or not should be clarified in further study.     

经颈静脉肝内门体静脉分流术治疗肝硬化门静脉高压共识意见

<正>门静脉高压症是肝硬化发展过程中的重要病理生理环节,也是肝硬化失代偿期的重要临床表现之一。经颈静脉肝内门体静脉分流术(transjugular intrahepatic portosystemic shunt,TIPS)通过在肝静脉与门静脉之间的肝实质内建立分流道,以微创的方式,从结构上显著降低门静脉阻力,是降低肝硬化患者门静脉压力的关键措施之一。选择恰当病例,可有效减少食管胃静脉曲张再出血和腹水复发等肝硬化并发症,改善肝硬化患者生活质量,减少或延缓对肝移植的需求[1-3]。TIPS应用于临床已有20余年,在经历了一系列观念、技术、器材和联合药物治疗的探索后,目前该技术的有效性和安     

Influence of different portal vein branches on hepatic encephalopathy during intrahepatic portal shunt via jugular vein

This letter is regarding the study titled ‘Targeted puncture of left branch of intrahepatic portal vein in transjugular intrahepatic portosystemic shunt (TIPS) to reduce hepatic encephalopathy’. Prior to the approval of TIPS dedicated stents (Viatorr stents) in China in October 2015, Fluency covered stents were typically used. As Fluency covered stents have a strong support force and axial elastic tension, a ‘cap’ may form if the stent is located too low at the end of the hepatic vein or too short at the end of the portal vein during surgery, leading to stent dysfunction. Since the blood shunted by the stent is from the main trunk of the portal vein, the correlation between the incidence of postoperative hepatic encephalopathy and the location of the puncture target (left or right portal vein branch) is worth discussion. Notably, no studies in China or foreign countries have proven the occurrence of left and right blood stratification after the accumulation of splenic vein and mesenteric blood flow in the main trunk of the portal vein in patients with cirrhotic portal hypertension.     

Effects of transjugular intrahepatic portosystemic shunt treatment of patients with liver cirrhosis and portal hypertension: Case series

Rationale:Transjugular intrahepatic portosystemic shunt (TIPS) is well established as an effective treatment tool for portal hypertension. However, the effects of TIPS in patients with liver cirrhosis and portal hypertension have not been adequately verified in clinical trials.Patient Concerns:To evaluate the effects of TIPS in patients with liver cirrhosis and portal hypertension with or without portal vein thrombosis (PVT).Interventions:A total of 55 patients with liver cirrhosis and portal hypertension received TIPS treatment from December 2014 to April 2018 were enrolled. Clinical data, including portal pressure, Child-Pugh score, and relevant complications were recorded.Outcomes:TIPS was successfully performed in 54 patients. The overall technical success rate was 98.19% without serious technical complications. After TIPS treatment, portal pressure was significantly reduced from 38.13 ± 4.00 cmH₂O to 24.14 ± 3.84 cmH₂O (P < 0.05). In addition, symptoms including gastrointestinal bleeding and ascites were improved after TIPS treatment. During the 6 to 21-month follow up, hepatic encephalopathy in 15 patients (27.8%), shunt dysfunction in 5 patients (9.3%), rebleeding in 12 patients (22.2%) and deterioration of liver function in 2 patients (3.7%) were recorded. Moreover, there were no significant differences in the rates of rebleeding and hepatic encephalopathy between patients with PVT and the non-PVT group, whereas the occurrence rate of TIPS dysfunction was higher in the PVT group, but not statistically significant.Lessons:TIPS treatment could alleviate the symptoms of liver cirrhosis and portal hypertension in individuals with or without PVT. However, complications during follow-up should be appropriately noted and addressed with corresponding treatments.     

Aneurysmal dilatation of the portal vein: A rare cause of portal hypertension

Aneurysmal dilatation of the portal vein (ADPV) is a rare cause of portal hypertension. We described a case of ADPV in a female patient who presented with ascites. Imaging studies revealed tortuosity and dilatation of the main portal vein with turbulent flow. Endoscopy revealed oesophageal varices. A liver biospy showed no abnormalities in liver histology. This is the first case of ascites as a complication of ADPV in the absence of liver cirrhosis, arteriovenous fistula or documented portal vein thrombosis. Hyperdynamic circulation and increased portal vein flow could be implicated in the pathogenesis of ascites in this setting.     

经颈静脉肝内门体分流术治疗原发性胆汁性肝硬化门静脉高压的效果及安全性

目的探讨经颈静脉肝内门体分流术(TIPS)应用于失代偿期原发性胆汁性肝硬化(PBC)患者的短期疗效及安全性。方法对2009年1月-2015年5月于南京大学医学院附属鼓楼医院行TIPS治疗的26例失代偿期PBC患者的临床数据进行回顾性分析。对比术前术后门静脉压力、肝功能、生存率等指标及术后1年的随访情况。用Friedman检验进行多个相关样本的秩和检验,Wilcoxon检验进行配对秩和检验,对整个随访期内患者生存率作Kaplan-Meier分析曲线。结果 26例患者术后平均门静脉压力为19.11(16.35~22.05)mm Hg,较术前27.93(25.26~30.87)mm Hg显著下降,差异具有统计学意义(Z=-4.199,P0.001)。术后3个月内TBil、终末期肝病模型评分、胆汁酸等较术前明显上升(χ2值分别为26.000、18.429、16.353,P值均0.001)。术后最常见并发症为发热,发生率为80.77%(21/26),其次为肝性脑病,发生率为19.23%(7/26),术后6、12个月生存率均为92.3%(24/26)。结论 TIPS治疗失代偿期PBC患者短中期疗效及安全性较好,长期疗效尚需进一步观察。     

经颈静脉肝内门体分流术研究进展及其在肝硬化门静脉高压治疗中的应用

近年来,随着基础研究及临床应用的进步,经颈静脉肝内门体分流术(TIPS)的成功率明显提高,支架再狭窄率及术后肝性脑病的发生率亦得到控制。结合笔者临床实践及国内外大会热点,就TIPS相关技术进展及其在肝硬化中的应用进行介绍,主要包括TIPS穿刺辅助技术、TIPS支架、TIPS相关肝性脑病及防治以及TIPS适应证和禁忌证、TIPS在肝硬化中解决的问题、肝硬化食管胃底静脉曲张破裂出血治疗中TIPS相对于其他治疗方法的优缺点。以期为TIPS更好的发展作一点贡献,让更多肝硬化患者获益。     

非肝硬化性门静脉高压症的研究现状

肝硬化是门静脉高压的最常见原因,但仍有约20%的门静脉高压继发于非肝硬化因素,称为非肝硬化性门静脉高压症(NCPH),在发展中国家发病率较高。NCPH是一组异源性的肝脏血管疾病,临床上多见的是特发性门静脉高压(IPH)、肝外门静脉血管阻塞(EHPVO),以及布加综合征、先天性肝纤维化和结节再生性增生等少见病。此类患者常常具有门静脉高压的证据,如反复发生的静脉曲张出血和脾脏肿大,但肝功能保存尚好。目前尚无诊断NCPH的统一标准,对其诊断仍是一个挑战。临床上往往采用排除性诊断,必要时可行肝穿刺活组织检查来确诊。介绍了IPH和EHPVO的发病机制、病理表现、诊断方法及治疗策略的选择,若能有效控制上消化道出血,NCPH被认为是预后相对良好的一类疾病。     

Transjugular intrahepatic portosystemic shunt with covered stents for hepatocellular carcinoma with portal vein tumor thrombosis

AIM: To evaluate transjugular intrahepatic portosystemic shunt(TIPS) with covered stents for hepatocellular carcinoma(HCC) with main portal vein tumor thrombus(PVTT). METHODS: Eleven advanced HCC patients(all male, aged 37-78 years, mean: 54.3 ± 12.7 years) presented with acute massive upper gastrointestinal bleeding(n = 9) or refractory ascites(n = 2) due to tumor thrombus in the main portal vein. The diagnosis of PVTT was based on contrast-enhanced computed tomography and color Doppler sonography. The patients underwent TIPS with covered stents. Clinical characteristics and average survival time of 11 patients were analyzed. Portal vein pressure was assessed before and after TIPS. The follow-up period was 2-18 mo. RESULTS: TIPS with covered stents was successfully completed in all 11 patients. The mean portal vein pressure was reduced from 32.0 to 11.8 mmHg(t = 10.756, P = 0.000). Gastrointestinal bleeding was stopped in nine patients. Refractory ascites completely disappeared in one patient and was alleviated in another. Hepatic encephalopathy was observed in six patients and was resolved with drug therapy. During the follow-up, ultrasound indicated the patency of the shunt and there was no recurrence of symptoms. Death occurred 2-14 mo(mean: 5.67 mo) after TIPS in nine cases, which were all due to multiple organ failure. In the remaining two cases, the patients were still alive at the 16- and 18-mo follow-up, respectively. CONCLUSION: TIPS with covered stents for HCC patients with tumor thrombus in the main portal vein is technically feasible, and short-term efficacy is favorable.     

经颈静脉肝内门体分流术治疗门静脉高压症

经颈静脉肝内门体分流术(TIPS)是一项专门治疗门静脉高压症的介入治疗新技术.该技术于1969年首先报道,经过30多年的探索与发展,已日臻成熟,现已被广泛用于伴有食管胃底静脉曲张出血,顽固性腹水、Budd-Chiari综合征(BCS)等的门静脉高压症的治疗,并取得了显著疗效.     

Extrahepatic portal vein obstruction in adults detected by ultrasound with frequent lack of portal hypertension signs

Extrahepatic portal vein obstruction (EHPO) was seen in 54 adult patients at the Chiba University Hospital and affiliated hospitals from 1978 to 1991. They were classified according to the background disease (Group A, unknown aetiology; Group B, benign disease; Group C, malignant disease). Among the initial symptoms and signs, abdominal pain was the most frequent in Group A (37%), and symptoms attributable to the primary disease in Groups B (44%) and C (75%). Definite or probable diagnosis was made in 45 of the 54 patients (81.8%) by ultrasound (US) examination carried out because of these symptoms and signs. Signs of portal hypertension were observed in 67% of patients; oesophageal varices were seen in 60%. Extrahepatic portal vein obstruction without portal hypertension signs was characterized by thick extensive hepatopetal collaterals or patency of some intrahepatic portal veins. Extrahepatic portal vein obstruction patients without portal hypertension remained free of its signs for more than 3 years of follow up and, in fact, EHPO without portal hypertension signs was a common occurrence. Emphasis is made on the diagnostic value of US examination which was useful in identifying the relation of clinical manifestation of EHPO to pathophysiology, and on the frequent lack of portal hypertension signs in this disease.     

Clinical study of transjugular intrahepatic portosystemic shunt combined with AngioJet thrombectomy for acute portal vein thrombosis in non-cirrhosis

To evaluate the outcomes of the transjugular intrahepatic portosystemic shunt (TIPS) combined with AngioJet thrombectomy in patients with noncirrhotic acute portal vein (PV) thrombosis.Retrospective analysis from January 2014 to March 2017, 23 patients underwent TIPS combined with AngioJet thrombectomy for acute PV thrombosis in noncirrhosis. The rates of technical success, the patency of the PV, liver function changes, and complications were evaluated.Twenty-three patients underwent combined treatment, with a technical success rate of 100%. Twenty-four hours after treatment, PV thrombosis grade was improved significantly (P = .001). Before and after treatment, Albumin (gm/dl), aspartate transaminase (IU/l), alanine transaminase (IU/l), and platelets (10⁹/L) were all significantly improved (P < .05). Minor complications include hematoma, hematuria, and hepatic encephalopathy. After 1 week of treatment, computed tomography scan revealed 8.7% (2/23) cases of hepatic envelope hematoma (thickness less than 2 cm). Hemoglobinuria occurred in 18/23 (78.3%) patients after treatment and returned to normal within 1 to 2 days. Two patients 2/23 (8.7%) had transient grade I encephalopathy after TIPS. The 1-year overall survival rate was 100% (23/23). No major complications during treatment in all patientsAngioJet thrombectomy via TIPS has a favorable short-term effect in clearing thrombus and alleviating symptoms in diffuse acute PVT. The long-term efficacy of this treatment needs to be further studied.     

Targeted puncture of left branch of intrahepatic portal vein in transjugular intrahepatic portosystemic shunt to reduce hepatic encephalopathy

BACKGROUND Transjugular intrahepatic portosystemic shunt(TIPS) is currently used for the treatment of complications of portal hypertension. The incidence of hepatic encephalopathy(HE) remains a problem in TIPS placement. It has been reported that the right branch mainly receives superior mesenteric venous blood while the left branch mainly receives blood from the splenic vein. We hypothesized that targeted puncture of the left portal vein would divert the non-nutritive blood from the splenic vein into the TIPS shunt; therefore, targeted puncture of the left branch of the intrahepatic portal vein during TIPS may reduce the risk of HE.AIM To evaluate the influence of targeted puncture of left branch of portal vein in TIPS on HE.METHODS A retrospective analysis of 1244 patients with portal-hypertension-related complications of refractory ascites or variceal bleeding who underwent TIPS from January 2000 to January 2013 was performed. Patients were divided into group A(targeting left branch of portal vein, n = 937) and group B(targeting right branch of portal vein, n = 307). TIPS-related HE and clinical outcomes were analyzed.RESULTS The symptoms of ascites and variceal bleeding disappeared within a short time.By the endpoint of follow-up, recurrent bleeding and ascites did not differ significantly between groups A and B(P = 0.278, P = 0.561, respectively).Incidence of HE differed significantly between groups A and B at 1 mo(14.94% vs36.80%, χ~2 = 4.839, P = 0.028), 3 mo(12.48% vs 34.20%, χ~2 = 5.054, P = 0.025), 6 mo(10.03% vs 32.24%, χ~2 = 6.560, P = 0.010), 9 mo(9.17% vs 31.27%, χ~2 = 5.357, P =0.021), and 12 mo(8.21% vs 28.01, χ~2 = 3.848, P = 0.051). There were no significant differences between groups A and B at 3 years(6.61% vs 7.16%, χ~2 = 1.204, P =0.272) and 5 years(5.01% vs 6.18%, χ~2 = 0.072, P = 0.562). The total survival rate did not differ between groups A and B(χ~2 = 0.226, P = 0.634, log-rank test).CONCLUSION Targeted puncture of the left branch of the intrahepatic portal vein during TIPS may reduce the risk of HE but has no direct influence on prognosis of portalhypertension-related complications.     

Hepatic encephalopathy due to a large intrahepatic communication between the portal vein and inferior vena cava

A 59 year old man with cirrhosis presented with encephalopathy and hyper-ammonaemia. T1 weighted magnetic resonance imaging demonstrated a large void tubular structure connecting the right posterior portal vein branch and the inferior vena cava through the right hepatic lobe inferiorly, and cine-mode imaging showed a flow within this channel. Clearly in this patient a significant portion of the portal venous blood was being shunted into the inferior vena cava, causing encephalopathy. The exact origin of this channel is not known, but several possibilities are discussed. It is also predicted that similar previously unknown large intrahepatic shunts will be discovered increasingly with the availability of modern imaging techniques.