Survival and clinical success of endovascular intervention in patients with Budd-Chiari syndrome: A systematic review

INTRODUCTION

Budd-Chiari syndrome (BCS) is a complex clinical disorder of hepatic venous outflow obstruction, originating from the accessory hepatic vein (HV), large HV, and suprahepatic inferior vena cava (IVC).^[1] This disorder includes both HV and IVC obstructions and hepatopathy.^[2] Partial or complete obstruction of the IVC with membranous or segmental lesions was considered to be the main cause of BCS in Asian countries.^[3] The membranous obstruction of the IVC contributes to two-thirds of patients with BCS in Asia.^[4] Most patients with BCS present late after developing symptoms or in their chronic conditions, whereas only a small number of patients present with an acute and fulminant type of BCS.^[5] BCS is more commonly seen in adults than in children; when seen in children, clinical manifestations are similar to those in adults.^[6] Endovascular intervention treatment has emerged as an advanced therapeutic option for patients with BCS. Transjugular intrahepatic portosystemic shunt (TIPS) procedures have rapidly replaced the traditional surgical shunt on account on their due to minimal invasiveness, low blood loss, low infection rate, quick recovery, shorter hospital stay, and increased long-term survival rate.^[7,8] TIPS significantly reduced portal venous pressure through placement of an artificial stent from the portal vein to the HV. The patency of shunts has greatly improved since the adoption of dedicated polytetrafluoroethylene stents.^[9] Recanalization is a physiological procedure that maintains natural blood flow in the HV/IVC.^[10] It can minimize the risk of hepatic encephalopathy, and remains a first-line treatment option for patients with BCS.^[11,12] One-third of short-length HV stenosis was treated with recanalization by percutaneous transluminal angioplasty (PTA) with or without stent placement.^[13,14] Recanalization has shown promising results in Asian countries with excellent clinical outcomes and higher survival rates.^[15] The European Association for the Study of the Liver recommended a stepwise therapeutic algorithm for BCS. The algorithm depends on treatment response, medical therapy with anticoagulant drugs, angioplasty, stent placement, TIPS, and liver transplantation.^[16] The prognosis of patients with BCS depends on the onset of obstruction in vessels with anatomical location and liver dysfunction. However, new developments and improvements in radiological endovascular therapy and early diagnosis have increased the survival rate of patients with BCS.

The present systematic review aimed to evaluate the survival rate of BCS after different types of endovascular intervention, clinical success after initial different types of endovascular intervention treatment, and survival rate of BCS in the publication year.

METHODS

Data selection

All published articles met the eligibility criteria according to the population, interventions, comparison, outcomes, and study results. The study selection process was demonstrated in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-analysis guidelines [Figure 1].

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Figure 1:

Preferred reporting items for systematic reviews and meta-analysis flow diagram of studies selection process.

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The inclusion criteria were: (1) Retrospective studies, prospective studies, including case-control studies were eligible; (2) all the previous studies reporting the survival rate and clinical success; (3) full article papers with detailed information and statistical results of intervention treatment; and (4) there were no publication data, publication language, or publication status restrictions.

Exclusion criteria were: (1) Duplicate studies; (2) studies that were not original papers; (3) studies unrelated to the subject matter of this review; (4) case reports; (5) comments; (7) essays; (8) abstracts; (9) not reporting relevant clinical outcomes; (10) lack of details results; (11) review articles; (12) fewer than ten patients; (13) studies unmatched inclusion criteria; and (14) studies with missing survival rate, re-intervention rate, and clinical success.

Data analysis

We summarized the 1- and 5 years survival rates according to different types of endovascular intervention treatment mortalities and publication years in an Excel worksheet. Then, a box plot was drawn to describe the median survival rate with range using SPSS software (version 16.0; SPSS, Chicago, IL, USA).

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Flow chart of indication of the endovascular management in BCS

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RESULTS

Clinical success in different types of interventional endovascular procedures

The overall clinical success rate of endovascular intervention in patients with BCS was 93.6%. The median clinical success of recanalization procedures was 51% (range: 32–70%) in 26 studies, in which the patients were treated with angioplasty with or without stent; for the combined procedures was 55.50% (range: 31‒79%) in 16 studies, in which patients were treated with recanalization (angioplasty with or without stent) and TIPS. TIPS procedure was 18.50% (range: 14– 53%) in 14 studies, where patients were treated with TIPS [Figure 2].

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Figure 2:

Box plot of median clinical success in different types of endovascular procedures (recanalization procedure in 26 studies; 51%, range: 32–70%, TIPS procedure in 14 studies; 18.50%, range: 14–53%, and combined procedures in 16 studies; 55.50%, range: 31– 79%). BCS: Budd-Chiari syndrome, TIPS: Transjugular intrahepatic portosystemic shunt.

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Survival rate at 1 year in different types of interventional endovascular procedures

The total survival rate at 1 year of endovascular treatment in 56 studies was 96.9%, whereas the median survival rate at 1 year of recanalization was 51% (range: 31‒70%) in 26 studies treated with angioplasty with or without stent placement. Similarly, the median survival rate at 1 year of combined procedures was 52.50% (range: 29–71%) in 16 studies using angioplasty, stent, and TIPS; and 17.50% (range: 13–51%) in 14 studies using TIPS [Figure 3].

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Figure 3:

Box plot of median survival at 1 year different type of endovascular treatment in BCS (recanalization procedure in 26 studies; 51%, range: 31–70%, TIPS procedure in 14 studies; 17.50%, range: 13–51%, and combined procedures in 16 studies; 52.50%, range: 29–71%). BCS: Budd-Chiari syndrome, TIPS: Transjugular intrahepatic portosystemic shunt.

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Survival rate at 5 years in different types of interventional endovascular procedures

The total survival rate at 5 years of endovascular treatment in 56 studies was 93.3%, whereas the median survival rate at 5 years of recanalization was 51% (range: 31‒66%) in 26 studies treated with angioplasty with or without stent placement. Similarly, the median survival rate at 5 years of combined procedures was 49.50% (range: 29–70%) in 16 studies using recanalization (angioplasty, stent) and TIPS; and 16% (range: 12–44%) in 14 studies using TIPS [Figure 4].

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Figure 4:

Box plot of median survival at 5 years different type of endovascular treatment in BCS (recanalization procedure in 26 studies; 51%, range: 31–66%, TIPS procedure in 14 studies; 16%, range: 12–44%, and combined procedures in 16 studies; 49%, range: 29–70%). BCS: Budd-Chiari syndrome, TIPS: Transjugular intrahepatic portosystemic shunt

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Year of publication survival rate at 1 year

We noted that the rate of survival had increased progressively in recent studies. The median survival rate at 1 year was 23.50% (range: 13‒34%) in four studies published before 2000 and 41% (range: 21–49%) in seven studies published between 2000 and 2005. Similarly, the median survival rate at 1 year was 35% (range: 13‒46%) in five studies published between 2006 and 2010 and 51% (range: 18‒92%) in 18 studies published between 2011 and 2015. The highest median survival rate, 56% (range: 36‒70%) was noted in studies published after 2015 [Figure 5].

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Figure 5:

Box plot of median survival at 1 year according to year of publication in endovascular treatment of BCS (<2000 = 23.50%, range: 13–34%; 2000–2005 = 41%, range: 21–49%; 2006–2010 = 35%, range: 13–46%; 2011–2015 = 51%, range: 18–92%; and >2015 = 56%, range: 36–70%). BCS: Budd-Chiari syndrome.

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Year of publication survival rate at 5 years

The median survival rate at 5 years was 22.50% (range: 12.5–34.5%) in four studies published before 2000, 41% (range: 21.5–47%) in seven studies published between 2000 and 2005, and 35% (range: 13–46%) in five studies published between 2006 and 2010. Similarly, the median survival rate was 48.50% (range: 18–91%) in 18 studies published between 2011 and 2015, and 55.50% (range: 34–64%) in 22 studies published after 2015 [Figure 6].

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Figure 6:

Box plot of median survival at 5 years according to year of publication in endovascular treatment of BCS (<2000 = 22.50%, range: 12.5–34.5%; 2000–2005 = 41%, range: 21.5–47%; 2006–2010 = 35%, range: 13–46%; 2011–2015 = 48.50%, range: 18–91%; and >2015 = 55.50%, range: 34–64%). BCS: Budd-Chiari syndrome.

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DISCUSSION

To the best of our knowledge, this is the first systematic review to present the survival rate and clinical success of different types of endovascular interventional procedures in BCS. We found that most patients with BCS were treated with recanalization rather than the TIPS procedure, our results also indicating that recanalization is more common with a better survival rate. In addition, due to the high rate of shunt dysfunction, re-intervention was more common in the TIPS procedure than in recanalization.^{[8,28,34,38,40]} However, these findings should be interpreted cautiously, as the range of the survival rate overlapped among different methods of endovascular treatment among individual studies.

The overall clinical success rate was 93.6%, and the survival rates at 1 and 5 years were 96.9% and 93.3%, respectively, for interventional endovascular treatment of BCS in 56 studies. The median survival rates at 1 and 5 years of recanalization were 51% and 51%, respectively, which were higher than those of TIPS treatment. Recanalization is a comparatively easier and quicker procedure than TIPS, and this study indicates that recanalization is more common than TIPS. In addition, in the subgroup analysis, the survival rate based on the year of publication showed a high median survival rate published after 2015. This finding indicates that the publication survival rate has progressively increased with the development of interventional endovascular therapy in recent years.

In the past few decades, modern techniques and developments in interventional endovascular therapy have contributed to progressive improvements in clinical outcomes and decreased mortality in patients with BCS. Before 1985, the survival rate at 1 and 10 years was approximately 60–70%, far less than the moderate survival rate offered by modern endovascular treatment in patients with BCS, as reported in recent studies.^[69,70] The treatment for BCS is best administered in an algorithm approach and depends on the response to the previous treatment.^[42,71] Medical therapy alone has a low success rate in BCS; however, interventional endovascular therapy provides high patency with good outcomes.^[72] In the Western countries, anticoagulation therapy and TIPS are the most commonly used treatment modalities for patients with BCS.^[73] However, recanalization has shown promising results in Asian countries with excellent clinical outcomes and higher survival rates.^[15]

HV recanalization was performed in patients with short-segment HV obstruction (<3 cm), and stenting was performed in long segment HV occlusion (>3 cm) with large collateral vein drainage.^[37] HV recanalization is usually difficult for BCS patients with segmental obstruction, whereas TIPS placement has been widely used for BCS patients who fail to HV recanalization.^[40,74]

Recanalization restoring the physiological hepatic blood flow in liver ^[35,40] whereas, TIPS reduce portal venous pressure resulting in decrease symptoms by physiological modification of hepatic venous flow in the patients of BCS.^[75] Recanalization can minimize the risk of hepatic encephalopathy and remains a first-line treatment option in patients with BCS.^[11,12] However, TIPS has less portal vein blood perfusion in the liver with patients of BCS than recanalization and a high risk of hepatic encephalopathy due to the formation of a blood ammonia level and impaired liver function after shunt placement.^[46]

In BCS, one-third of short-length stenosis was treated with recanalization by PTA with or without stent placement.^[13,14,55] Tripathi et al. followed the long-term outcome of recanalization in 63 patients with BCS^[55] and compared it with previously reported 59 BCS patients treated with TIPS.^[42] The survival rates for recanalization at 1, 5, and 10 years were 97%, 89%, and 85%, respectively, which were comparable to the survival rates for TIPS. However, procedural complications and hepatic encephalopathy were significantly different (9.5% vs. 27.1%) and (0% vs. 18%), respectively.

In the past two decades, TIPS has been successfully used to treat BCS patients with a long-term survival rate.^[10,76] Recently, an increasing number of patients with BCS have been managed using TIPS procedure.^[77] The common indications for TIPS in patients with BCS include obstruction of all three HVs, refractory ascites, diffuse HV obstruction, portal hypertension, failed PTA, and occurrence of technical and clinical difficult to maintain long-term HV outcome patency.^[78] Several previous studies have shown that TIPS can increase the survival rate in patients with BCS.^[21-23,79] Qi et al. systematically reviewed the role of TIPS in the treatment of BCS and showed that the survival rates at 1 year and 5 years were 80–100% and 74–78%, respectively.^[79] Similarly, another study examining the outcomes of interventional treatment in BCS^[14] demonstrated that the survival rate of the TIPS at 1 and 5 years was 87% and 72%, and the survival rates of recanalization at 1 and 5 years were 96% and 89%, respectively. The authors also claimed that BCS patients treated with recanalization had a better survival rate than those treated using the TIPS procedure.^[14] In the current systematic review, the survival rates at 1 and 5 years of recanalization were 98.5% and 95.3%, and the survival rates at 1 and 5 years for TIPS were 93.5% and 86.4%, respectively. Compared to the previous studies, our results showed a progressive increase in the survival rate.^[14]

This systematic review has several limitations. First, several relevant full articles were excluded due to different analysis results and missing long-term follow-up records. Second, in the available studies, the number of patients treated using the TIPS procedure was lower than those treated with recanalization procedures. Third, the survival rate in combined (recanalization, stent, and TIPS) studies was not recorded separately. Fourth, during subgroup analysis of survival rate, a scattered distribution was observed for the year of publication. However, we noted a rapid increase in the median survival rate in studies published after 2015.

CONCLUSION

Our findings suggest that the median survival rate at 1 and 5 years of recanalization treatment is higher than that of TIPS treatment, and recanalization provides better clinical improvement. The publication year findings strongly suggest progressive improvement in intervention endovascular therapy for BCS. Thus, interventional therapy restoring the physiologic hepatic venous outflow of the liver can be considered as the treatment of choice for patients with BCS, which is a physiological modification procedure.