Review article: the aetiology of primary Budd-Chiari syndrome - differences between the West and China

Qi, Xun; Han, Guohong; Guo, Xiaozhong; Stefano, Valerio De; Xu, Ke; Lu, Zhaojun; Xu, Hao; Mancuso, Andrea; Zhang, W.; Han, Xinwei; Valla, Dominique; Fan, Daiming

doi:10.1111/apt.13815

Cited by 65 publications

(58 citation statements)

References 164 publications

(321 reference statements)

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“…Largely, BCS can be classified based on the regional prevalence: BCS in western countries (wBCS) and in Asian countries including China (aBCS). There are several major differences in aetiology, types, clinical manifestations and treatment between wBCS and aBCS . In western countries, where myeloproliferative neoplasms are the most common causes of wBCS and thrombotic obstruction of the HVs with patent of the inferior vena cava (IVC) account for more than half of wBCS cases.…”

Section: Introductionmentioning

confidence: 99%

Long‐term outcomes of individualized treatment strategy in treatment of type I Budd‐Chiari syndrome in 456 patients

Ding

Han

Liu

et al. 2019

Liver International

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Abbreviations: BCS, Budd-Chiari syndrome; HV, hepatic vein; ITS, individualized treatment strategy; IVC, inferior vena cava; MELD score, model for end-stage liver disease score; MOVC, membranous obstruction of the inferior vena cava; PTA, percutaneous transluminal balloon angioplasty; SOVC, segmental obstruction of the inferior vena cava; TIPS, transjugular intrahepatic portosystemic shunt.Abstract Aim: To evaluate individualized treatment strategy (ITS) and long-term outcomes of endovascular treatment of Budd-Chiari syndrome (BCS) with obstructed inferior vena cava (IVC) based on different degrees of hepatic vein (HV) involvement. , 456 consecutive patients with BCS with obstructed IVC underwent endovascular treatment with ITS. All patients received IVC recanalization. Then, 426 patients with at least one patent HV received no additional treatment. Twenty-fivepatients with membranous or segmental occlusion of HVs underwent HV recanalization and for the remaining five patients with diffuse HVs occlusion, a transjugular intrahepatic portosystemic shunt (TIPS) was performed.Results: The endovascular treatment was technically successful in 455 of the 456 patients (99.8%). The complication rate was 5.0% (23/456), with major complications in 13 patients (2.8%) and minor complications in 10 patients (2.2%). Median follow-up time was 60.5 months (range, 4-120 months). The cumulative 1-, 2-, 5-and 10-year primary vessel patency rates were 93.6%, 89.9%, 80.5% and 74.3% respectively and the cumulative 1-, 2-, 5-, 10-year secondary patency rates were 99.8%, 99.8%, 98.2% and 97.2% respectively. The cumulative 1-, 2-, 5-and 10-year survival rates were 98.4%, 95.8%, 91.2% and 76.5% respectively. Illness duration and decreased serum albumin were independent predictors of survival. Conclusion:The ITS for Asian BCS with obstructed IVC and varying degrees of HV involvement appears to be effective and with good long-term outcomes. K E Y W O R D Sangioplasty, Budd-Chiari syndrome, hepatic vein, inferior vena cava

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Section: Introductionmentioning

confidence: 99%

Long‐term outcomes of individualized treatment strategy in treatment of type I Budd‐Chiari syndrome in 456 patients

Ding

Han

Liu

et al. 2019

Liver International

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“…The biggest criticism of AASLD indication for the management of BCS is that they are based on the assumption that further treatment should be done for BCS only when hemodynamic consequences on portal hypertension become clinically evident, such as giving no consideration to the chronic ischemic liver damage effects on hepatic function and to the possibility of preventing liver failure by relieving impaired hepatic veins outflow [3,5,[12][13][14] . Moreover, patients receiving only medical therapy often have a bad outcome.…”

Section: Early Versus Delayed Interventional Treatment Of Bcsmentioning

confidence: 99%

“…Recently, I reported a proposal of a new algorithm for the management of BCS (Figure 2), in which medical therapy is suggested only for patients without any sign of portal hypertension, whether early interventional treatment when either any symptom or sign of portal hypertension appears, with the aim of preventing hepatic fibrosis development, disease progression, and finally improving outcome [3][4][5][6][7] . As I suggested, the same considerations about both timing and choice of treatment in BCS should probably apply to both adults and children [47,48] .…”

Section: Early Versus Delayed Interventional Treatment Of Bcsmentioning

confidence: 99%

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Timing of transjugular intrahepatic portosystemic shunt for Budd-Chiari syndrome: An Italian hepatologist’s perspective

Mancuso

2017

Journal of Translational Internal Medicine

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Budd-Chiari syndrome (BCS) management flow-chart is derived from experts' opinion and is not evidence-based. Guidelines suggest BCS management should follow a stepwise strategy: medical therapy as first-line treatment, revascularization or transjugular intrahepatic portosystemic shunt (TIPS) if no response to medical therapy, and liver transplant as rescue therapy. Recent evidence suggests that only medical therapy results in a bad long-term outcome. The biggest criticism of guidelines is the indication that BCS should receive further treatment only when hemodynamic consequences of portal hypertension become clinically evident. Recent data support that in BCS liver fibrosis could arise from chronic microvascular ischemia. A reasoning model of BCS physiopathology is that impaired hepatic vein outflow has hemodynamic consequences on portal hypertension development and causes hepatic fibrosis and liver failure through chronic ischemic damage. On this assumption is the concept that relieving liver congestion could ameliorate liver function and prevent development of BCS complications. Recently, early interventional treatment with TIPS for BCS has been reported to be effective. Early TIPS seems to be the best option for BCS management. Future multicenter controlled studies should compare the outcome of BCS treated with early interventional treatment compared with stepwise strategy.

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“…Differently, in China BCS respectively has a higher incidence, is mainly characterized by segmental or membranous inferior vena cava (IVC) obstruction with or without HVs occlusion, has a relatively chronic course and there is a less relevant role of PD. 1,2 Albeit the considerations about physiopathology are similar be- Differently from the ITS where sole medical therapy for BCS is considered futile, the Step by Step strategy (SSS), indicated for the management of BCS in the West, 3 suggests sole medical therapy as the first line. However, there are good reasons to prefer early intervention for BCS also in the West.…”

mentioning

confidence: 99%

Budd‐Chiari syndrome in the West and the East: Same syndrome, different diseases

Mancuso¹

2019

Liver International

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Review article: the aetiology of primary Budd-Chiari syndrome - differences between the West and China

Cited by 65 publications

References 164 publications

Long‐term outcomes of individualized treatment strategy in treatment of type I Budd‐Chiari syndrome in 456 patients

Long‐term outcomes of individualized treatment strategy in treatment of type I Budd‐Chiari syndrome in 456 patients

Timing of transjugular intrahepatic portosystemic shunt for Budd-Chiari syndrome: An Italian hepatologist’s perspective

Budd‐Chiari syndrome in the West and the East: Same syndrome, different diseases

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