One article to master: The treatment and management of primary Budd-Chiari syndrome | Clinical essentials

Budd-Chiari Syndrome (BCS) is a kind of posterior hepatic portal that is caused by the occlusion of the hepatic vein and the inferior vena cava above its opening, often accompanied by inferior vena cava hypertension.
Pulse hypertension.

Primary BCS refers to the presence of venous thrombosis, and secondary BCS refers to mechanical obstruction caused by malignant tumors, abscesses, cysts and other reasons.

This review will focus on the treatment management of primary BCS-stepwise treatment.

Yimaitong compiles and organizes, please do not reprint without authorization.

Overview Primary BCS is often the result of multiple risk factors.
The most common factors are heredity and acquired thrombosis tendency.

Because the disease is relatively rare, it is necessary to maintain a high degree of clinical suspicion, especially in young adult patients with new ascites, hepatomegaly, or caudate lobe enlargement.

Although there is a lack of high-quality evidence guided by randomized controlled trials, progress has been made in the management of BCS through individualized, step-by-step, multidisciplinary strategies (including anticoagulation and other drug treatments, and intravascular interventions).
Body shunts (TIPSs), surgical shunts, and liver transplantation.

Implementing incremental stepped multidisciplinary management in an individualized way is the recommended method for BCS treatment.

The main goals of treatment include improving the symptoms and signs of portal hypertension and protecting liver function.

Anticoagulation and drug therapy In retrospect, although there is no relevant research to clarify the optimal intensity of anticoagulation therapy, it is still recommended to use heparin and vitamin K antagonists for anticoagulation therapy for BCS patients.

It is recommended to start low-molecular-weight heparin (LMWH) anticoagulation therapy immediately after diagnosis, and titrate it to an anti-Xa factor activity of 0.
5 IU/mL-0.
8 IU/mL.

In 43 patients who received continuous warfarin treatment, the target INR range was 2-3, and the follow-up results at 23 months were that more than 60% of patients had ascites regression and liver normalization.

Although it has not yet been approved, direct oral anticoagulants (DOAC) have been used to treat BCS patients with clear indications.
For patients who have experienced repeated thrombosis at the therapeutic level of warfarin and/or low molecular weight heparin, this may Means another choice.

A recent study compared the prognosis of patients with acute venous thrombosis in atypical sites (such as visceral veins) and typical sites (deep veins of the extremities/pulmonary embolism) with DOACs.
The study showed that the recurrence rate of thrombosis and the rate of major hemorrhage were similar, indicating that DOACs treatment The effect on visceral thrombosis may be as effective as other thrombosis.

However, more research is needed before the standard is used.

In a few cases, recombinant tissue plasminogen activator has been used for systemic thrombolytic therapy, but there is little evidence for its use.

Systemic thrombolytic therapy as the main treatment for BCS may be limited to the following conditions: the thrombus load is too high and other endovascular treatments or surgical treatments cannot be performed.

In addition, systemic thrombolytic therapy is unlikely to benefit patients with chronic BCS.

If acute thrombosis is caused or exacerbated by intravascular devices, locally delivered thrombolytic agents may increase the possibility of vascular recanalization.

Due to the use of anticoagulants, BCS patients may experience heavy bleeding.

Bleeding is related to the invasive operation of BCS treatment.
The most common bleeding is portal hypertensive bleeding, non-portal hypertensive gastrointestinal bleeding and reproductive tract bleeding.

The severity of BCS is related to the prognosis after bleeding events.

For patients with myeloproliferative diseases, cytoreductive therapy can reduce the risk of thrombosis recurrence by addressing the underlying causes.

Hydroxyurea is the first-line treatment for potential patients with polycythemia vera and essential thrombocythemia.

For patients with polycythemia vera or myelofibrosis who have no response or intolerance to hydroxyurea, ruxolitinib can be considered.

The participation of hematology experts is particularly important in guiding the treatment of underlying diseases of patients with myeloproliferative diseases.

An important aspect of the initial treatment of BCS patients is to deal with the complications of portal hypertension, such as ascites, encephalopathy, renal insufficiency, and gastrointestinal bleeding.

Patients need to be screened for gastroesophageal varices.
If found, non-selective beta blockers can be used to prevent them.

For patients with non-cirrhotic portal hypertension who have variceal bleeding, secondary prevention measures include beta-blockers and endoscopic varicose ligation.

Percutaneous angioplasty and stent placement Intravascular approaches, such as angioplasty and stent placement, have been widely used in the past 20 years.

Encourage the use of high-quality diagnostic imaging to help guide interventional treatment methods.

When short stenosis of the hepatic vein or inferior vena cava is suspected, angioplasty is usually performed.

Angioplasty can be combined with the placement of permanent stents or retrievable stents, especially when there is an inferior vena cava obstruction.

The results of a recently published randomized trial showed that compared with angioplasty alone, patients with angioplasty combined with a stent have higher vascular patency (98% vs.
60%) and a higher survival rate without restenosis within 3 years (90% vs.
60.
4%).

Although the combination of angioplasty and local thrombolysis of streptokinase or urokinase is unstable and is related to the high incidence of major bleeding, it can be used in rare cases.
The benefit of local thrombolysis may be limited to intravascular surgery Acute recurrence of thrombosis occurred later.

Transjugular intrahepatic portosystemic shunt involves the establishment of portosystemic shunt.
Surgical options include lateral contralateral portal vena cava shunt and combined lateral contralateral portal vena cava and vena cava shunt.

The type of surgery performed depends on the type and location of the vein blockage.

With the widespread application of transjugular intrahepatic portosystemic shunt and endovascular treatment, this surgical method is less common.

Additional tests include angiography and portal pressure gradient measurement to determine if portal shunting is feasible.

Surgical recanalization is more common in patients with inferior vena cava obstruction and/or rupture of the obstructive membrane or reconstruction of the inferior vena cava.

Surgery is more risky than staged percutaneous treatment.

With the development of experience in percutaneous and minimally invasive endovascular treatment, surgical shunts may become less common.

When liver decompensation is severe, progressive, or percutaneous or surgical treatment fails, liver transplantation should be considered.

For patients with underlying myeloproliferative diseases, it is recommended to treat with hydroxyurea and aspirin after transplantation to reduce the possibility of thrombosis recurrence and avoid the risk of bleeding associated with anticoagulation.

Liver transplantation When fulminant liver failure or decompensated liver cirrhosis is accompanied by BCS, causing progressive liver decompensation, liver transplantation is the last resort.

Although BCS is a rare indication for liver transplantation, the outcome of treatment is favorable and has been greatly improved over time.

A recent study showed that there was no significant difference in the long-term survival rate of BCS patients with or without underlying myeloproliferative disease after transplantation.

Recent prognostic analysis of BCS patients after liver transplantation shows that the 10-year survival rate of patients is 68%-84%.

The relevant guidelines for the treatment of Budd-Chiari syndrome are shown in the table below.

(Click to view larger image) Summary The treatment of BCS usually requires multidisciplinary, individualized, and step-by-step management.

Despite attempts at intravascular recanalization or surgical shunting, liver transplantation is the last option for BCS patients who do not respond to drug therapy or who have continuous/progressive liver decompensation.

Clinicians need to maintain a high degree of suspicion of BCS, and should consider typical features, including ascites, hepatomegaly, or caudate lobe enlargement.
Timely identification is the key to preventing and dealing with the emergence of critical illness.

 Yimaitong compiled from: Lamia YK Haque, Joseph K.
Lim.
Budd-Chiari Syndrome An Uncommon Cause of Chronic Liver Disease that Cannot Be Missed.
Clinics in Liver Disease.
VOLUME 24, ISSUE 3, P453-481, AUGUST 01, 2020 .
DOI: https://doi.
org/10.
1016/j.
cld.
2020.
04.
012