J Clin Med Res

Journal of Clinical Medicine Research, ISSN 1918-3003 print, 1918-3011 online, Open Access

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Review

Volume 14, Number 4, April 2022, pages 147-157

State of the Art, Current Perspectives, and Controversies of Budd-Chiari Syndrome: A Review

Figure 2. Types of Budd-Chiari syndrome according to levels of obstruction in Table 1. Type I: (Truncal) obstruction involves the IVC ± HVs. Type II: (Radicular) with obstruction at the level of HVs. Type III: (Veno-oclusive) obstruction at the level of small centrilobular veins. HV: hepatic vein; IVC: inferior vena cava.

Figure 3. Types of Budd-Chiari syndrome according to levels of obstruction in Table 2. Type I: Hepatic vein obstruction or thrombosis without IVC obstruction or compression. Type II: Hepatic vein obstruction or thrombosis with IVC obstruction or thrombosis. Type III: Isolated hepatic venous webs. Type IV: Isolated IVC webs. RHV: right hepatic vein; MHV: middle hepatic vein; LHV: left hepatic vein; IVC: inferior vena cava.

**Table 1.** Three Types of BCS Based on the Level of Obstruction
Type	Level of obstruction
Reprinted with permission from Reference 3. BCS: Budd-Chiari syndrome; IVC: inferior vena cava.
I	Obstruction of IVC with or without secondary hepatic vein occlusion
II	Obstruction of major hepatic veins
III	Obstruction of the small centrilobular venules (considered by some as veno-occlusive disease)

Table 1. Three Types of BCS Based on the Level of Obstruction

Type

Level of obstruction

Reprinted with permission from Reference 3. BCS: Budd-Chiari syndrome; IVC: inferior vena cava.

Obstruction of IVC with or without secondary hepatic vein occlusion

Obstruction of major hepatic veins

III

Obstruction of the small centrilobular venules (considered by some as veno-occlusive disease)

**Table 2.** Four Types of BCS Based on the Level of Obstruction
Type	Level of obstruction
Reprinted with permission from Reference 3. BCS: Budd-Chiari syndrome; IVC: inferior vena cava.
I	Hepatic vein obstruction or thrombosis without IVC obstruction or compression
II	Hepatic vein obstruction or thrombosis with IVC obstruction or thrombosis
III	Isolated hepatic venous webs
IV	Isolated IVC webs

Table 2. Four Types of BCS Based on the Level of Obstruction

Type

Level of obstruction

Reprinted with permission from Reference 3. BCS: Budd-Chiari syndrome; IVC: inferior vena cava.

Hepatic vein obstruction or thrombosis without IVC obstruction or compression

Hepatic vein obstruction or thrombosis with IVC obstruction or thrombosis

III

Isolated hepatic venous webs

Isolated IVC webs

**Table 3.** Classification of BCS According to the Duration of Disease
Type	Duration of disease
Reprinted with permission from Reference 3. BCS: Budd-Chiari syndrome.
Fulminant	Present with hepatic encephalopathy within 8 weeks of development of jaundice
Acute	Short duration (< 1 month), ascites, hepatic necrosis without formation of venous collaterals
Subacute	Insidious onset (1 - 6 months), ascites, minimal hepatic necrosis, and portal and hepatic venous collaterals
Chronic	(> 6 months) Complications of cirrhosis in addition to findings in the subacute form

Table 3. Classification of BCS According to the Duration of Disease

Type

Duration of disease

Reprinted with permission from Reference 3. BCS: Budd-Chiari syndrome.

Fulminant

Present with hepatic encephalopathy within 8 weeks of development of jaundice

Acute

Short duration (< 1 month), ascites, hepatic necrosis without formation of venous collaterals

Subacute

Insidious onset (1 - 6 months), ascites, minimal hepatic necrosis, and portal and hepatic venous collaterals

Chronic

(> 6 months) Complications of cirrhosis in addition to findings in the subacute form

**Table 4.** Types of Acute on Chronic BCS
Type	Pathology	Management
Reprinted with permission from Reference 11. ACLF: acute-on-chronic liver failure; BCS: Budd-Chiari syndrome; HV: hepatic vein; TIPSS: transjugular intrahepatic portosystemic shunt.
A	Acute hepatic vein thrombosis or stent block precipitates ACLF in a BCS	Urgent recanalization as per anatomy operations: 1. Thrombectomy or thrombosis with stenting; 2. HV stenting; 3. TIPSS
B	Non-thrombotic acute insult precipitates ACLF in a chronic BCS	B1: BCS treated successfully previously, treat like any other ACLF B2: BCS untreated previously: 1. Treatment of BCS after recovery from acute insult; 2. Liver transplant
C	Acute hepatic vein thrombosis precipitates ACLF in a non-vascular chronic liver disease	Operations: 1. Thrombectomy or thrombosis with stenting; 2. HV stenting; 3. Liver transplant

Table 4. Types of Acute on Chronic BCS

Type

Pathology

Management

Reprinted with permission from Reference 11. ACLF: acute-on-chronic liver failure; BCS: Budd-Chiari syndrome; HV: hepatic vein; TIPSS: transjugular intrahepatic portosystemic shunt.

Acute hepatic vein thrombosis or stent block precipitates ACLF in a BCS

Urgent recanalization as per anatomy operations: 1. Thrombectomy or thrombosis with stenting; 2. HV stenting; 3. TIPSS

Non-thrombotic acute insult precipitates ACLF in a chronic BCS

B1: BCS treated successfully previously, treat like any other ACLF
B2: BCS untreated previously: 1. Treatment of BCS after recovery from acute insult; 2. Liver transplant

Acute hepatic vein thrombosis precipitates ACLF in a non-vascular chronic liver disease

Operations: 1. Thrombectomy or thrombosis with stenting; 2. HV stenting; 3. Liver transplant

**Table 5.** Prognostic Indices for BCS
Prognostic index	Parameters	Formula	Interpretation
Reprinted with permission from Reference 11. AIIMS-HVOTO: All India Institute of Medical Sciences Hepatic Venous Outflow Tract Obstruction; BCS: Budd-Chiari syndrome; INR: international normalized ratio; OLT: orthotopic liver transplantation; TIPSS: transjugular intrahepatic portosystemic shunt.
Clichy	Ascites, Child-Pugh score, age, creatinine	(Ascites score × 0.75) + (Pugh score × 0.28) + (Age × 0.037) + (Creatinine × 0.0036)	5-year survival < 5.4: 95% > 5.4: 62%
New Clichy score	Ascites, Child-Pugh score, age, creatinine, pathological form (acute, chronic, or both)	0.95 × Ascites score + 0.35 × Pugh score + 0.047 × Age + 0.0045 × Serum creatinine + (2.2 × form III)	5-year survival < 5.1: 100% > 5.4: 65%
Rotterdam score	Encephalopathy, ascites, prothrombin time, bilirubin	(1.27 × Encephalopathy) + (1.04 × Ascites) + (0.72 × Prothrombin time) + (0.004 × Bilirubin)	5-year survival Class I (0 - 1.1): 89% Class II (1.1 - 1.5): 74% Class III (> 1.5): 42%
BCS-TIPSS score	Bilirubin, age, INR	Age (years) × 0.08 + Bilirubin (mg/dL) × 0.16 + INR × 0.63	1-year OLT-free survival < 7: 95% > 7: 12%
AIIMS-HOVTO score	Response to therapy and Child-Pugh score	1.2 × Response to therapy + 0.8 × Child class	5-year survival < 3: 92% 3.1 - 4: 79% > 4: 39%

Table 5. Prognostic Indices for BCS

Prognostic index

Parameters

Formula

Interpretation

Reprinted with permission from Reference 11. AIIMS-HVOTO: All India Institute of Medical Sciences Hepatic Venous Outflow Tract Obstruction; BCS: Budd-Chiari syndrome; INR: international normalized ratio; OLT: orthotopic liver transplantation; TIPSS: transjugular intrahepatic portosystemic shunt.

Clichy

Ascites, Child-Pugh score, age, creatinine

(Ascites score × 0.75) + (Pugh score × 0.28) + (Age × 0.037) + (Creatinine × 0.0036)

5-year survival
< 5.4: 95%
> 5.4: 62%

New Clichy score

Ascites, Child-Pugh score, age, creatinine, pathological form (acute, chronic, or both)

0.95 × Ascites score + 0.35 × Pugh score + 0.047 × Age + 0.0045 × Serum creatinine + (2.2 × form III)

5-year survival
< 5.1: 100%
> 5.4: 65%

Rotterdam score

Encephalopathy, ascites, prothrombin time, bilirubin

(1.27 × Encephalopathy) + (1.04 × Ascites) + (0.72 × Prothrombin time) + (0.004 × Bilirubin)

5-year survival
Class I (0 - 1.1): 89%
Class II (1.1 - 1.5): 74%
Class III (> 1.5): 42%

BCS-TIPSS score

Bilirubin, age, INR

Age (years) × 0.08 + Bilirubin (mg/dL) × 0.16 + INR × 0.63

1-year OLT-free survival
< 7: 95%
> 7: 12%

AIIMS-HOVTO score

Response to therapy and Child-Pugh score

1.2 × Response to therapy + 0.8 × Child class

5-year survival
< 3: 92%
3.1 - 4: 79%
> 4: 39%

**Table 6.** Category of Response to Therapy or Clinical Success After Endovascular Treatment for BCS
Category	Residual stenosis	PG	Ascites	Liver function tests
Reprinted with permission from Reference 63. BCS: Budd-Chiari syndrome; PG: pressure gradient.
Excellent	0 - 25	Hemodynamically comparable reduction of PG	Complete reduction (if present)	Improved
Good	25 - 30	Comparable residual PG	Stable (not requiring paracentesis or diuretics)	Stable
Fair	30 - 50	Improved PG	Controlled with paracentesis or diuretics	Mildly deteriorated
Poor	> 50	Minimal or no improvement	Increasing	Cross derangement

Table 6. Category of Response to Therapy or Clinical Success After Endovascular Treatment for BCS