01Approach to the Critical Patient/attcp55

Immediate priorities

›Fulminant hepatic failure recognition

›Encephalopathy with coagulopathy

›INR >1.5 with grade II or higher encephalopathy

›Transplant-free survival historically 37–42% without intervention

›Rapidly rising bilirubin

›Conjugated hyperbilirubinemia as early marker of outflow obstruction

›Bilirubin >170 mmol/l indicates severe injury

›Massive ascites with renal failure

›Hepatorenal physiology — creatinine rising despite volume

›Serum lactate if hepatic ischemia suspected

›Variceal hemorrhage

›Hematemesis or melena in setting of portal hypertension

›If active GI bleeding, initiate octreotide 50 mcg IV bolus then 50 mcg/hr infusion

›Airway and hemodynamic stabilization

›Airway protection in encephalopathy

›GCS <8 or inability to protect airway — proceed to intubation

›Rapid sequence intubation preferred

›Hemodynamic targets

›MAP >65 mmHg

›SBP >90 mmHg

›IV access and volume resuscitation

›Two large-bore IVs or central access

›Avoid aggressive crystalloid — worsens ascites and coagulopathy

Monitoring and targets

›Monitoring bundle

›Continuous cardiac monitoring

›Tachyarrhythmia from electrolyte derangement or encephalopathy

02History/hx71

Presentation pattern

›Temporal classification

›Acute presentation

›Rapid-onset severe RUQ pain

›Marked transaminase elevation — AST/ALT >1000 IU/L

›Onset over days; may progress to fulminant failure

›Subacute presentation — most common

›Insidious ascites and hepatomegaly over weeks to months

›Abdominal distension and weight gain from ascites

›Early satiety and anorexia

›Chronic presentation

›Discovered incidentally or via portal hypertension complications

›Approximately 20% asymptomatic at diagnosis

›Variceal bleeding or refractory ascites as presenting complication

›Cardinal symptoms

›Abdominal pain

›Right upper quadrant or diffuse

›Often the presenting complaint across all subtypes

›Ascites

›Rapid-onset ascites is a hallmark of BCS

›Increasing girth and weight gain

›Jaundice

›Timing and progression

›Conjugated hyperbilirubinemia

›Lower extremity edema

›Bilateral — suggests IVC involvement

›Scrotal edema in males

03Physical Exam/exam36

Vital signs

›Hemodynamic assessment

›Tachycardia — decompensation marker

›Hypotension if severely decompensated

›Fever uncommon unless superinfected ascites (spontaneous bacterial peritonitis)

›Oxygen saturation — hepatopulmonary syndrome if SpO2 <96% supine

Abdominal examination

›Hepatomegaly

›Often tender to palpation

›Caudate lobe hypertrophy — palpable in some cases

›Caudate lobe drains directly into IVC; hypertrophy is compensatory

›Ascites

›Shifting dullness

›Fluid wave in tense ascites

›Rapid-onset distinguishes BCS from cirrhotic ascites

›Splenomegaly

›Portal hypertension or underlying MPN

›Thrombocytopenia from hypersplenism

›Abdominal wall veins

›Pattern extending to flanks and back — IVC collaterals

›Distinct from typical periumbilical caput medusae of portal hypertension

›Flow direction differentiates portal HTN from IVC obstruction

Systemic examination

›Extremity findings

›Bilateral lower extremity edema — IVC obstruction

›Scrotal edema or varicosities

›Absence of unilateral edema distinguishes IVC from DVT-only pathology

›Skin findings

04Differential Diagnosis/ddx32

Hepatic vascular mimics

›Right-sided heart failure / constrictive pericarditis

›Hepatic congestion with ascites, elevated JVP

›ICD-10: I50.9 (heart failure), I31.1 (constrictive pericarditis)

›Echocardiography differentiates — BCS has normal cardiac function

›Sinusoidal obstruction syndrome (SOS/VOD)

›Post-hematopoietic stem cell transplant or high-dose chemotherapy

›Similar congestion pattern, different clinical context

›ICD-10: K76.5

›Tricuspid regurgitation

›Pulsatile hepatomegaly, elevated JVP

›Echocardiography diagnostic

Hepatic parenchymal disease

›Cirrhosis — any etiology

›Viral, alcoholic, NASH — BCS should be considered in unexplained liver disease

›ICD-10: K74.60

›Doppler ultrasound distinguishes patent vs. obstructed hepatic veins

›Acute hepatitis — viral or drug-induced

›Marked transaminase elevation overlaps with acute BCS

›ICD-10: B17.9 (viral), K71.6 (drug-induced)

›Hepatic vein patency on Doppler excludes BCS

›Portosinusoidal vascular disease

›Formerly idiopathic portal hypertension

›Can mimic chronic BCS with intrahepatic collaterals

Vascular obstruction mimics

›Portal vein thrombosis (without hepatic vein involvement)

›ICD-10: I81

›Hepatic veins patent on Doppler

05Laboratory Tests/lt59

Hepatic function panel

›Aminotransferases

›AST/ALT markedly elevated in acute BCS — may exceed 1000 IU/L

›AST > ALT pattern characteristic of BCS

›Mild or normal elevation in chronic BCS

›Bilirubin

›Conjugated hyperbilirubinemia predominates

›Severity reflects degree of hepatocyte injury and cholestasis

›Albumin

›Low in subacute/chronic disease — hepatic synthetic failure

›Normal or near-normal in early acute BCS

›Alkaline phosphatase

›Elevated from biliary congestion and hepatocyte injury

Coagulation studies

›PT/INR

›Prolonged INR — hepatic synthetic failure marker

›INR >1.5 with encephalopathy defines fulminant presentation

›Factor V level

›Prognostic marker — factor V <25% indicates severe injury

›Not affected by vitamin K deficiency; reflects true synthetic function

›aPTT and fibrinogen

›DIC screen in fulminant presentation

›Fibrinogen low if DIC coexists

Hematologic evaluation

›Complete blood count with differential

›Erythrocytosis — polycythemia vera clue

›Thrombocytosis — essential thrombocythemia clue

›Leukocytosis — MPN or infection

06Diagnostic Tests/dt70

Scoring Systems

›Rotterdam BCS Prognostic Score

›Variables: ascites, encephalopathy, PT, bilirubin

›Predicts intervention-free survival

›Validated in European cohorts

›Higher scores correlate with need for TIPS or transplant

›BCS-TIPS Prognostic Index (BCS-PI)

›Variables: age, bilirubin, INR

›Predicts outcomes after TIPS placement

›Guides patient selection for TIPS vs. transplant listing

›West Haven Criteria — hepatic encephalopathy grading

›Grade I: Trivial altered sleep, impaired concentration

›Grade II: Lethargy, disorientation, asterixis

›Grade III: Somnolence, confusion, gross disorientation

›Grade IV: Coma

MRI

›Indications

›Confirmatory imaging when Doppler ultrasound is equivocal

›Surgical and interventional planning

›MRI has highest AUC (90.8%) for diagnosis of BCS among imaging modalities

›MRI venography protocol

›Contrast-enhanced MRI with hepatic venous phase

›Dynamic gadolinium sequences — arterial, portal venous, hepatic venous phases

›Characteristic findings

›Absent hepatic vein opacification with preserved IVC enhancement

›Patchy mosaic parenchymal enhancement at arterial phase

›Caudate lobe hypertrophy with heterogeneous liver morphology

›Intrahepatic and extrahepatic venovenous collaterals

›Thrombus signal in hepatic veins — T1 bright in subacute thrombosis

›Pearls

›Superior to CT for soft tissue characterization and thrombosis age

›Avoids radiation — preferred in younger patients and pregnant women

›Liver elastography can be performed to assess fibrosis and monitor treatment response

CT

›Indications

›Initial cross-sectional imaging when Doppler is inadequate

›Emergency evaluation — faster acquisition than MRI

›CT venography for IVC and hepatic vein anatomy prior to intervention

›Protocol

›Contrast-enhanced CT with triple-phase protocol

›Arterial, portal venous, and delayed hepatic venous phases

›Characteristic findings

›Absent hepatic vein opacification at hepatic venous phase

›Patchy heterogeneous liver enhancement — "mosaic" pattern

›Caudate lobe hypertrophy with relatively preserved enhancement

›Intrahepatic collateral vessels — tortuous enhancing vessels replacing hepatic veins

›Extrahepatic collaterals in IVC obstruction

›IVC narrowing or thrombus with collateral venous drainage

›Limitations

›Radiation exposure limits repeated use in younger patients

›Contrast nephropathy risk in hepatorenal syndrome

›MRI superior for thrombus characterization

Ultrasound

›Doppler ultrasound — first-line imaging

›Sensitivity 85–89% when performed by experienced operator

›Recommended as initial imaging by ACG and AASLD

›Noninvasive, no radiation, bedside availability

›Diagnostic findings

›Hepatic vein thrombus — echogenic material within hepatic veins

›Nonvisualization of hepatic veins — absent flow on color Doppler

›Absent or reversed hepatic venous flow on spectral Doppler

›Caudate vein diameter >3 mm — compensatory collateral

›Caudate lobe hypertrophy — echogenic, enlarged caudate relative to right lobe

›Intrahepatic collateral vessels — tortuous intraparenchymal channels

›Splenomegaly and ascites — portal hypertension findings

›Additional ultrasound findings

›Heterogeneous liver echogenicity

›Absent normal triphasic hepatic venous waveform

›IVC assessment — thrombus, web, or extrinsic compression

›Hepatic venography

›"Spiderweb pattern" — confirmatory when noninvasive imaging equivocal

›Gold standard for interventional planning

›Intravascular ultrasound (IVUS) adjunct aids lesion characterization

›Determines need for stenting vs. angioplasty alone

07Disposition/dispo31

Admission criteria

›ICU-level admission

›Fulminant liver failure — encephalopathy with coagulopathy

›Early transplant listing if factor V <25%

›Organ-system support monitoring

›Variceal hemorrhage with hemodynamic instability

›Severe hepatorenal syndrome — creatinine rapidly rising

›Grade III/IV hepatic encephalopathy

›General ward admission

›New-onset ascites with liver dysfunction requiring diuretic initiation

›Anticoagulation initiation requiring monitoring

›Subacute BCS with any organ dysfunction

›Grade I/II encephalopathy, stable hemodynamics

›GI bleeding controlled, hemodynamically stable

Transfer criteria

›Transfer to tertiary liver center

›All acute and subacute BCS — early referral strongly recommended by ACG/AASLD

›Interventional radiology with TIPS or DIPS capability required

›Liver transplantation program access if fulminant or TIPS failure

›Multidisciplinary team: hepatology, IR, transplant surgery, hematology

›Transfer timing

›Emergent: Fulminant liver failure, variceal hemorrhage not controlled locally

›Urgent: Within 24–48 hours for subacute BCS without stabilization

›Elective: Stable chronic BCS for outpatient specialist evaluation

Discharge criteria

›Outpatient management

›Chronic BCS discovered incidentally with stable liver function

›Initiate anticoagulation and thrombophilia workup as outpatient

08Treatment/t89

Anticoagulation therapy

›Initial anticoagulation — LMWH preferred

›Enoxaparin 1 mg/kg subcutaneously twice daily

›Renal dose adjustment if creatinine clearance <30 ml/min

›Monitor anti-Xa levels in hepatic failure — standard dosing unreliable

›Bridge until therapeutic warfarin achieved

›Avoid unfractionated heparin long-term

›High risk of heparin-induced thrombocytopenia (HIT) in BCS

›UFH acceptable short-term in renal failure or pre-procedural

›Long-term anticoagulation — warfarin (VKA)

›Target INR 2–3

›Lifelong anticoagulation recommended — even without identified prothrombotic disorder

›Recurrent thrombosis risk remains high without anticoagulation

›Direct oral anticoagulants (DOACs)

›Emerging data suggest safety and efficacy in BCS

›Not yet standard of care — evidence remains limited

›Consider in patients intolerant of warfarin or difficulty with INR monitoring

›Avoid if severe hepatic impairment (Child-Pugh C) — DOACs are hepatically metabolized

Portal hypertension management

›Ascites management — stepwise

›Sodium restriction: 2 g/day (88 mmol/day)

›Spironolactone 100 mg orally once daily

›Titrate by 100 mg every 3–5 days

›Maximum 400 mg/day

›Monitor potassium — hyperkalemia risk in hepatorenal syndrome

›Furosemide 40 mg orally once daily

›Add if inadequate response to spironolactone alone

›Typical ratio spironolactone:furosemide 100:40 mg

›Titrate furosemide to maximum 160 mg/day

›Large-volume paracentesis

›Indicated for tense ascites or respiratory compromise

›Albumin replacement 6–8 g per litre of ascites removed

›>5 litres removed — albumin infusion mandatory to prevent post-paracentesis circulatory dysfunction

›Variceal bleeding prophylaxis

›Nonselective beta-blockers — primary and secondary prophylaxis

›Propranolol 20 mg orally twice daily

›Titrate to resting heart rate 55–60 beats/min

›Maximum 320 mg/day

›Nadolol 40 mg orally once daily

›Titrate to resting heart rate 55–60 beats/min

›Maximum 160 mg/day

›Endoscopic variceal ligation (EVL)

›Secondary prophylaxis after index variceal bleed

›Repeat every 2–4 weeks until varices eradicated

›Active variceal hemorrhage

›Octreotide 50 mcg IV bolus then 50 mcg/hr continuous infusion

›Continue for 3–5 days

›Class I recommendation

›Ceftriaxone 1 g IV daily for 7 days

›Antibiotic prophylaxis reduces risk of SBP and improves survival

›Class I recommendation for cirrhotic variceal bleed

›Urgent endoscopy within 12 hours — EVL or sclerotherapy

Underlying cause treatment

›Myeloproliferative neoplasms

›Cytoreductive therapy if MPN identified

›Hydroxyurea 500–1000 mg orally twice daily

›Titrate to normalize WBC and platelet counts

›Target platelet count <400 x10^9/L

›Ruxolitinib for JAK2-positive MPN with inadequate response to hydroxyurea

›Phlebotomy for polycythemia vera

›Target hematocrit <45% (male), <42% (female)

›Paroxysmal nocturnal hemoglobinuria

›Eculizumab — anti-C5 complement inhibitor

›600 mg IV weekly for 4 weeks, then 900 mg every 2 weeks

›Meningococcal vaccination mandatory before initiation

›Dramatically reduces thrombotic risk in PNH-BCS

›Antiphospholipid syndrome

›Warfarin preferred over DOACs for thrombotic APS

›Target INR 2–3; some guidelines suggest 3–4 for recurrent thrombosis

›Hydroxychloroquine as adjunct — 200–400 mg/day

Interventional procedures — stepwise approach

›Step 1: Medical therapy (all patients)

›Anticoagulation + portal hypertension management as above

›Approximately 44% of patients managed with medical therapy alone

›Step 2: Angioplasty with or without stenting

›For short-segment hepatic vein or IVC stenosis/web

›Angioplasty first — stenting reserved for angioplasty failure

›Stenting may compromise future TIPS access — defer if possible

›Success rate >70% in Asian populations with IVC-predominant disease

›Lower success approximately 10% in European populations

›Step 3: TIPS or DIPS

›Indicated if angioplasty fails or not feasible

›PTFE-covered stents preferred — lower occlusion rates

›5-year transplant-free survival 72–78%

›Direct intrahepatic portocaval shunt (DIPS) — transcaval approach

›Required in >40% of BCS patients due to inaccessible hepatic veins

›Performed at tertiary centers with expertise only

›TIPS as bridge to liver transplantation in deteriorating patients

›Step 4: Liver transplantation

›Reserved for TIPS failure or fulminant liver failure

›5-year post-transplant survival 70–92%

›Lifelong anticoagulation post-transplant mandatory

›Recurrent thrombosis in up to 20% post-transplant without anticoagulation

09Special Populations/sp68

Pregnancy

›Epidemiology and risk

›BCS can present during pregnancy or postpartum period

›Pregnancy itself a prothrombotic state — increased VTE risk

›Prevalence approximately 1% of BCS cases are pregnancy-associated

›Fetal outcomes remain poor even when maternal outcomes favorable

›Diagnosis in pregnancy

›Doppler ultrasound — first-line imaging; safe in pregnancy

›MRI without gadolinium if ultrasound equivocal

›Gadolinium avoided — crosses placenta; safety data limited

›CT avoided in first trimester if possible — radiation exposure

›Treatment modifications

›LMWH preferred anticoagulant throughout pregnancy

›Does not cross placenta; safe for fetus

›Warfarin teratogenic in first trimester — AVOID

›Warfarin causes fetal hemorrhage — avoid in third trimester

›DOACs contraindicated in pregnancy — fetotoxic

›Diuretics used cautiously

›Spironolactone — anti-androgenic effects on male fetus; avoid if possible

›Furosemide safer but monitor amniotic fluid volume

›Variceal management — EVL safe in pregnancy, preferred over beta-blockers for prophylaxis

›Nonselective beta-blockers — fetal bradycardia and growth restriction risk; use lowest effective dose

›Delivery planning

›Multidisciplinary planning: hepatology, maternal-fetal medicine, anesthesia

›LMWH bridging around delivery

›Resume anticoagulation within 12–24 hours postpartum

Geriatric

›Epidemiology in older adults

›BCS less common in elderly — typically affects younger patients

›When present in elderly: malignancy-related secondary BCS more likely

›MPN prevalence increases with age — JAK2 testing still essential

›Diagnostic considerations

›Constrictive pericarditis and right heart failure more prevalent — must exclude

›Echocardiography critical in elderly with hepatic congestion

›Chronic liver disease from NAFLD or alcohol — competing diagnosis

›Treatment modifications

›Anticoagulation

›LMWH dose adjustment for reduced renal clearance — CrCl monitoring

›Warfarin — increased bleeding risk; narrow therapeutic window more critical

›Fall risk assessment — head injury risk on anticoagulation

›Diuretics

›Spironolactone — caution with hyperkalemia in renal impairment

›Furosemide — risk of volume depletion and AKI in elderly

›Interventional procedures

›TIPS — age itself not a contraindication if liver function adequate

›Frailty assessment prior to major procedures

›Transplant candidacy — age-related comorbidities limit suitability

Pediatrics

›Epidemiology in children

›Rare in children — etiology differs from adults

›Hypercoagulable states still predominate

›Congenital IVC webs or IVC anomalies more common than in adults

›Sepsis-related hepatic vein thrombosis in neonates

›Etiology in pediatric BCS

›Inherited thrombophilias — factor V Leiden, protein C/S deficiency

›PNH in adolescents

›Inflammatory conditions — Behcet disease, IBD

›Umbilical catheter-related thrombosis in neonates

›Diagnosis in children

›Doppler ultrasound first-line

›MRI preferred over CT to avoid radiation in pediatric population

›Thrombophilia workup adapted — age-appropriate reference ranges

›Treatment in children

›LMWH dosing by weight

›Enoxaparin 1 mg/kg subcutaneously twice daily (therapeutic)

›Monitor anti-Xa 4 hours post-dose — target 0.5–1.0 IU/ml

›Warfarin dosing — variable; weight-based starting dose 0.1–0.2 mg/kg

›Target INR 2–3; frequent INR monitoring required

›TIPS in children — technically feasible at experienced pediatric centers

›Small vessel size — requires experienced interventionalist

›Liver transplantation — outcomes comparable to adults in pediatric series

10Background/b70

Epidemiology

›Incidence and prevalence

›Incidence 0.5–2 per million in Western countries

›Incidence 5–7 per million in Asia — IVC disease predominates

›Rare condition overall — high index of suspicion required

›Most commonly affects younger patients (mean age 30s–40s)

›Etiology distribution

›Myeloproliferative neoplasms — 40–50% of cases

›Oral contraceptive use — approximately 22%

›Antiphospholipid syndrome — 10–12%

›PNH — 7–12%

›Multiple prothrombotic conditions coexist in 25–46%

›No cause identified in approximately 15–20% (idiopathic)

›Outcomes

›5-year overall survival with stepwise management: 77–82%

›Transplant-free survival with TIPS: 72–78% at 5 years

›Post-transplant 5-year survival: 70–92%

›Annual HCC incidence approximately 4% in Western populations

›Fulminant BCS — transplant-free survival historically 37–42%

Pathophysiology

›Mechanism of venous obstruction

›Thrombosis of hepatic venous outflow tract

›Involvement from small intrahepatic venules to hepatic vein-IVC junction

›Caudate lobe drains directly into IVC — spared in most cases, causing compensatory hypertrophy

›Obstruction types

›Primary BCS: Thrombotic — most common in Western populations

›Secondary BCS: Extrinsic compression, malignant invasion, membranous obstruction

›IVC-predominant disease: More common in Asian populations — IVC webs or membranes

›Hepatic consequences of outflow obstruction

›Sinusoidal congestion

›Increased sinusoidal pressure causes hepatocyte injury

›Zone 3 (pericentral) hepatocyte necrosis — "congestive hepatopathy"

›Progressive fibrosis leading to cirrhosis in chronic BCS

›Portal hypertension

›Elevated hepatic sinusoidal pressure transmitted to portal system

›Ascites, varices, splenomegaly, encephalopathy

›Hepatic arterial buffer response

›Hepatic arterial flow increases compensatorily

›Caudate lobe preferentially perfused — hypertrophy

›Thrombosis pathophysiology

›Virchow triad application in BCS

›Hypercoagulability: MPN, thrombophilias, OCP use

›Stasis: Post-surgical, post-partum

›Endothelial injury: Less relevant than hypercoagulability

›JAK2 V617F mutation in MPN-associated BCS

›Gain-of-function mutation in JAK-STAT signaling pathway

›Drives clonal hematopoiesis with thrombotic phenotype

›JAK2-positive BCS may have low allele burden — next-generation sequencing improves detection

Therapeutic Considerations

›Rationale for stepwise approach

›Step-up strategy: Medical — angioplasty — TIPS — transplant

›Recommended by ACG 2020 Clinical Guideline and AASLD 2021 Practice Guidance

›Goal: Restore hepatic venous outflow with least invasive effective intervention

›Anticoagulation rationale

›Prevents thrombus propagation and promotes recanalization

›Recommended even in absence of identified prothrombotic disorder

›Most patients require lifelong anticoagulation

›Hepatic synthetic failure complicates monitoring — factor Xa assay preferred over INR alone

›TIPS mechanism in BCS

›Creates intrahepatic shunt bypassing obstructed hepatic veins

›Decompresses portal hypertension without requiring patent hepatic veins

›PTFE-covered stents have superior patency rates vs. bare metal

›Encephalopathy rate after TIPS: 20–30%; manageable with lactulose/rifaximin

›HCC surveillance rationale

›BCS confers HCC risk comparable to viral cirrhosis

›Annual incidence approximately 4% in Western populations

›HCC diagnosis in BCS requires histological confirmation

›Perturbed vascular architecture makes imaging criteria unreliable

›Ultrasound + AFP every 6 months — standard surveillance protocol

›Monitoring liver treatment response

›Liver elastography: Values decrease as congestion improves with treatment

›No validated timeline for escalating therapy — decisions based on clinical trajectory

›Biochemical response: Improving bilirubin, albumin, INR indicates treatment success

11Patient Discharge Instructions/pdi27

copy discharge instructions

›Your diagnosis and condition

›You have been diagnosed with Budd-Chiari syndrome — a rare condition where the veins draining blood from your liver become blocked by a blood clot

›This causes increased pressure in your liver and can lead to fluid accumulation, jaundice, and liver damage

›You will need ongoing specialist care and monitoring for this condition

›Your medications

›Take all prescribed blood thinners exactly as directed — do not stop without consulting your doctor

›If prescribed warfarin: regular blood tests (INR checks) are required to ensure safe dosing

›If prescribed water pills (diuretics): take as prescribed and limit salt intake to 2 grams per day

›Do not take NSAIDs (ibuprofen, naproxen, aspirin) — they increase bleeding risk and can worsen kidney function

›Diet and lifestyle

›Strict low-salt diet: avoid processed foods, canned goods, restaurant food, and adding salt

›Avoid alcohol completely — further liver damage is life-threatening in this condition

›Adequate protein intake is important — do not follow a low-protein diet without medical guidance

›Fluid restriction only if your doctor specifically instructs you

›Follow-up appointments

›Hepatology (liver specialist) appointment within 1–2 weeks — mandatory

›Hematology (blood specialist) appointment for blood disorder workup — scheduled

›Blood tests as directed by your doctor

›Liver ultrasound every 6 months for liver cancer surveillance

›Return to emergency department immediately for

›Vomiting blood or passing black/tarry stools — variceal hemorrhage

›Rapidly increasing abdominal swelling or severe abdominal pain

›Confusion, excessive drowsiness, or inability to be roused — encephalopathy

›New or worsening yellowing of skin or eyes

›Fever with abdominal pain — possible infection in abdominal fluid

›Signs of bleeding: unusual bruising, blood in urine, or prolonged bleeding from cuts

›Sudden swelling of one or both legs

12References/r40

Guidelines and key sources

›ACG Clinical Guideline: Disorders of the Hepatic and Mesenteric Circulation (2020)

›Simonetto DA, Singal AK, Garcia-Tsao G, et al.

›American Journal of Gastroenterology. 2020

›DOI: 10.14309/ajg.0000000000000486

›Provides stepwise management algorithm and diagnostic recommendations

›AASLD Practice Guidance: Vascular Liver Disorders and Portal Vein Thrombosis (2021)

›Northup PG, Garcia-Pagan JC, Garcia-Tsao G, et al.

›Hepatology. 2021

›DOI: 10.1002/hep.31646

›Evidence-based guidance on anticoagulation and interventional management

›Primary Budd-Chiari Syndrome — NEJM Review (2023)

›Garcia-Pagan JC, Valla DC

›New England Journal of Medicine. 2023

›DOI: 10.1056/NEJMra2207738

›Comprehensive review of pathophysiology, diagnosis, and treatment

Landmark studies

›Budd-Chiari Syndrome: Presentation, Management, and Prognosis (2025)

›Amjad W, Thuluvath PJ.

›American Journal of Gastroenterology. 2025

›PMID: 41384820

›Updated epidemiology, prothrombotic risk data, and management outcomes

›Diagnostic Accuracy of Doppler Ultrasound, CT and MRI in Budd-Chiari Syndrome (2020)

›Gupta P, Bansal V, Kumar-M P, et al.

›British Journal of Radiology. 2020

›PMID: 32150462

›Meta-analysis: MRI AUC 90.8%; Doppler sensitivity 85–89%

›Myeloproliferative Neoplasms in Budd-Chiari Syndrome — Meta-Analysis (2012)

Evidence & Review

Evidence & Review