Pathophysiology of Primary Budd-Chiari Syndrome: A Narrative Review

Area of Science:

• Hepatology
• Vascular Medicine
• Gastroenterology

Background:

• Budd-Chiari syndrome (BCS) is a rare hepatic vascular disorder characterized by hepatic venous outflow obstruction.
• It leads to sinusoidal congestion, portal hypertension, and centrilobular necrosis, modeling postsinusoidal portal hypertension.

Purpose of the Study:

• To provide an integrated analysis of BCS pathophysiology based on obstruction site.
• To highlight etiopathogenesis, clinical phenotypes, and epidemiologic variations.
• To emphasize diagnostic and management strategies for improved patient outcomes.

Main Methods:

• Review of pathophysiology based on obstruction site (major hepatic veins, small veins, inferior vena cava).
• Analysis of compensatory mechanisms, clinical phenotypes, and prothrombotic states.
• Emphasis on diagnostic tools (Doppler ultrasonography, CT, MRI) and management (anticoagulation, TIPS, transplantation).

Main Results:

• Pathophysiology varies by obstruction site, influencing clinical presentation from asymptomatic to acute/chronic forms.
• Prothrombotic states (myeloproliferative neoplasms, thrombophilias) and acquired conditions are key etiologies.
• Diagnostic accuracy and therapeutic decisions are enhanced by understanding these mechanisms.

Conclusions:

• Understanding BCS pathophysiology, etiopathogenesis, and clinical variability is crucial.
• A stepwise diagnostic and management approach, including early anticoagulation and TIPS, improves survival.
• Further research into epidemiologic variations can refine clinical implications.