Noncirrhotic portal hypertension: current trends and future directions

Noncirrhotic portal hypertension (NCPH) comprises a diverse group of vascular liver disorders characterized by elevated portal pressure without cirrhosis. Due to overlapping clinical features, distinguishing NCPH from cirrhosis and porto-sinusoidal vascular disorder (PSVD) remains challenging. This review explores recent advancements in diagnosis, differentiation, and evolving treatment strategies.

NCPH is characterized by preserved liver function and near-normal hepatic venous pressure gradients (HVPG). It shares risk factors with PSVD, including infections, drugs, toxins, and prothrombotic conditions. Diagnostic advancements, such as liver stiffness measurement (LSM) and splenic stiffness measurement (SSM), offer noninvasive differentiation from cirrhosis, while liver biopsy remains crucial for confirming PSVD and noncirrhotic portal fibrosis (NCPF). Imaging is reliable for diagnosing extrahepatic portal vein obstruction (EHPVO). Transjugular intrahepatic portosystemic shunts (TIPS) for refractory variceal bleeding or ascites, achieving rebleeding control in 72-80% of cases. Surgical shunts and splenectomy remain essential for uncontrolled bleeding and portal biliopathy, demonstrating excellent variceal control (93-95%).

NCPH requires a high index of suspicion for diagnosis. Differentiation from cirrhosis and PSVD relies on clinical, histological, and hemodynamic assessments. Management focuses on endoscopic, interventional, and surgical strategies tailored to disease severity. Future research should standardize diagnostic criteria, explore targeted therapies, and refine prognostic tools to improve outcomes.