Hepatic Venous Pressure Gradient and Elastography in Porto-sinusoidal Vascular Disorder

Porto-sinusoidal vascular disorder (PSVD) is considered a rare cause of portal hypertension (PH), resulting from specific histological alterations that essentially affect the small portal branches and sinusoids, in the absence of cirrhosis.In recent years, the recognition and importance of PSVD has increased, notably due to the widespread use of transient elastography (TE). However, the definitive diagnosis of PSVD can only be established through liver biopsy. Recent data show that PSVD should be suspected in patients with PH and TE ≤ 20 kPa and liver biopsy should be considered in this context.The investigators hypothesize that hepatic venous pressure gradient (HVPG) and magnetic resonance liver elastography (MRE) may help in the selection of liver biopsy candidates for the diagnosis of PSVD.The primary objective of the study is to describe HVPG and MRE values and liver biopsy findings in patients with PH and TE ≤ 20 kPa. The search for serum markers that can distinguish these patients from those with cirrhotic portal hypertension without the need for liver biopsy will also be the object of this study.50 patients will be included, prospectively and retrospectively, in a comparative study between diagnostic methods, with a cross-sectional design.

Inclusion Criteria:Age ≥ 18 years;Patients with specific signs of portal hypertension:Endoscopic: esophagogastric/ectopic varices;On imaging (US, CT or MRI): portosystemic collateral veins;Transient hepatic elastography with valid values ≤ 20 kPa;Signed written informed consent form.Exclusion Criteria:Contraindications to HVPG or percutaneous liver biopsy:PregnancyAllergy to iodineChronic kidney disease with creatinine clearance < 50 ml/minAnticoagulationRNI > 1.5Platelets < 50,000/mm3Confounding factors:1. Hepatitis C treated with SVRConditions that exclude the diagnosis of PSVD:History of bone marrow transplantBudd-ChiariCongestive heart failure or Fontan surgeryAbernethy's SyndromeHereditary hemorrhagic telangiectasiaChronic cholestatic diseasesNeoplastic hepatic infiltrationSarcoidosisCongenital hepatic fibrosisHepatosplenic schistosomiasisPortal cavernoma / thrombosis with complete occlusion of the main portal vein.