Diagnostic Role of the Hepatic Venous Pressure Gradient and Elastography in Porto-sinusoidal Vascular Disorder With Portal Hypertension
Hepatic vein pressure gradient measurement
+ Ultrasound-guided percutaneous liver biopsy
+ Multiparametric Abdominal Magnetic Resonance with Elastography
Idiopathic Noncirrhotic Portal Hypertension+1
+ Digestive System Diseases
+ Hypertension, Portal
Diagnostic Study
Summary
Study start date: March 1, 2023
Actual date on which the first participant was enrolled.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. Primary objectives are: * To describe the measurement of the hepatic venous pressure gradient (in mmHg) in patients with portal hypertension and transient hepatic elastography ≤ 20 kPa. * To describe hepatic (in kPa) and splenic (in kPa) stiffness measured by magnetic resonance elastography in patients with portal hypertension and transient hepatic elastography ≤ 20 kPa. * To describe the frequency of major histological findings for the diagnosis of portal sinusoidal vascular disorder (obliterative portal venopathy, regenerative nodular hyperplasia and incomplete septal cirrhosis) in patients with portal hypertension and transient hepatic elastography ≤ 20 kPa. Secondary objectives are: * To describe the frequency of hepatic vein-to-vein communications in patients with portal hypertension and transient hepatic elastography ≤ 20 kPa. * To describe the frequency of minor histological findings for the diagnosis of portal sinusoidal vascular disease (portal tract abnormalities, architectural disturbances, nonzonal sinusoidal dilatation, mild perisinusoidal fibrosis) in patients with portal hypertension and transient hepatic elastography ≤ 20 kPa. * To compare the serum values of von Willebrand antigen factor (IU/mL) between patients diagnosed with porto-sinusoidal vascular disorder and those diagnosed with cirrhosis, after analysis of liver biopsy. * To compare the serum titers of procollagen III amino-terminal peptide (mcg/l) between patients diagnosed with portosinusoidal vascular disorder and those diagnosed with cirrhosis, after analysis of liver biopsy. * To compare the serum titers of anti-endothelial cell antibodies between patients diagnosed with portosinusoidal vascular disorder and those diagnosed with cirrhosis, after analysis of liver biopsy. 50 patients will be included, prospectively and retrospectively, in a comparative study between diagnostic methods, with a cross-sectional design.
Protocol
This section provides details of the study plan, including how the study is designed and what the study is measuring.50 patients to be enrolled
Total number of participants that the clinical trial aims to recruit.Diagnostic Study
Eligibility
Researchers look for people who fit a certain description, called eligibility criteria: person's general health condition or prior treatments.Any sex
Biological sex of participants that are eligible to enroll.From 18 to 80 Years
Range of ages for which participants are eligible to join.Healthy volunteers not allowed
If individuals who are healthy and do not have the condition being studied can participate.Conditions
Pathology
Criteria
Inclusion Criteria: * Age ≥ 18 years; * Patients with specific signs of portal hypertension: 1. Endoscopic: esophagogastric/ectopic varices; 2. 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: 1. Pregnancy 2. Allergy to iodine 3. Chronic kidney disease with creatinine clearance \< 50 ml/min 4. Anticoagulation 5. RNI \> 1.5 6. Platelets \< 50,000/mm3 * Confounding factors: 1\. Hepatitis C treated with SVR * Conditions that exclude the diagnosis of PSVD: 1. History of bone marrow transplant 2. Budd-Chiari 3. Congestive heart failure or Fontan surgery 4. Abernethy's Syndrome 5. Hereditary hemorrhagic telangiectasia 6. Chronic cholestatic diseases 7. Neoplastic hepatic infiltration 8. Sarcoidosis 9. Congenital hepatic fibrosis 10. Hepatosplenic schistosomiasis 11. Portal cavernoma / thrombosis with complete occlusion of the main portal vein.
Study Plan
Find out more about all the medication administered in this study, their detailed description and what they involve.One single intervention group is designated in this study
This study does not include a placebo group
Treatment Groups
Group I
ExperimentalStudy Objectives
Primary Objectives
Secondary Objectives
Study Centers
These are the hospitals, clinics, or research facilities where the trial is being conducted. You can find the location closest to you and its status.This study has 1 location
Hospital Universitário Clementino Fraga Filho / Universidade Federal do Rio de Janeiro (UFRJ)
Rio de Janeiro, BrazilOpen Hospital Universitário Clementino Fraga Filho / Universidade Federal do Rio de Janeiro (UFRJ) in Google Maps