Porto-sinusoidal vascular disease (PSVD) is a rare clinical entity characterized by significant portal hypertension in the absence of cirrhosis on liver histology, which may or may not show specific alterations of the portal vein, sinusoids, or hepatic lobular architecture. Currently, diagnosis of this condition necessarily requires a liver biopsy and, despite some differences detected on imaging studies-and particularly on liver and spleen elastography-PSVD remains indistinguishable from cirrhosis using non-invasive tests.
Contrast-enhanced ultrasound (CEUS) is an easy-to-perform, repeatable, and cost-effective examination that enables real-time assessment of parenchymal or focal liver lesion perfusion. Moreover, the application of dynamic contrast-enhanced ultrasound (DCE-US-i.e., contrast-enhanced ultrasound followed by quantitative perfusion analysis using dedicated software, such as the VueBox Software that will be used in this study) allows integration of CEUS qualitative assessment with quantitative evaluation of tissue perfusion through analysis of time-intensity curves generated during contrast transit. From this analysis, several perfusion-related parameters can be derived (for example, peak enhancement, time to peak, or area under the curve), which have already proven useful in improving differential diagnosis of focal liver lesions and in predicting treatment response and systemic therapy outcomes.
To date, the use of DCE-US for the diagnosis of PSVD has not yet been described; however, based on the underlying histological alterations associated with this disease, it is reasonable to hypothesize that parameters obtained with this technique in the liver parenchyma of patients with PSVD may differ from those measured in patients with liver cirrhosis. The aim of the present project is to apply DCE-US in patients with PSVD and in patients with cirrhosis to evaluate potential significant differences in perfusion parameters, and to assess the feasibility of a non-invasive differential diagnosis between the two conditions using this technique in combination with elastography and bidimensional ultrasound data to develop a multiparametric diagnostic score.
PSVD group
Histologically confirmed diagnosis of porto-sinusoidal vascular disease (PSVD);
Presence of clinically significant portal hypertension, evidenced by at least one specific sign of portal hypertension (e.g., imaging evidence of collateral circulation or porto-systemic shunts, endoscopic evidence of esophageal or gastric varices, or history of gastrointestinal variceal bleeding);
Age ≥ 18 years;
PSVD group (cases)
Presence of other causes of portal hypertension, including but not limited to: history of bone marrow transplantation, Budd-Chiari syndrome or hepatic venous outflow obstruction, hepatic schistosomiasis, Abernethy malformation, hereditary hemorrhagic telangiectasia, sarcoidosis, congenital hepatic fibrosis, or chronic cholestatic liver diseases;
Presence of portal, spleno-mesenteric, or hepatic vein thrombosis;
Prior hepatic or splenic surgery;
Parallel
Evaluation of quantitative perfusion parameters at dynamic contrast-enhanced ultrasound through Vuebox Software
DevicePatients from the two arms will undergoing dynamic contrast-enhanced ultrasound, and CEUS videoclips will be analyzed through the software VueBox to evaluate perfusion parameters. These parameters will be compared among the two arms and integrated with other ultrasound-derived data to analyze differences and to elaborate a multiparametric diagnostic score
Peak enhancement (PE) measured on DCE-US (VueBox)
Maximum intensity of the signal in the time-intensity curve at dynamic contrast-enhanced ultrasound
Time to peak (TTP) measured on DCE-US (VueBox)
The time required from the beginning of the exam to reach the peak enhancement
Area under the time-intensity curve (AUC) measured on DCE-US (VueBox)
The total area under the time-intensity curve reflecting the quantity of contrast media passed through the region of interest
Wash-in rate (slope) measured on DCE-US (VueBox)
Tangent at the ascending part of the time-intensity curve
Diagnostic sensitivity and specificity of predefined perfusion parameter cut-offs for identifying PSVD (ROC analysis)
Differences among PSVD and cirrhosis in perfusion parameters will be analyzed in order to identify the cut-offs with the best sensitivity and specificity to identify PSVD from DCE-US.
Performance of a multiparametric diagnostic score combining DCE-US parameters, liver stiffness, spleen stiffness, and B-mode ultrasound features
The mentioned cut-offs in DCE-US parameters will be combined with the bidimensional ultrasound data, Doppler data and elastography data to elaborate a noninvasive diagnostic score to predict the presence of PSVD
Correlation between quantitative DCE-US parameters and risk of portal vein thrombosis at 12 months.
DCE-US parameters will be correlated with the incidence of portal vein thrombosis in order to detect if these parameters may be predictive of developing this condition, potentially allowing to identify the cohort of patients who would benefit more from prophylactic anticoagulation
Incidence of portal vein thrombosis at 12 months
The proportion of patients developing porta vein thrombosis at 12 months
Click on the lead sponsor to view all of their trials.
This trial is not recruiting at the moment. You can still explore other options:
