Multiparametric Ultrasound for the Noninvasive Diagnosis of Porto-sinusoidal Vascular Liver Disorder

Inclusion criteria

- 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;
• Ability to understand the study information and provide written informed consent;

Inclusion criteria

- Cirrhosis group

• Diagnosis of liver cirrhosis confirmed by liver histology or, alternatively, by compatible findings on imaging, laboratory tests, and physical examination together with a positive history for at least one known cause of chronic liver disease;
• 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;
• Ability to understand the study information and provide written informed consent.

Exclusion criteria

- 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;
• Presence of primary or secondary malignant liver tumors;
• Presence of a transjugular intrahepatic portosystemic shunt (TIPS) device;
• Congenital anomalies of the liver or biliary tract;
• History of heart failure;
• Contraindications to administration of SonoVue (sulfur hexafluoride microbubbles), including: prior allergic reaction to the active substance or any excipients, known right-to-left shunts, severe pulmonary hypertension (pulmonary artery pressure > 90 mmHg), uncontrolled systemic hypertension, or adult respiratory distress syndrome;
• Inadequate sonographic visualization of the right hepatic lobe;
• Pregnancy.

Exclusion criteria

- Cirrhosis group

• Decompensated cirrhosis or Child-Pugh class C;
• Cryptogenic cirrhosis;
• Presence of other causes of portal hypertension (same list as for PSVD exclusions: history of bone marrow transplantation, Budd-Chiari syndrome or hepatic venous outflow obstruction, hepatic schistosomiasis, Abernethy malformation, hereditary hemorrhagic telangiectasia, sarcoidosis, congenital hepatic fibrosis, chronic cholestatic diseases);
• Presence of portal, spleno-mesenteric, or hepatic vein thrombosis;
• Prior hepatic or splenic surgery;
• Presence of primary or secondary malignant liver tumors;
• Presence of a transjugular intrahepatic portosystemic shunt (TIPS) device;
• Congenital anomalies of the liver or biliary tract;
• History of heart failure;
• Contraindications to administration of SonoVue (sulfur hexafluoride microbubbles), including: prior allergic reaction to the active substance or any excipients, known right-to-left shunts, severe pulmonary hypertension (pulmonary artery pressure > 90 mmHg), uncontrolled systemic hypertension, or adult respiratory distress syndrome;
• Inadequate sonographic visualization of the right hepatic lobe;
• Pregnancy.