Background
In 2019, the term porto-sinusoidal vascular disorder (PSVD) was proposed to describe a group of rare vascular diseases of the liver featuring lesions encompassing the portal venules and sinusoids, irrespective of the presence or absence of portal hypertension (1).
The pathogenesis of PSVD is unknown but relies on the development of vascular changes within the liver. Associated conditions, including disorders of immunity, blood diseases and prothrombotic conditions, infections, congenital or familial defects, and drug exposure have been documented in about 50% of patients with PSVD (1,2). In some cases, more than one of these conditions can coexist.
Over the past few years, our hepatology unit has observed several cases of recurrent portal hypertension following liver transplantation, that was attributed to PSVD. There is currently no knowledge on this very specific condition. Our observations serve as the basis for proposing this study, with the aim of identifying the risk factors associated with this disorder, assess the disease course, evaluate the histological profile, and assess patient outcome.
Inclusion criteria
- Patients with PSVD and portal hypertension that developed after liver transplantation
- PSVD is defined according to internationally adopted diagnostic criteria:
- 1 specific sign of portal hypertension: gastric, esophageal or ectopic varices, portal hypertensive bleeding, porto-systemic collaterals on imaging
OR
- 1 specific histological lesion of PSVD: obliterative portal venopathy, nodular regenerative hyperplasia, incomplete septal fibrosis
OR
- 1 non-specific sign of portal hypertension (ascites, platelet count <150 000/mm3, spleen size >= 13 cm in largest axis) AND 1 non-specific histological lesion of PSVD (portal tract abnormalities, architectural disturbance, non-zonal sinusoidal dilatation, mild perisinusoidal fibrosis)
Study design and data collection
This is multicentric retrospective study.
Patients who were transplanted till 30/06/2024 and who developed PSVD with portal hypertension post-transplantation will be identified from local clinical and liver pathology databases. Participating centers will be asked to review the pathology reports of liver biopsies performed in their population of liver transplantation recipients to limit the risk of selection bias.
Data will be retrieved from the medical records of the included patients. Data will be pseudonymized. Follow-up will start at the time of diagnosis of PSVD and will end on 30/09/2024. The following data categories will be retrospectively collected by the local investigators and centralized for analysis by the principal investigator of this study:
- Demographics data: age, sex, etiology and severity of liver disease, …
- Medical history / risk factors for PSVD: myeloproliferative disorders, coagulation disorders, systemic or auto-immune comorbidities, prior cancer therapies, …
- Data regarding the liver transplantation, peri- and post-operative period: date, type of graft, complications, revisions, rejections, …
- Data regarding the donor: age, cause of death, …
- Data regarding immunosuppression and other medication history
- Data related to the recurrence of portal hypertension post-liver transplantation: clinical signs, HVPG measurements, …
- Histological data: report of liver biopsy or explant liver in case of re-transplantation
- Data related to the management of portal hypertension: NSBB, diuretics, TIPS, re-transplantation, …
- Survival