Abdominal surgery in patients with chronic extra-hepatic portal vein obstruction
This retrospective case-control study aims to evaluate the outcome after abdominal surgery in patients with non-cirrhotic chronic extra-hepatic portal vein obstruction (EHPVO), when compared to patients without EHPVO. Patients with known EHPVO who underwent abdominal surgery between 2000 and 2020 will be included, and compared with patients without EHPVO who had a similar surgical intervention. A special attention will be paid to portal-hypertension related complications, and bleeding complications.
- To retrospectively assess the outcome of adult patients with EHPVO who underwent abdominal surgery (except surgical portosystemic shunt).
- To retrospectively assess the impact of portal decompression (either surgical portosystemic shunt or radiological shunt) before or at the time of surgery on postoperative outcome.
Long-term outcome of patients with Budd-Chiari Syndrome
Background: Budd Chiari Syndrome is a rare vascular liver disease with major deleterious complications if not treated and intervened on time. Current literature recommends stepwise treatment of BCS, based on a progressively escalating invasiveness with a good overall Overt Liver Transplantation free survival.
However currently there are no definite consensus on long-term follow-up studies to aid to protocolize management on the choice of best treatment for each individual and the correct timing on when make the clinical decision of stepping up on the treatment algorithm.
Methods: European Retrospective multicentric study
AIMS AND PURPOSE OF THE STUDY:
- To assess the long-term outcome of patients with BCS.
- To evaluate the incidence and predictive factors of interventions in patients with BCS on the long-term outcome.
- Establish the best treatment for patients with BCS that initially received a TIPS in whom during follow-up an asymptomatic thrombosis/dysfunction of TIPS occurs.
Incidence and predictive factors of recurrent thrombosis in non-cirrhotic portal vein thrombosis. VALIDATION COHORT.
Background: Recurrent thrombosis (or rethrombosis, RT) is one of the main threats of chronic non-cirrhotic portal vein thrombosis (NC-PVT). Clinical guidelines only recommend long-term anticoagulation to prevent rethrombosis in patients with a recognized prothrombotic disorder or with a previous history of intestinal venous ischemia or thrombosis.
To describe the rate of rethrombosis in NC-PVT and to identify its predictive factors.
Previous results: We included 114 patients with NC-PVT due to myeloproliferative neoplasm (n=29), thrombophilia disorders (n=12), exclusively local factors (n=36) or idiopathic PVT (n=37). According to current guidelines, patients with prothrombotic disorders were on long-term anticoagulation (except for those with specific contraindications) while most patients with idiopathic/local factor were not anticoagulated. Patients were followed-up with regular scheduled imaging studies, and RT was identified in 17 patients, with a cumulative probability of rethrombosis of 2.6%, 12.9% and 19.2% at 1, 5 and 10 years respectively. Most RT (82%) occurred in the subgroup of patients with idiopathic/local etiology not receiving long-term anticoagulation. In this subpopulation, factor V ≥90% and factor VIII≥150% were independently associated with RT.
VALIDATION COHORT: We aim at validating these results that suggest that patients with idiopathic or associated with chronic PVT not receiving long term anticoagulation that present high levels of Factor V and/or factor VIII are at higher risk of re-thrombosis.
Methods: enrollment of patients with idiopathic/local factor chronic PVT that have not received long-term anticoagulation in which the extension of the thrombosis has been thoroughly established at baseline and that have had a periodical imaging follow-up that ensured the detection of asymptomatic re-thrombosis during follow-up.
Regarding the determination of factor V and VIII, as they are not routinely included in most thrombophilia studies these patients will need to have a biobank plasma sample to do this analysis. The biobank sample should have been obtained not long after the chronic PVT diagnosis.
Percutaneous Portal Vein Recanalization using Self-Expandable Nitinol Stents in Patients with Non-cirrhotic Non-tumoral Portal Vein Occlusion
This retrospective study tries to determine when portal vein recanalization (PVR) is useful in case of symptomatic portal vein occlusion (PVO). Based on previous experience, portal vein recanalization (PVR) could be a therapeutic option in patients with symptomatic portal vein obstruction (PVO) when at least a portion of the segmental branches of the largest liver segments (V or VIII) remained patent. In the present study, we would like extend our experience in this field by collecting data from patients with PVO from the European vascular disease of the liver group VALDIG. Patients with PVO who underwent PVR after 2010 will be included. Data from patients with PVO from the European vascular disease of the liver group VALDIG will be retrospectively reviewed and follow-up data will be updated. We will classify patients according to the vascular extent of PVO before PVR and assess long-term stent patency in patients with and without TIPS insertion, in those who received and who did not receive anticoagulation therapy, and to the type and duration of treatment.
The main aim of this study will be to validate the usefulness of the classification of PVO according to the vascular extent of PVO to decide when PVR is feasible. In addition, we also aim to assess the effect of anticoagulation therapy on long-term stent patency and to assess the benefit of TIPS in addition to PVR on long-term stent patency.
Development of portal hypertension after acute portal vein thrombosis. A long-term follow-up study
Background: A few studies have evaluated the long-term evolution of patients after APVT. Previously studies the follow-up was relatively short (a median of 4 years follow-up in patients with a much longer life expectancy).
Hypothesis: Patients with APVT have a risk of developing long-term PHS, regardless of recanalization. Also, recanalized patients can develop PHS due to liver parenchyma distortion after APVT, presence of portal sinusoidal vascular disease, micro-thrombosis or failure to identify small vessel thrombosis by conventional imaging studies.
• Main Objective of the study : Evaluate in a large cohort of patients with Non-cirrhotic APVT, with at least 12m of follow up after diagnosis, the risk of developing PHS in relation to the degree of recanalization achieved with treatment.
• Objective 2: Identificacion of factors predicting recanalization, survival, high-risk esophageal varices, variceal bleeding, ascites, hepatic encephalopathy, portal cholangiopathy, presence of porto-systemic shunts and re-thrombosis episodes.
• One of main inclusion criteria: Is to have one contrast-enhanced CT scan or MRI staging the extension of APVT at diagnosis and other image study during follow-up, more than 12m.
Menopause, female hormones and PSVD
Background: Although the exact pathogenetic mechanism of PSVD is not fully understood but a possible key element is formation of microthrombi in the small portal branches causing presinusoidal portal hypertension.
Before menopause, women are reasonably protected against venous thrombosis by their circulating estrogens; nevertheless, this protection ends after menopause.
On the other hand hormonal therapy (oral contraceptives and postmenopausal replacement therapy) increases the risk of venous thromboembolism and whether it may impact PSVD development is unknown.
- Evaluate the impact of female hormones in PSVD onset/progression
- Evaluate the association between PSVD onset and menopausal state
- Evaluate the frequency of hormone therapy in women with PSVD, both in pre and postmenopausal women
- Determine the impact of hormone therapy in the severity of PSVD
Natural history of Portosinusoidal Vascular Disease (PSVD) and Liver Transplantation
Background: Patients with PSVD usually have preserved liver function, however sometimes liver transplantation is required.
Factors predicting the need of OLT and the real applicability in IPH patients are not known.
Post-OLT outcome has never been reported in a large cohort of patients.
Methods: European Retrospective multicentric study
- To evaluate in a large international cohort of consecutive patients diagnosed of IPH followed in centers of the VALDIG.
- Factors predicting the need of OLT and the applicability in real life.
- Evaluate the long term outcome of patients with PSVD that had been submitted to LT (survival, recurrence..).
Medically assisted reproduction in women with vascular liver diseases
This retrospective study tries to determine the safety and efficacy of medically assisted reproduction in women with known Budd-Chiari syndrome, portal vein thrombosis or porto-sinusoidal vascular disease. Women with vascular liver diseases who underwent medically assisted reproduction between 2000 and 2018 will be included. This includes controlled pharmacologic stimulation of ovarian follicles, paired or not with assisted reproduction technologies. Vascular liver disease should be diagnosed prior to medically assisted reproduction.
The aim of this retrospective study is thus to determine the safety and efficacy of medically assisted reproduction in women with known Budd-Chiari syndrome, portal vein thrombosis or vascular porto-sinusoidal disease.
PNH and Splanchnic Vein Thrombosis
Data from patients with PNH and splanchnic vein thrombosis from the European vascular disease interest group VALDIG and French vascular network will be retrospectively reviewed. Only patients with a diagnosis of splanchnic vein thrombosis made between 2000 and 2016 will be included.
We will compare the evolution of thrombosis in patients who received a “curative” treatment for PNH, including eculizumab and bone marrow transplantation, to patients who did not receive one of these 2 types of treatments, either because it was not available or when the patient did not have HLA compatible donor.
VALDIG Database and Registry
Vascular diseases of the liver are a heterogeneous group of rare and if untreated fatal disorders that include portal vein thrombosis, Budd-Chiari syndrome, non-cirrhotic portal hypertension, sinusoidal obstruction syndrome, hereditary hemorrhagic teleangiectasia and others.
Current knowledge about patients suffering from these disorders is insufficient and no biological samples are available to study the mechanisms of these diseases and the effects of treatments.
Therefore, with the support of a grant from EASL, we are running this cohort study by including and following up patients on the platform redcap.ctu.unibe.ch. If you are member of VALDIG you can request a password and username (contact firstname.lastname@example.org) to participate to this study by including patients from your center into the database (a complete set of data) or into the registry (a limited set of data).
- To characterize the population with vascular liver disease in Switzerland by defining history, outcome and risk factors.
- To build a bio-bank with blood from these patients.
For more information and the study descriptions see here