Valdig Projects

Background: Cerebral venous thrombosis (CVT) and splanchnic vein thrombosis (SVT) share prothrombotic factors, but it is unclear whether patients with both events represent a distinct phenotype with different prognosis than isolated SVT. Objective: To describe clinical/etiological characteristics of SVT+CVT and compare presentation and SVT-related outcomes versus isolated SVT under identical eligibility criteria. Methods: Retrospective observational cohort study using cross-linked databases...

Background: Portal cholangiopathy is a frequent but incompletely characterized complication of extrahepatic portal vein thrombosis, predominantly affecting non-cirrhotic patients. While biliary abnormalities are commonly detected on imaging, clinically relevant disease is largely restricted to grade III portal cholangiopathy. Evidence regarding the natural history and optimal management of symptomatic disease remains limited, and therapeutic strategies are heterogeneous. In recent years,...

Project Summary Background Chronic obliterative portal vein thrombosis (PVT) remains one of the most challenging vascular conditions in liver transplantation, particularly in patients with Yerdel grade III–IV disease. Although historically regarded as a relative contraindication, advances in endovascular techniques now allow for portal vein recanalization combined with TIPS (PVR-TIPS). This approach restores physiological portal flow, facilitates end-to-end portal anastomosis, and may improve...

1. RESEARCH QUESTIONS The term porto-sinusoidal vascular disorder (PSVD) describes a group of vascular diseases of the liver featuring typical histologic lesions of the portal venules and hepatic sinusoids often causing portal hypertension in the absence of cirrhosis (1). Although this condition is considered a rare disease, homogenization in nomenclature and increasing awareness for this condition have fuelled recent reports on an unexpectedly high prevalence of histologic PSVD features,...

Background Vascular liver diseases (VLD) such as porto-sinusoidal vascular disorder (PSVD), Budd-Chiari syndrome (BCS), and non-cirrhotic portal vein thrombosis (NCPVT) are rare but potentially severe conditions. While hormonal therapies are a known risk factor for VLD in cisgender individuals, data on transgender individuals undergoing gender-affirming hormone therapy (GAHT) remain anecdotal, mainly due to the rarity of these conditions. Unlike in cisgender patients, where hormonal therapy is...

The COST action EURO-VALDI-NET aims to create a pan-European multidisciplinary co-operative network of stakeholders, bringing together scientists, clinicians, industry partners, and patients associations, to address the vascular liver diseases problems. Through the creation of shared data registries on main relevant basic and clinical aspects, conference calls, meetings, workshops, as well as training schools, this Action will coordinate efforts aiming at advancing the understanding of...

Chronic non-cirrhotic extrahepatic portal vein obstruction (CNC-EHPVO) is a rare condition that can lead to severe portal hypertension and its associated complications, including gastrointestinal bleeding, portal cholangiopathy, ascites, and extension or recurrence of portal thrombosis. Managing these complications is particularly challenging due to the lack of standardized treatment protocols.   Recanalization of CNC-EHPVO has emerged as a strategy for managing severe portal hypertension...

Background Transjugular intrahepatic portosystemic shunt (TIPS) is a well-established approach for the treatment of portal hypertension-related complications in patients with cirrhosis [1]. Patients with porto-sinusoidal vascular disorder (PSVD) can experience portal hypertension-related complications similar to patients with cirrhosis. Still, they deeply differ from these patients in terms of age, pathophysiology of the underlying liver disease and comorbidities. They usually present a...

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,...

Porto-Sinusoidal Vascular Disorders (PSVD) is a heterogeneous clinico-pathological entity characterized by alterations of small liver vessels. The most common liver lesions include “nodular regenerative hyperplasia (NRH)” and obliterative portal venopathy (1,2). Patients with PSVD can developed portal hypertension and its complications as well as portal thrombosis in 1/3 of patients at five years (2-5). In patients with PSVD and signs of portal hypertension, 10 years survival decreases to 56...

Background In 2019, VALDIG proposed the term of porto-sinusoidal vascular liver disease (PSVD) to describe a group of rare vascular liver entities characterized by histological lesions involving portal venules and/or sinusoids and absence of cirrhosis, with or without signs of portal hypertension (1). Recently, the Baveno VII consensus workshop preferred the term “disorder” instead of “disease” so that PSVD now stands for “porto-sinusoidal vascular liver disorder”. PSVD is defined as follows:...

Rationale: The first DOACs have been approved for treatment of thromboembolism in children. Based on the risk benefit profiles published so far, we expect DOACs to be widely used in children. The strict inclusion criteria for participation in the RCTs limit their generalizability, particularly to those with serious medical conditions that account for a significant proportion of pediatric VTE patients in clinical practice. Systematic exclusion of children with elevated liver enzymes or liver...

Cross-sectional observational study of a multicenter cohort of patients with common variable immunodeficiency. - Main variable: porto-sinusoidal vascular disease. With or without portal hypertension. Secondary variables: demographic, clinical, analytical, hemodynamic, radiological, and histological data where available, as well as the patient's clinical evolution. - Study phases: 1) Patient identification and data collection: Identification of patients in each center and collection of...

Increased resistance to portal blood flow is the main pathophysiological mechanisms leading to the development of portal hypertension (PH) in patients either with cirrhosis or in patients with non-cirrhotic portal vein thrombosis (NCPVT).  Pathophysiology of PH in cirrhosis has been extensively reviewed and discussed. Briefly, the increase in resistance is mainly located at intrahepatic level and has two main components:  one structural and the other dynamic (due to an increase in intrahepatic...

Sinusoidal obstruction syndrome (SOS) is a rare but dismal complication of high-dose chemotherapy with or without hematopoietic stem cell transplantation (HSCT),  with a survival of 20% for the very severe form of the disease, despite defibrotide and modern intensive care. Data on liver transplantation for SOS in this setting are limited. The only available study is a review article gathering 20 cases, including 5 from an American transplantation database and 15 derived from 15 case reports...

Chronic extrahepatic portal vein obstruction (CEPVO) in the absence of cirrhosis refers to a portal vein thrombosis present or persistent for more than 6 months or to a portal cavernoma. Main complications of CEPVO are related to portal hypertension, and probability of having high-risk varices (i.e. large varices, or small varices with red spot signs, or variceal hemorrhage revealing portal cavernoma) is about 55% at baseline endoscopy (1). Plus, probability of developing high-risk varices...

While the available data is robust regarding the prognostic significance of poor nutritional status and sarcopenia in advanced liver disease, there have been few attempts to characterize the emergence and role of these variables in patients with splanchnic vein thrombosis.

Sarcopenia, a disorder that involves the accelerated loss of skeletal muscle mass and function, is a prevalent complication among patients with cirrhosis and is associated with a poor prognosis. Patients with porto-sinusoidal vascular disorder (PSVD), a condition in which patients develop portal hypertension (PH) in the absence of cirrhosis, may present with muscle wasting and impaired muscle function, leading to sarcopenia. However, the presence of this complication has never been well...

Dear all, We would like to launch a new study entitled “The natural history of early porto-sinusoidal vascular disorder” within the VALDIG network. Currently, there is a gap in knowledge regarding the natural history of patients with histological lesions of PSVD and without signs of portal hypertension. In this study, we will aim to describe the natural history of early stages of PSVD (without signs of portal hypertension) and to clarify risk factors for disease progression. We believe that...

Protocol author: Dr. Sarah Raevens
Email: sarah.raevens@uzgent.be
Address:
Department of Gastro-Enterology and Hepatology, Ghent University Hospital
Corneel Heymanslaan 10
9000 Gent, Belgium

Work packages: WP 1: Data collection, patient inclusion and prespecification of the experiments WP 2: Training and testing of the neural networks with image data WP 3: Training and testing of a multimodal neural network WP 4: Comparing the models WP 5: Reverse engineering of the best performing model

LEFT-SIDED PORTAL HYPERTENSION SECONDARY TO LOCALLY ADVANCED PANCREATIC CANCER: CLINICAL CHARACTERISTICS, THERAPEUTIC APPROACH AND OUTCOME. MULTICENTRIC INTERNATIONAL REGISTER. V 1.0. 29/Jan/2021 Left-sided portal hypertension (LSPH), also known as sinistral or segmental hypertension, is a localized form of portal hypertension (PH) that usually occurs as a result of splenic vein thrombosis (SVT) (1,2). It represents less than 5% of all cases of PH, distinguished from other forms by preserved...

In 2019, VALDIG proposed the term of porto-sinusoidal vascular liver disease (PSVD) to describe a group of rare vascular liver entities characterized by histological lesions involving portal venules and/or sinusoids and absence of cirrhosis, with or without signs of portal hypertension (1). Recently, the Baveno VII consensus workshop preferred the term “disorder” instead of “disease” so that PSVD now stands for “porto-sinusoidal vascular liver disorder”. PSVD is defined as follows: Adequate...

The Coronavirus disease 2019 (Covid-19) pandemic and the coronavirus SARS-CoV-2 have dominated health care systems during the last 1.5 years. Worldwide, almost 200 million people were infected, leading to approximately 4 million deaths. In Europe, almost 33 million people were tested positive for Covid-19, leading to 750.000 deaths. The quick spread around the globe stimulated pharmaceutical companies to develop highly effective vaccines, which are currently widely in use. Safety data did not...

A multi-center, retrospective and prospective registry of neonates, children and adults with congenital porto-systemic shunts Congenital portosystemic shunts (CPSS) are a rare malformation in humans the systemic repercussions of which are potentially severe. They include signs and symptoms such as hepatic encephalopathy, pulmonary vascular diseases, benign or malignant liver tumors and biological and metabolic disorders. The true incidence of CPSS is not known. CPSS can be diagnosed at any age...

Vascular liver diseases (VLD) are a group of rare liver disorders consequence of alterations in the liver venous tree and of unknown etiology. SARS-CoV-2 pandemic has led to a huge number of research studies in patients with liver disease that have demonstrated an increased risk of severe disease and death in patients with chronic liver disease (CLD). However, data on COVID-19 susceptibility, thrombotic risk and outcome in patients with VLD is not totally known. In the last months, the...

The novel coronavirus pandemic leads to rapid dissemination around the world. COVID-19 has been causing death primarily due to pneumonia and respiratory failure. One of the complications which has been described in COVID-19 infection is the increased systemic inflammatory response from the virus which leads to a higher risk of hypercoagulability and associated thromboembolic disease [1, 2]. Splanchnic vein thrombosis occurring outside the setting of cirrhosis is a rare condition which is...

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...

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...

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...

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...

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...

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

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...

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...