Valdig Projects

Liver and spleen stiffness by 2D-shear-wave elastography to detect porto-sinusoidal vascular liver disorder in patients with signs of portal hypertension

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...

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Splanchnic vein thrombosis and/or Budd-Chiari Syndrome after SARS-COV-2 vaccination

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...

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International Registry of Congenital Porto-Systemic Shunts (IRCPSS)

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...

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COVID-19 Vaccine Efficacy and Safety in patients with Vascular Liver Disease

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...

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Splanchnic vein thrombosis in patients with COVID-19 infection

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...

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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...

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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...

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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...

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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...

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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...

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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...

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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...

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