Liver vascular diseases include portal vein thrombosis (with or without cirrhosis), portal-sinusoidal vascular disease, Budd-Chiari syndrome, hepatic sinusoidal obstruction syndrome, non-obstructive sinusoidal dilatation and peliosis hepatis, visceral artery aneurysms, and hepatic arteriovenous fistulas. Importantly, with the exception of portal vein thrombosis in cirrhosis, the others are rare conditions. A substantial body of evidence has been published on the diagnosis, pharmacological treatment, and interventional therapy of liver vascular disorders since the European Association for the Study of the Liver issued the previous version of the clinical practice guidelines in 2016. These guidelines are based on a comprehensive review of relevant literature, providing recommendations for key clinical challenges and emphasizing implementation of individualized treatment by combining individual risk factors and clinical presentations. Thus, multidisciplinary collaborative management involving hepatologists, hematologists, pathologists, interventional radiologists, and surgeons is crucial in this field. The aim of this guideline is to provide guidance for the management of liver vascular diseases based on the best available evidence.

Behçet's disease (BD) is a chronic, relapsing multisystem inflammatory disorder classified as a variable-vessel vasculitis. Venous thrombosis is common, and Budd-Chiari syndrome (BCS) represents one of the most severe manifestations, whereas coronary artery involvement is rare and associated with poor prognosis. Concomitant presentation of BCS and coronary pseudoaneurysm has been rarely reported. A 23-year-old man with a history of BCS caused by thrombotic occlusion of the retrohepatic inferior vena cava (IVC), previously treated with balloon dilatation and anticoagulation, presented with acute chest pain and dyspnea. Transthoracic echocardiography revealed severe anterior wall motion abnormalities with a left ventricular ejection fraction of 25% and findings suggestive of a left anterior descending artery (LAD) pseudoaneurysm with suspected contained rupture, which were further supported by contrast-enhanced computed tomography. Coronary angiography demonstrated diffuse critical stenosis of the proximal LAD with pseudoaneurysm formation and severely impaired distal flow. Percutaneous coronary intervention with sequential stent implantation, including a covered stent, was performed to seal the pseudoaneurysm and restore coronary perfusion. Subsequent targeted history-taking revealed recurrent oral aphthous ulcers, scrotal ulceration, and lower-limb skin lesions, fulfilling the International Criteria for Behçet's Disease. Immunosuppressive therapy was initiated following revascularization, resulting in significant clinical and functional improvement during follow-up. In young patients presenting with myocardial infarction in the absence of conventional cardiovascular risk factors, particularly those with coexisting Budd-Chiari syndrome (BCS) or unexplained venous thrombosis, Behçet's disease (BD) should be strongly considered. Multimodality cardiovascular imaging is essential not only for establishing the diagnosis but also for guiding interventional strategies and subsequent immunosuppressive therapy in vascular Behçet's disease, and for prognostic assessment.

Vascular liver diseases are recognized risk factors for the development of hepatocellular carcinoma (HCC) in non-cirrhotic livers. While the association is well established in conditions like Budd-Chiari syndrome, which have defined surveillance strategies, data regarding HCC in non-cirrhotic portal fibrosis/idiopathic portal hypertension (NCPF/IPH) remain limited. We report a case of incidentally detected HCC in a 52-year-old woman with NCPF/IPH and preserved liver function. She initially presented with portal hypertension without hepatic decompensation and was diagnosed with NCPF/IPH based on histology and clinical criteria. Sixty-four months after her diagnosis, imaging revealed a large well-defined hepatic mass lesion, which showed arterial-phase hyperenhancement and porto-venous-phase washout with markedly elevated alpha-fetoprotein levels, consistent with HCC. Notably, the tumor developed in the absence of any prior hepatic decompensation and was detected incidentally during evaluation for portal vein thrombosis. This case highlights that HCC can occur in NCPF/IPH despite preserved liver function, the absence of severe portal hypertension, and prior nodules; it may also remain clinically silent. To our knowledge, this represents the first reported case of HCC in NCPF/IPH from India.

The reno-hemiazygos venous connection is a rare developmental variant that arises from the persistence of transient embryonic communications. A 75-year-old male underwent contrast-enhanced abdominal computed tomography (CT) for evaluation of abdominal pain. Imaging revealed a normal left renal vein (LRV) draining into the inferior vena cava (IVC) and an additional posterior connection from the LRV to the hemiazygos vein. No evidence of IVC obstruction or renal abnormality was present. Recognition of a reno-hemiazygos connection is important to prevent misinterpretation as a pathologic retroperitoneal vessel or lymphadenopathy, to appreciate its potential role as a collateral pathway in cases of IVC obstruction or Budd-Chiari syndrome, and to aid in preoperative planning to minimize the risk of vascular injury or bleeding during retroperitoneal or renal surgeries..

Background Coronavirus disease 2019 (COVID-19) has been associated with systemic effects that may influence thrombotic risk. While venous thromboembolism has been widely reported, national mortality trends for splanchnic thrombosis, including portal vein thrombosis, Budd-Chiari syndrome, mesenteric venous thrombosis, and mesenteric ischemia, remain poorly characterized. Methods The CDC WONDER Multiple Cause of Death database was queried for 2018-2023. Annual deaths, population counts, and crude mortality rates per 100,000 persons were extracted and stratified by year and sex. Mortality patterns during the pre-pandemic period (2018-2019) were compared with the pandemic/post-pandemic period (2020-2023) using descriptive trend analysis. Results A total of 32,738 deaths were attributed to splanchnic thrombosis during the study period. Annual deaths increased from 4,819 in 2018 to 5,986 in 2023. The pre-COVID-19 annual average was 4,931 deaths compared with 5,719 deaths during 2020-2023, representing a 16.0% relative increase. Crude mortality rates rose from 1.5 per 100,000 (2018-2019) to 1.7-1.8 per 100,000 (2021-2023). Increases were observed in both sexes. Conclusions Mortality from splanchnic thrombosis increased during the COVID-19 era. These findings demonstrate a temporal rise in mortality; however, the study design is descriptive and does not establish causality. Further research is needed to evaluate potential contributing factors and clinical implications.

Introduction-Aim: Latent myeloproliferative neoplasms (MPNs) have been reported in splanchnic vein thrombosis (SVT). This is the first Tunisian study aimed at evaluating the frequency of JAK2V617F, MPL, and CALR mutations in SVT and analyzing their correlation with hematological parameters Methods: This study, conducted between 2013 and 2017, included non-malignant and non-cirrhotic SVT cases. JAK2V617F and MPL mutations were detected by real-time PCR, and the CALR mutation was screened by PCR and direct sequencing. Results: The JAK2V617F mutation was detected in 20.1% of 233 SVT cases, with a significantly higher prevalence in Budd-Chiari syndrome (BCS). MPL and CALR mutations were not detected. The frequency of latent MPN was 36.2% in SVT, 31.7% in portal vein thrombosis, and 66.6% in BCS. Patients with SVT and hemoglobin levels ≥14.5 g/dL in men, 11.4 g/dL in women, Leukocytes count ≥6100/mm³, and platelet count ≥238,000/mm³ could be tested for the JAK2V617F mutation. This strategy avoids 89.5% of unnecessary tests in patients below these thresholds. A platelet count ≥238,000/mm³ was an independent factor correlated with the JAK2V617F mutation and a strong predictor of latent MPN (OR=17.3; 95% CI [2.8-105.1]; p=0.002). Conclusion: Screening for JAK2V617F is useful for diagnosing latent MPNs revealed by SVT, while MPL and CALR mutations are rare and not recommended.

Obliterative hepatocavopathy, also known as membranous obstruction of the inferior venacava, is a frequently underdiagnosed vascular condition, seen particularly in low socioeconomic populations in India, Nepal and other parts of Asia. This viewpoint presents a distinctive radiographic analogy- the "Acrobatic Dolphin" mimic-observed during venacavography in a patient with membranous inferior venacava obstruction. The dolphin-like configuration, best appreciated in the left anterior oblique view, featured a conical beak-shaped membrane formed by flow dynamics that served both as a diagnostic clue and as an endovascular procedural guide for successful membranotomy. Moreover, recognition of such radiological signs provides powerful mnemonics that enhance clinical education.