Diagnosis and Management of Hepatic Vascular Disease

A special issue of Diagnostics (ISSN 2075-4418). This special issue belongs to the section "Pathology and Molecular Diagnostics".

Deadline for manuscript submissions: closed (31 August 2023) | Viewed by 5764

Special Issue Editor

Institute of Liver & Biliary Sciences, New Delhi, India
Interests: oncopathology; vascular liver diseases; bone marrow dysfunction in liver diseases

Special Issue Information

Dear Colleagues, 

Vascular liver diseases are relatively rare liver diseases, which can be broadly classified in two groups—Inflow and outflow vascular diseases. Majority of liver vascular diseases are inflow vascular type, there could be extrahepatic portal vein thrombosis and intrahepatic also considered as non-cirrhotic portal hypertension (NCPH). While the extrahepatic portal vein thrombosis management is based on antithrombotic and vascular interventions, the management of symptomatic NCPH patients is challenging. Moreover there is heterogeneity in histology non-cirrhotic portal hypertension; the etiology remains obscured and hence they are considered as idiopathic non-cirrhotic portal hypertension (INCPH). The challenges in INCPH are that overlapping disease processes fall under the umbrella of INCPH that might have some prognostically useful subcategories. Patients with the same histological features may present without portal hypertension. Sometimes the hepatic pressure measurements are like cirrhosis that raises suspicion of parenchymal collapse. The quantitative assessment of parenchymal collapse remains a challenging problem. The management of INCPH is only symptomatic due to lack of understanding of etiology. The outflow vascular disease known as Budd Chiari Syndrome is relatively easier to diagnose based on acute clinical presentation but subtle asymptomatic chronic cases need expert radio diagnosis and are difficult to manage.

Dr. Chhagan Bihari
Guest Editor

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Keywords

  • extrahepatic portal vein thrombosis
  • portal hypertension
  • idiopathic non-cirrhotic portal hypertension
  • Budd Chiari syndrome
  • antithrombotics

Published Papers (2 papers)

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Review

14 pages, 1478 KiB  
Review
A Narrative Review on Non-Cirrohotic Portal Hypertension: Not All Portal Hypertensions Mean Cirrhosis
by Michele Fiordaliso, Giuseppe Marincola, Barbara Pala, Raffaella Muraro, Mariangela Mazzone, Maria Carmela Di Marcantonio and Gabriella Mincione
Diagnostics 2023, 13(20), 3263; https://doi.org/10.3390/diagnostics13203263 - 20 Oct 2023
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Abstract
Non-cirrhotic portal hypertension (NCPH), also known as idiopathic non-cirrhotic portal hypertension (INCPH) and porto-sinusoidal vascular disorder (PSVD), is a rare disease characterized by intrahepatic portal hypertension (IPH) in the absence of cirrhosis. The precise etiopathogenesis of IPH is an area of ongoing research. [...] Read more.
Non-cirrhotic portal hypertension (NCPH), also known as idiopathic non-cirrhotic portal hypertension (INCPH) and porto-sinusoidal vascular disorder (PSVD), is a rare disease characterized by intrahepatic portal hypertension (IPH) in the absence of cirrhosis. The precise etiopathogenesis of IPH is an area of ongoing research. NCPH diagnosis is challenging, as there are no specific tests available to confirm the disease, and a high-quality liver biopsy, detailed clinical information, and an expert pathologist are necessary for diagnosis. Currently, the treatment of NCPH relies on the prevention of complications related to portal hypertension, following current guidelines of cirrhotic portal hypertension. No treatment has been studied that aimed to modify the natural history of the disease; however, transjugular intrahepatic porto-systemic shunt (TIPS) placement, shunt and liver transplantation are considerable symptomatic options. In this review, we discuss the heterogeneity of NCPH as well as its etiopathogenesis, clinical presentation and management issues. Starting from the assumption that portal hypertension does not always mean cirrhosis, cooperative studies are probably needed to clarify the issues of etiology and the possible genetic background of this rare disease. This knowledge might lead to better treatment and perhaps better prevention. Full article
(This article belongs to the Special Issue Diagnosis and Management of Hepatic Vascular Disease)
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15 pages, 12843 KiB  
Review
Transjugular Intrahepatic Portosystemic Shunt in Chronic Portal Vein Thrombosis—From Routine Recommendations to Demanding Scenarios
by Sasidharan Rajesh, Shobhit Singh and Cyriac Abby Philips
Diagnostics 2022, 12(12), 3100; https://doi.org/10.3390/diagnostics12123100 - 09 Dec 2022
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Abstract
Portal vein thrombosis (PVT), particularly the presence of portal cavernoma, was traditionally considered a relative contraindication for transjugular intrahepatic portosystemic shunting (TIPS) due to the technical difficulties in accessing and maneuvering the portal vein and avoiding the high risk for bleeding periportal collaterals. [...] Read more.
Portal vein thrombosis (PVT), particularly the presence of portal cavernoma, was traditionally considered a relative contraindication for transjugular intrahepatic portosystemic shunting (TIPS) due to the technical difficulties in accessing and maneuvering the portal vein and avoiding the high risk for bleeding periportal collaterals. However, the last decade has seen a surge in the number of studies—mostly case reports and small series of patients—demonstrating that TIPS is not only technically feasible in the vast majority of these patients but also provides effective and long-term control of symptoms associated with portal hypertension in cases refractory to the standard line of therapy. The present article aims to provide a concise but exhaustive overview of the role and the standard and technically difficult TIPS placement scenarios in patients with chronic non-malignant PVT and with and without underlying liver disease. The review is strategically punctuated by exemplary instances from the authors’ experience. Full article
(This article belongs to the Special Issue Diagnosis and Management of Hepatic Vascular Disease)
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