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Updated: Dec 8, 2025

Invasive Hemodynamic Characterization of the Portal-hypertensive Syndrome in Cirrhotic Rats
Published on: August 1, 2018
Causes and Management of Non-cirrhotic Portal Hypertension
Stefania Gioia1, Silvia Nardelli2, Lorenzo Ridola2
1Department of Translational and Precision Medicine, Sapienza University of Rome, Rome, Italy. stensgioia@hotmail.com.
Insights
Non-cirrhotic portal hypertension (NCPH) encompasses porto-sinusoidal vascular disease (PSVD) and chronic portal vein thrombosis (PVT). Management of these conditions and their complications mirrors that for cirrhosis, with anticoagulation being crucial.
Area of Science:
- Hepatology
- Gastroenterology
- Vascular Medicine
Background:
- Non-cirrhotic portal hypertension (NCPH) is a complex condition with diverse underlying causes.
- Porto-sinusoidal vascular disease (PSVD) and chronic portal vein thrombosis (PVT) are primary contributors to NCPH in Western populations.
Purpose of the Study:
- To provide a comprehensive overview of the nomenclature, diagnostic features, and management strategies for PSVD and chronic PVT.
- To highlight the importance of recognizing and actively diagnosing these conditions in at-risk patients.
Main Methods:
- Literature review and synthesis of current knowledge on PSVD and chronic PVT.
- Analysis of diagnostic criteria and therapeutic approaches for NCPH.
Main Results:
- Management of NCPH involves treating associated conditions and portal hypertension (PH).
- PH management in PSVD/PVT parallels that in cirrhosis, with Transjugular Intrahepatic Porto systemic Shunt (TIPS) and liver transplantation as options for severe cases.
- Anticoagulation is vital for treating thrombosis in PSVD and preventing recurrence in portal cavernoma.
Conclusions:
- Current management of portal hypertension complications in NCPH aligns with cirrhosis protocols.
- Further large-scale cooperative studies are needed to elucidate the natural history of NCPH and refine its management strategies.
Purpose Of The Review:
Non-cirrhotic portal hypertension (NCPH) includes a heterogeneous group of conditions. The aim of this paper is to make an overview on the denominations, diagnostical features and management of porto-sinusoidal vascular disease (PSVD) and chronic portal vein thrombosis (PVT) being the main causes of NCPH in the Western world.
Recent Findings:
The management of NCPH consists in the treatment of associated diseases and of portal hypertension (PH). PH due to PSVD or PVT is managed similarly to PH due to cirrhosis. TIPS placement and liver transplantation are considerable options in patients with refractory variceal bleeding/ascites and with progressive liver failure. Anticoagulation is a cornerstone both in the treatment of thrombosis in PSVD and in the prevention of thrombosis recurrence in patients with portal cavernoma. Physicians should be aware of the existence of PSVD and chronic PVT and actively search them in particular settings. To now, the management of portal hypertension-related complications in NCPH is the same of those of cirrhosis. Large cooperative studies on the natural history of NCPH are necessary to better define its management.
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