From the Guidelines
Non-cirrhotic portal hypertension is primarily caused by pre-hepatic, intra-hepatic, and post-hepatic obstructions, resulting from various insults such as infections, drugs or toxins, immune disorders, or thrombophilic states, as stated in the 2014 practice guideline by the American Association for the Study of Liver Diseases 1. The main causes of non-cirrhotic portal hypertension include:
- Pre-hepatic causes: portal vein thrombosis, which may result from hypercoagulable states, abdominal infections, or trauma
- Intra-hepatic causes: nodular regenerative hyperplasia, partial nodular transformation, and idiopathic portal hypertension (also called non-cirrhotic portal fibrosis)
- Post-hepatic causes: Budd-Chiari syndrome (hepatic vein thrombosis) and right-sided heart failure, which impede blood flow from the liver These conditions lead to increased resistance to portal blood flow, resulting in portal hypertension manifesting as varices, ascites, and splenomegaly, despite preserved liver function. According to the EASL clinical practice guidelines: vascular diseases of the liver, published in 2016, the diagnosis of non-cirrhotic portal hypertension requires the exclusion of cirrhosis and other causes of non-cirrhotic portal hypertension 1. Management of non-cirrhotic portal hypertension should focus on treating the underlying cause and preventing complications like variceal bleeding, as recommended by the EASL clinical practice guidelines 1.
From the Research
Causes of Non-Cirrhotic Portal Hypertension
- Non-cirrhotic portal hypertension (NCPH) is mainly related to vascular disorders in the portal system, granuloma formation with periportal fibrosis or genetic alterations affecting the hepatobiliary system 2
- The causes of NCPH can be classified into intrahepatic or extrahepatic etiologies, with vascular lesions being the most common cause 3
- Intrahepatic causes include porto-sinusoidal vascular disease (PSVD) and chronic portal vein thrombosis (PVT) 4
- Extrahepatic causes include extrahepatic portal vein obstruction (EHPVO) and schistosomiasis 2, 5
- Other causes of NCPH include non-cirrhotic portal fibrosis (NCPF), also known as idiopathic portal hypertension (IPH) or 'hepatoportal sclerosis' 5
- Antiphospholipid syndrome (APS) can also cause non-cirrhotic portal vein thrombosis (PVT) 6
Vascular Disorders
- Porto-sinusoidal vascular disease (PSVD) is a main cause of NCPH in the Western world 4
- Chronic portal vein thrombosis (PVT) is another common cause of NCPH 4
- Extrahepatic portal vein obstruction (EHPVO) is a common cause of NCPH in developing countries 5
- Non-cirrhotic portal fibrosis (NCPF) is characterized by 'obliterative portovenopathy' leading to portal hypertension (PHT) 5