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Updated: Jul 20, 2025

Invasive Hemodynamic Characterization of the Portal-hypertensive Syndrome in Cirrhotic Rats
Published on: August 1, 2018
Evolving Understanding of Noncirrhotic Portal Hypertension
1Department of Pathology, Memorial Sloan Kettering Cancer Center, 1275 York Avenue, New York, NY 10065, USA.
Insights
Noncirrhotic portal hypertension, including porto-sinusoidal vascular disease (PSVD), has various causes. Understanding its pathological features is crucial for diagnosis, especially when the etiology is unknown.
Area of Science:
- Hepatology
- Vascular Medicine
- Pathology
Background:
- Cirrhosis is a common cause of portal hypertension.
- Noncirrhotic portal hypertension arises from circulatory issues in prehepatic, intrahepatic, or posthepatic circulation.
- Intrahepatic causes are further classified as presinusoidal, sinusoidal, or postsinusoidal.
Purpose of the Study:
- To discuss the diagnostic pathological features of noncirrhotic portal hypertension.
- To emphasize the characteristics of porto-sinusoidal vascular disease (PSVD).
- To address the poorly understood etiology of idiopathic noncirrhotic portal hypertension, now classified as PSVD.
Main Methods:
- Review of diagnostic pathological features.
- Focus on histological findings in noncirrhotic portal hypertension.
- Analysis of cases with unknown etiology (idiopathic PSVD).
Main Results:
- Noncirrhotic portal hypertension encompasses diverse etiologies.
- Porto-sinusoidal vascular disease (PSVD) is a key diagnostic consideration.
- Pathological examination is essential for differentiating causes.
Conclusions:
- Accurate pathological assessment is vital for diagnosing noncirrhotic portal hypertension.
- Further research is needed to elucidate the causes of idiopathic PSVD.
- Understanding PSVD's features aids in managing portal hypertension without cirrhosis.
Abstract:
Although cirrhosis is one of the most common causes of portal hypertension, noncirrhotic portal hypertension can result from hemodynamic perturbations occurring in the prehepatic, intrahepatic, and posthepatic circulation. Intrahepatic portal hypertension can be further subclassified relative to the hepatic sinusoids as presinusoidal, sinusoidal, and postsinusoidal. For many of these differential diagnoses, the etiology is known but the cause of idiopathic noncirrhotic portal hypertension, recently included in porto-sinusoidal vascular disease (PSVD), remains poorly understood. Herein, we discuss the diagnostic pathological features of noncirrhotic portal hypertension, with an emphasis on PSVD.
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