门静脉高压往往被认为是肝硬化的标志性伴随表现，以门静脉系统压力升高和侧支循环建立为特征。然而，非肝硬化性门静脉高压 （non-cirrhotic portal hypertension, NCPH）在临床中并不罕见。根据发病机制不同，NCPH可分为肝前性、肝性和肝后性。NCPH的临床特点和管理策略均有别于肝硬化门静脉高压。近年来，一系列研究发现，代谢相关脂肪性肝病可引起肝窦内皮功能障碍和肝内血管改变，在发生肝硬化之前即可导致门静脉压力显著升高，已成为NCPH的重要病因之一。本文总结了代谢相关NCPH的发病机制、临床特点、诊断与鉴别诊断、治疗与管理策略及未来研究方向等，旨在提高临床医师对该类疾病的认知水平和精准管理能力。

Portal hypertension has often been regarded as a hallmark manifestation of cirrhosis, characterized by elevated pressure in the portal venous system and the establishment of collateral circulation. However, non-cirrhotic portal hypertension (NCPH) is not rare in clinical practice. Based on the different underlying pathogenetic mechanisms, NCPH can be classified into pre-hepatic, hepatic, and post-hepatic types, whose clinical features and management strategies differ significantly from those of cirrhosis-related portal hypertension. In recent years, a series of studies have demonstrated that metabolic associated fatty liver disease can induce liver sinusoidal endothelial dysfunction and intrahepatic vascular alterations, leading to a significant increase in portal pressure before the development of cirrhosis, thereby becoming an important cause of NCPH. This article summarizes the pathogenesis, clinical characteristics, diagnosis and differential diagnosis, therapeutic and management strategies, as well as future research directions, etc., aiming to enhance clinical physicians’ awareness of this disease and precision management capabilities for this disease.