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HCM合并PH的发病机制研究进展
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Abstract:
肺动脉高压(Pulmonary hypertension, PH)是一种以肺血管系统为核心的病理综合征，而肥厚型心肌病(Hypertrophic cardiomyopathy, HCM)是最常见的遗传性心肌病之一。HCM患者出现PH后，可能使患者进展为晚期心衰，严重影响HCM患者的生命质量及预后。但近年来HCM相关的PH并未被作为热点进行研究。本文就肺血管阻力、肺血管重构两个方面对HCM患者发生PH的发病机制进行综述。
Pulmonary hypertension (PH) is a pathological syndrome with the pulmonary vasculature system as the core, and hypertrophic cardiomyopathy (HCM) is one of the most common genetic cardiomy-opathies. After the occurrence of PH in HCM patients, patients may progress to advanced heart fail-ure, which seriously affects the quality of life and prognosis of HCM patients. However, HCM related PH has not been studied as a hot spot in recent years. This article reviews the pathogenesis of PH in HCM patients from two aspects: pulmonary vascular resistance and pulmonary vascular remodel-ing.
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