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肺动脉高压:让我们总结一下!

Pulmonary Hypertension: Let's Take Stock!

作者信息

Cacia Michele, Imbalzano Egidio, Ciconte Vincenzo Antonio, Vatrano Marco

机构信息

Cardiology Unit, Azienda Ospedaliero Universitaria "Renato Dulbecco", P.O. "Pugliese", 88100 Catanzaro, Italy.

Department of Clinical and Experimental Medicine, University of Messina, 98125 Messina, Italy.

出版信息

Life (Basel). 2025 Jul 18;15(7):1137. doi: 10.3390/life15071137.

Abstract

Pulmonary hypertension (PH) encompasses a group of conditions characterized by elevated pulmonary arterial pressure, with pulmonary arterial hypertension (PAH) representing a distinct and severe subset. This review provides a comprehensive overview of the current classification system, highlighting the five clinical groups of PH and the specific hemodynamic criteria defining PAH. We discuss the complex pathophysiological mechanisms underlying PAH, including vascular remodeling, endothelial dysfunction, and genetic predisposition. Advances in diagnostic approaches are explored. Current treatment strategies targeting key molecular pathways such as endothelin, nitric oxide, and prostacyclin are reviewed alongside novel and investigational therapies. Prognostic indicators and risk stratification tools are evaluated to guide clinical management. Finally, we underscore the critical role of expert centers in accurate diagnosis, multidisciplinary care, and enrollment in clinical trials, which collectively improve patient outcomes in this challenging disease spectrum.

摘要

肺动脉高压(PH)包括一组以肺动脉压力升高为特征的病症,其中肺动脉高压(PAH)是一个独特且严重的亚组。本综述全面概述了当前的分类系统,重点介绍了PH的五个临床组以及定义PAH的特定血流动力学标准。我们讨论了PAH潜在的复杂病理生理机制,包括血管重塑、内皮功能障碍和遗传易感性。探讨了诊断方法的进展。回顾了当前针对内皮素、一氧化氮和前列环素等关键分子途径的治疗策略以及新型和研究性疗法。评估了预后指标和风险分层工具以指导临床管理。最后,我们强调了专家中心在准确诊断、多学科护理和临床试验入组方面的关键作用,这些共同改善了这一具有挑战性的疾病谱中患者的预后。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3889/12298260/8022a5c12b17/life-15-01137-g001.jpg

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