作者：常开丽，刘演龙，李昇铃，戴海龙

单位：

650051云南省昆明市，昆明医科大学附属延安医院心血管内科 云南省心血管疾病重点实验室 云南省心脏疾病临床医学中心

Units：

Department of Cardiology/Key Laboratory of Cardiovascular Disease of Yunnan Province/Clinical Medicine Center for Cardiovascular Disease of Yunnan Province, Yan'an Hospital Affiliated to Kunming Medical University, Kunming 650051, China

关键词：

肺动脉高压；心脏病；综述

Keywords：

Pulmonary arterial hypertension; Heart diseases; Review

CLC：

R 541.5 R 541

DOI：

10.12114/j.issn.1008-5971.2024.00.018

Funds：

国家自然科学基金资助项目（82060018，81700438）；云南省科技计划项目（202301AY070001-300， 202101AS070043，202102AA310003-7，202105AF150019）

摘要：

　临床上肺高血压（PH）分为五大类，包括肺动脉高压（PAH）、左心疾病所致PH、肺部疾病和/或 低氧所致PH、慢性血栓栓塞性PH和/或其他肺动脉阻塞性病变所致PH、未明原因和/或多因素所致PH，先天性心脏病 相关性肺动脉高压（CHD-PAH）属于PAH。PAH会降低先天性心脏病患者的运动耐量和生活质量，增加其死亡率。 但CHD-PAH的发病机制尚未完全明确，且目前关于CHD-PAH诊疗方案的相关研究较少。本文介绍了CHD-PAH的定 义、分类、分期、流行病学特征、病因学、病理生理变化、诊断、病情评估、治疗方法及预后，并指出未来需要进一 步研究其独特的病理生理学机制，制定专门针对此类疾病的诊疗策略，包括危险分层、手术指征评估和靶向药物选 择，以期规范此类疾病的诊治，使CHD-PAH患者获益最大。

Abstract：

In clinical practice, pulmonary hypertension is classified into five categories, including pulmonary arterial hypertension (PAH) , PH caused by left heart disease, PH caused by lung diseases and/or hypoxia, chronic thromboembolic PH and/or PH caused by other pulmonary artery obstructive lesions, PH caused by unknown causes and/or multiple factors, of which pulmonary arterial hypertension associated with congenital heart disease (CHD-PAH) belongs to PAH. PAH can reduce exercise tolerance and quality of life in patients with congenital heart disease, and increase their mortality rate. However, the pathogenesis of CHD-PAH has not been fully defined, and there are few studies on the diagnosis and treatment of CHD-PAH. This paper introduces the definition, classification, staging, epidemiological characteristics, etiology, pathophysiological changes, diagnosis, disease assessment, treatment method and prognosis of CHD-PAH, and points out that further research is needed on its unique pathophysiological mechanisms in the future, and specialized diagnosis and treatment strategies for this disease should be developed, including risk stratification, surgical indication evaluation, and targeted drug selection, in order to standardize the diagnosis and treatment of this disease and maximize the benefits for CHD-PAH patients.

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