Pulmonary hypertension

IF 76.9 1区 医学 Q1 MEDICINE, GENERAL & INTERNAL Nature Reviews Disease Primers Pub Date : 2024-01-04 DOI:10.1038/s41572-023-00486-7
Ana Mocumbi, Marc Humbert, Anita Saxena, Zhi-Cheng Jing, Karen Sliwa, Friedrich Thienemann, Stephen L. Archer, Simon Stewart
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Abstract

Pulmonary hypertension encompasses a range of conditions directly or indirectly leading to elevated pressures within the pulmonary arteries. Five main groups of pulmonary hypertension are recognized, all defined by a mean pulmonary artery pressure of >20 mmHg: pulmonary arterial hypertension (rare), pulmonary hypertension associated with left-sided heart disease (very common), pulmonary hypertension associated with lung disease (common), pulmonary hypertension associated with pulmonary artery obstructions, usually related to thromboembolic disease (rare), and pulmonary hypertension with unclear and/or multifactorial mechanisms (rare). At least 1% of the world’s population is affected, with a greater burden more likely in low-income and middle-income countries. Across all its forms, pulmonary hypertension is associated with adverse vascular remodelling with obstruction, stiffening and vasoconstriction of the pulmonary vasculature. Without proactive management this leads to hypertrophy and ultimately failure of the right ventricle, the main cause of death. In older individuals, dyspnoea is the most common symptom. Stepwise investigation precedes definitive diagnosis with right heart catheterization. Medical and surgical treatments are approved for pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension. There are emerging treatments for other forms of pulmonary hypertension; but current therapy primarily targets the underlying cause. There are still major gaps in basic, clinical and translational knowledge; thus, further research, with a focus on vulnerable populations, is needed to better characterize, detect and effectively treat all forms of pulmonary hypertension.

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肺动脉高压
肺动脉高压包括一系列直接或间接导致肺动脉内压力升高的疾病。肺动脉高压(罕见)、与左侧心脏疾病相关的肺动脉高压(非常常见)、与肺部疾病相关的肺动脉高压(常见)、与肺动脉阻塞相关的肺动脉高压(通常与血栓栓塞性疾病有关)(罕见),以及机制不明和/或多因素的肺动脉高压(罕见)。全世界至少有 1%的人口受到肺动脉高压的影响,而在低收入和中等收入国家,肺动脉高压的发病率更高。各种形式的肺动脉高压都与肺血管阻塞、硬化和血管收缩等不良血管重塑有关。如果不积极治疗,就会导致右心室肥大,最终导致右心室衰竭,这是导致死亡的主要原因。在老年人中,呼吸困难是最常见的症状。在通过右心导管检查明确诊断之前,应进行逐步检查。肺动脉高压和慢性血栓栓塞性肺动脉高压的药物和手术治疗已获批准。其他形式的肺动脉高压也有新的治疗方法,但目前的治疗主要针对潜在病因。在基础、临床和转化知识方面仍存在重大差距;因此,需要进一步开展研究,重点关注易感人群,以更好地描述、检测和有效治疗各种形式的肺动脉高压。
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来源期刊
Nature Reviews Disease Primers
Nature Reviews Disease Primers Medicine-General Medicine
CiteScore
76.70
自引率
0.20%
发文量
75
期刊介绍: Nature Reviews Disease Primers, a part of the Nature Reviews journal portfolio, features sections on epidemiology, mechanisms, diagnosis, management, and patient quality of life. The editorial team commissions top researchers — comprising basic scientists and clinical researchers — to write the Primers, which are designed for use by early career researchers, medical students and principal investigators. Each Primer concludes with an Outlook section, highlighting future research directions. Covered medical specialties include Cardiology, Dermatology, Ear, Nose and Throat, Emergency Medicine, Endocrinology, Gastroenterology, Genetic Conditions, Gynaecology and Obstetrics, Hepatology, Haematology, Infectious Diseases, Maxillofacial and Oral Medicine, Nephrology, Neurology, Nutrition, Oncology, Ophthalmology, Orthopaedics, Psychiatry, Respiratory Medicine, Rheumatology, Sleep Medicine, and Urology.
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