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肺动脉高压的最新进展

Updates on Pulmonary Hypertension.

作者信息

Paudyal Vivek, Thapa Rubi, Basnet Sagarika, Sharma Munish, Surani Salim, Varon Joseph

机构信息

Department of General Practice and Emergency Medicine, Karnali Academy of Health Sciences, Jumla, Nepal.

Department of Internal Medicine, Kathmandu Medical College and Teaching Hospital, Kathmandu, Nepal.

出版信息

Open Respir Med J. 2025 Feb 10;19:e18743064344024. doi: 10.2174/0118743064344024250203101417. eCollection 2025.

Abstract

Pulmonary Arterial Hypertension (PAH) is an uncommon condition with high mortality. It is an underrecognized condition both in developing and developed countries, especially in developing countries, due to a lack of advanced healthcare facilities and resources for timely diagnosis. More than half of the individuals diagnosed with PAH live less than five years after diagnosis. In recent years, tremendous advancements have been made in diagnostic and therapeutic strategies for PAH patients. Phosphodiesterase 5 (PDE5) inhibitors, endothelin receptor antagonists, and prostacyclin inhibitors in various forms (oral, inhaled, intravenous, or subcutaneous) have been the cornerstone of medical treatment. Atrial septostomy, heart and lung transplant, balloon pulmonary angioplasty, and pulmonary thromboendarterectomy are existing therapeutic options currently available. There has been a continuous effort to introduce newer therapies to improve life expectancy and modify disease. Newer therapies have shown promising results but require future data to guarantee long-term safety and efficacy. We aim to discuss a few of these critical updates in the constantly evolving field of PAH.

摘要

肺动脉高压(PAH)是一种死亡率高的罕见疾病。由于缺乏先进的医疗设施和资源进行及时诊断,在发展中国家和发达国家,尤其是发展中国家,这是一种未得到充分认识的疾病。超过一半被诊断为PAH的患者在诊断后存活时间不到五年。近年来,PAH患者的诊断和治疗策略取得了巨大进展。磷酸二酯酶5(PDE5)抑制剂、内皮素受体拮抗剂以及各种形式(口服、吸入、静脉或皮下)的前列环素抑制剂一直是药物治疗的基石。房间隔造口术、心肺移植、球囊肺动脉成形术和肺动脉血栓内膜剥脱术是目前现有的治疗选择。一直在不断努力引入更新的疗法以提高预期寿命并改善疾病状况。更新的疗法已显示出有希望的结果,但需要未来的数据来确保长期安全性和有效性。我们旨在讨论PAH不断发展的领域中的一些关键进展。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3bd0/12046238/3e5759715d28/TORMJ-19-E18743064344024_F1.jpg

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