Ameri Pietro, Mercurio Valentina, Pollesello Piero, Anker Markus S, Backs Johannes, Bayes-Genis Antoni, Borlaug Barry A, Burkhoff Daniel, Caravita Sergio, Chan Stephen Y, de Man Frances, Giannakoulas George, González Aránzazu, Guazzi Marco, Hassoun Paul M, Hemnes Anna R, Maack Cristoph, Madden Brendan, Melenovsky Vojtech, Müller Oliver J, Papp Zoltan, Pullamsetti Soni Savai, Rainer Peter P, Redfield Margaret M, Rich Stuart, Schiattarella Gabriele G, Skaara Hall, Stellos Kostantinos, Tedford Ryan J, Thum Thomas, Vachiery Jean Luc, van der Meer Peter, Van Linthout Sophie, Pruszczyk Piotr, Seferovic Petar, Coats Andrew J S, Metra Marco, Rosano Giuseppe, Rosenkranz Stephan, Tocchetti Carlo Gabriele
Department of Internal Medicine, University of Genova, Genoa, Italy.
Cardiac, Thoracic, and Vascular Department, IRCCS Ospedale Policlinico San Martino, Genoa, Italy.
Eur J Heart Fail. 2024 Apr;26(4):707-729. doi: 10.1002/ejhf.3236. Epub 2024 Apr 19.
Pulmonary hypertension (PH) associated with left heart failure (LHF) (PH-LHF) is one of the most common causes of PH. It directly contributes to symptoms and reduced functional capacity and negatively affects right heart function, ultimately leading to a poor prognosis. There are no specific treatments for PH-LHF, despite the high number of drugs tested so far. This scientific document addresses the main knowledge gaps in PH-LHF with emphasis on pathophysiology and clinical trials. Key identified issues include better understanding of the role of pulmonary venous versus arteriolar remodelling, multidimensional phenotyping to recognize patient subgroups positioned to respond to different therapies, and conduct of rigorous pre-clinical studies combining small and large animal models. Advancements in these areas are expected to better inform the design of clinical trials and extend treatment options beyond those effective in pulmonary arterial hypertension. Enrichment strategies, endpoint assessments, and thorough haemodynamic studies, both at rest and during exercise, are proposed to play primary roles to optimize early-stage development of candidate therapies for PH-LHF.
与左心衰竭(LHF)相关的肺动脉高压(PH-LHF)是PH最常见的病因之一。它直接导致症状并降低功能能力,对右心功能产生负面影响,最终导致预后不良。尽管到目前为止已经测试了大量药物,但PH-LHF仍没有特异性治疗方法。本科学文献阐述了PH-LHF的主要知识空白,重点是病理生理学和临床试验。确定的关键问题包括更好地理解肺静脉与小动脉重塑的作用、多维表型分析以识别适合不同治疗的患者亚组,以及开展结合小型和大型动物模型的严格临床前研究。预计这些领域的进展将为临床试验设计提供更好的信息,并扩展治疗选择,使其超出对肺动脉高压有效的治疗方法。建议采用富集策略、终点评估以及静息和运动期间的全面血流动力学研究,以在优化PH-LHF候选疗法的早期开发中发挥主要作用。