European Respiratory Journal ( IF 16.6 ) Pub Date : 2024-10-31 Maron, B. A., Bortman, G., De Marco, T., Huston, J. H., Lang, I. M., Rosenkranz, S. H., Vachiery, J.-L., Tedford, R. J.
Left heart disease (LHD) is the most common cause of pulmonary hypertension (PH), which may be classified further as isolated post-capillary (ipcPH) or combined post- and pre-capillary PH (cpcPH). The 7th World Symposium on Pulmonary Hypertension PH-LHD task force reviewed newly reported randomised clinical trials and contemplated novel opportunities for improving outcome. Results from major randomised clinical trials reinforced prior recommendations against the use of pulmonary arterial hypertension therapy in PH-LHD outside of clinical trials, and suggested possible harm. Greater focus on phenotyping was viewed as one general strategy by which to ultimately improve clinical outcomes. This is potentially achievable by individualising ipcPH versus cpcPH diagnosis for patients with pulmonary arterial wedge pressure within a diagnostic grey zone (12–18 mmHg), and through a newly developed PH-LHD staging system. In this model, PH accompanies LHD across four stages (A=at risk, B=structural heart disease, C=symptomatic heart disease, D=advanced), with each stage characterised by progression in clinical characteristics, haemodynamics and potential therapeutic strategies. Along these lines, the task force proposed disaggregating PH-LHD to emphasise specific subtypes for which PH prevalence, pathophysiology and treatment are unique. This includes re-interpreting mitral and aortic valve stenosis through a contemporary lens, and focusing on PH within the hypertrophic cardiomyopathy and amyloid cardiomyopathy clinical spectra. Furthermore, appreciating LHD in the profile of PH patients with chronic lung disease and chronic thromboembolic pulmonary disease is essential. However, engaging LHD patients in clinical research more broadly is likely to require novel methodologies such as pragmatic trials and may benefit from next-generation analytics to interpret results.
中文翻译:
与左心疾病相关的肺动脉高压
左心疾病 (LHD) 是肺动脉高压 (PH) 的最常见原因,可进一步分为孤立性毛细血管后 (ipcPH) 或毛细血管后和毛细血管前联合 PH (cpcPH)。第 7 届世界肺动脉高压研讨会 PH-LHD 工作组回顾了新报告的随机临床试验,并考虑了改善结果的新机会。主要随机临床试验的结果强化了先前的建议,即在临床试验之外反对在 PH-LHD 中使用肺动脉高压治疗,并表明可能存在危害。更加关注表型被视为最终改善临床结果的一种通用策略。对于肺动脉楔压在诊断灰色区 (12-18 mmHg) 内的患者,以及通过新开发的 PH-LHD 分期系统,有可能通过个体化 ipcPH 与 cpcPH 诊断来实现这一点。在这个模型中,PH 伴随 LHD 经历四个阶段 (A=有风险,B=结构性心脏病,C=有症状的心脏病,D=晚期),每个阶段的特点是临床特征、血流动力学和潜在治疗策略的进展。沿着这些思路,工作组建议分解 PH-LHD,以强调 PH 患病率、病理生理学和治疗独特的特定亚型。这包括通过现代视角重新解释二尖瓣和主动脉瓣狭窄,并关注肥厚型心肌病和淀粉样变型心肌病临床光谱中的 PH。此外,在患有慢性肺病和慢性血栓栓塞性肺病的 PH 患者概况中了解 LHD 是必不可少的。 然而,更广泛地让 LHD 患者参与临床研究可能需要新的方法,例如实用试验,并且可能会受益于下一代分析来解释结果。