Department of Medicine, University of Maryland School of Medicine, Baltimore, MD, USA.
The University of Maryland - Institute for Health Computing, Bethesda, MD, USA.
Eur Respir J. 2024 Oct 31;64(4). doi: 10.1183/13993003.01344-2024. Print 2024 Oct.
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 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.
左心疾病（LHD）是肺动脉高压（PH）最常见的原因，其可进一步分为孤立性毛细血管后（ipcPH）或合并毛细血管前和后 PH（cpcPH）。第 7 届世界肺动脉高压与左心疾病 PH-LHD 专题研讨会工作组回顾了新报告的随机临床试验，并思考了改善预后的新机会。来自主要随机临床试验的结果强化了先前关于在临床试验之外将肺动脉高压治疗应用于 PH-LHD 的建议，且提示可能存在危害。更注重表型被视为最终改善临床结局的一般策略之一。这可以通过对肺动脉楔压处于诊断灰色区域（12-18mmHg）的患者进行个体化毛细血管后-毛细血管前 PH 诊断来实现，也可以通过新开发的 PH-LHD 分期系统来实现。在该模型中，PH 伴随 LHD 经历四个阶段（A=有风险，B=结构性心脏病，C=症状性心脏病，D=晚期），每个阶段的特征是临床特征、血液动力学和潜在治疗策略的进展。按照这种思路，工作组提出将 PH-LHD 细分，以强调 PH 患病率、病理生理学和治疗方法独特的特定亚型。这包括通过当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性心肌病临床谱内的 PH。此外，了解慢性肺部疾病和慢性血栓栓塞性肺病患者 PH 患者的 LHD 情况至关重要。然而，更广泛地让 LHD 患者参与临床研究可能需要新的方法，如实用临床试验，并且可能受益于下一代分析技术来解释结果。
