The clinical classification of pulmonary hypertension (PH) has guided diagnosis and treatment of patients with PH for several decades. Discoveries relating to underlying mechanisms, pathobiology and responses to treatments for PH have informed the evolution in this clinical classification to describe the heterogeneity in PH phenotypes. In more recent years, advances in imaging, computational science and multi-omic approaches have yielded new insights into potential phenotypes and sub-phenotypes within the existing clinical classification. Identification of novel phenotypes in pulmonary arterial hypertension (PAH) with unique molecular profiles, for example, could lead to new precision therapies. Recent phenotyping studies have also identified groups of patients with PAH that more closely resemble patients with left heart disease (group 2 PH) and lung disease (group 3 PH), which has important prognostic and therapeutic implications. Within group 2 and group 3 PH, novel phenotypes have emerged that reflect a persistent and severe pulmonary vasculopathy that is associated with worse prognosis but still distinct from PAH. In group 4 PH (chronic thromboembolic pulmonary disease) and sarcoidosis (group 5 PH), the current approach to patient phenotyping integrates clinical, haemodynamic and imaging characteristics to guide treatment but applications of multi-omic approaches to sub-phenotyping in these areas are sparse. The next iterations of the PH clinical classification are likely to reflect several emerging PH phenotypes and improve the next generation of prognostication tools and clinical trial design, and improve treatment selection in clinical practice.

几十年来，肺动脉高压 (PH) 的临床分类一直指导着 PH 患者的诊断和治疗。与 PH 的潜在机制、病理学和治疗反应相关的发现为描述 PH 表型异质性的临床分类的演变提供了信息。近年来，成像、计算科学和多组学方法的进步对现有临床分类中的潜在表型和亚表型产生了新的见解。例如，通过独特的分子谱鉴定肺动脉高压（PAH）的新表型可能会带来新的精准疗法。最近的表型分析研究还发现，多组 PAH 患者与左心疾病（第 2 组 PH）和肺部疾病（第 3 组 PH）患者更相似，这具有重要的预后和治疗意义。在第 2 组和第 3 组 PH 中，出现了新的表型，反映了持续且严重的肺血管病变，这种病变与较差的预后相关，但仍与 PAH 不同。在第 4 组 PH（慢性血栓栓塞性肺病）和结节病（第 5 组 PH）中，当前的患者表型分型方法整合了临床、血流动力学和影像学特征来指导治疗，但多组学方法在这些领域进行亚表型分型的应用很少。 PH 临床分类的下一次迭代可能会反映几种新兴的 PH 表型，并改进下一代预测工具和临床试验设计，并改善临床实践中的治疗选择。