Pulmonary hypertension (PH) is a haemodynamic condition characterised by elevation of mean pulmonary arterial pressure (mPAP) >20 mmHg, assessed by right heart catheterisation. Pulmonary arterial wedge pressure (PAWP) and pulmonary vascular resistance (PVR) distinguish pre-capillary PH (PAWP ≤15 mmHg, PVR >2 Wood Units (WU)), isolated post-capillary PH (PAWP >15 mmHg, PVR ≤2 WU) and combined post- and pre-capillary PH (PAWP >15 mmHg, PVR >2 WU). Exercise PH is a haemodynamic condition describing a normal mPAP at rest with an abnormal increase of mPAP during exercise, defined as a mPAP/cardiac output slope >3 mmHg/L/min between rest and exercise. The core structure of the clinical classification of PH has been retained, including the five major groups. However, some changes are presented herewith, such as the re-introduction of "long-term responders to calcium channel blockers" as a subgroup of idiopathic pulmonary arterial hypertension, the addition of subgroups in group 2 PH and the differentiation of group 3 PH subgroups based on pulmonary diseases instead of functional abnormalities. Mitomycin-C and carfilzomib have been added to the list of drugs with "definite association" with PAH. For diagnosis of PH, we propose a stepwise approach with the main aim of discerning those patients who need to be referred to a PH centre and who should undergo invasive haemodynamic assessment. In case of high probability of severe pulmonary vascular disease, especially if there are signs of right heart failure, a fast-track referral to a PH centre is recommended at any point during the clinical workup.

肺动脉高压 （PH） 是一种血液动力学疾病，其特征是平均肺动脉压 （mPAP） 升高 >20 mmHg，通过右心导管检查进行评估。肺动脉楔压 （PAWP） 和肺血管阻力 （PVR） 区分毛细血管前 PH （PAWP ≤15 mmHg，PVR >2 Wood 单位 （WU））、孤立的毛细血管后 PH （PAWP >15 mmHg，PVR ≤2 WU） 和毛细血管后和毛细血管前 PH 组合（PAWP >15 mmHg，PVR >2 WU）。运动 PH 是一种血流动力学疾病，描述静息时 mPAP 正常，运动时 mPAP 异常增加，定义为静息和运动之间的 mPAP/心输出量斜率 >3 mmHg/L/min。保留了 PH 临床分类的核心结构，包括五大类。然而，本文提出了一些变化，例如重新引入“钙通道阻滞剂的长期反应者”作为特发性肺动脉高压的一个亚组，在第 2 组 PH 中增加亚组，以及根据肺部疾病而不是功能异常区分第 3 组 PH 亚组。丝裂霉素 C 和卡非佐米已被添加到与 PAH “明确关联”的药物清单中。对于 PH 的诊断，我们建议采用阶梯式方法，其主要目的是识别哪些患者需要转诊至 PH 中心，哪些患者应该接受有创血流动力学评估。如果严重肺血管疾病的可能性很高，尤其是有右心衰竭的迹象，建议在临床病情检查期间的任何时候快速转诊至 PH 中心。