Division of Pulmonology, Department of Internal Medicine, Medical University of Graz, Graz, Austria
Ludwig Boltzmann Institute for Lung Vascular Research Graz, Graz, Austria.
Eur Respir J. 2024 Oct 31;64(4). doi: 10.1183/13993003.01324-2024. Print 2024 Oct.
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)是一种血流动力学状态,其特征为平均肺动脉压(mPAP)升高>20mmHg,通过右心导管术进行评估。肺小动脉楔压(PAWP)和肺血管阻力(PVR)可区分前毛细血管 PH(PAWP≤15mmHg,PVR>2 伍德单位(WU))、孤立性后毛细血管 PH(PAWP>15mmHg,PVR≤2WU)和前、后毛细血管混合 PH(PAWP>15mmHg,PVR>2WU)。运动性 PH 是一种血流动力学状态,描述静息时 mPAP 正常,但运动时 mPAP 异常升高,定义为静息和运动之间 mPAP/心输出量斜率>3mmHg/L/min。PH 的临床分类的核心结构已保留,包括五个主要组。然而,这里也提出了一些变化,例如将“长期钙通道阻滞剂反应者”重新引入特发性肺动脉高压作为一个亚组,在 2 组 PH 中增加亚组,并根据肺部疾病而不是功能异常对 3 组 PH 亚组进行区分。丝裂霉素 C 和卡非佐米已被添加到与 PAH 有“明确关联”的药物列表中。对于 PH 的诊断,我们提出了一种逐步方法,主要目的是区分那些需要转诊到 PH 中心并进行有创血流动力学评估的患者。在存在严重肺血管疾病的高概率情况下,特别是如果存在右心衰竭的迹象,建议在临床评估的任何阶段都快速转诊到 PH 中心。