Scagliola Riccardo
Cardiology Division, Cardinal G. Massaia Hospital, Asti, Italy.
Pulmonary Hypertension Outpatient Clinic, University of Genoa, Genoa, Italy.
Cardiol Res. 2025 Jun 16;16(4):306-311. doi: 10.14740/cr2067. eCollection 2025 Aug.
Although pulmonary arterial hypertension (PAH) usually affects young people with a low burden of cardiovascular comorbidities, epidemiologic changes over time have been providing a codified phenotype of subjects with PAH, characterized by a hemodynamic profile compatible with pure pre-capillary pulmonary hypertension (PH), associated with increased risk factors for left heart disease (LHD). Compared with the younger subjects belonging to the classical PAH phenotype, those with PAH and LHD phenotype share several distinctive features. They include: 1) the older mean age at diagnosis of PAH; 2) peculiar hemodynamic features, characterized by a trend toward lower values of mean pulmonary arterial pressure and pulmonary vascular resistances, and higher values of pulmonary artery wedge pressure; 3) greater clinical deterioration; 4) more impaired exercise capacity; 5) higher mortality risk; 6) weaker response to PAH-targeted treatment; and 7) higher rate of PAH drug discontinuation. Physicians must be aware of such peculiar phenotype of PAH. This is advisable for providing a comprehensive diagnostic workup, in order to reduce the risk of PH misclassification and provide the most appropriate decision-making approach.
尽管肺动脉高压（PAH）通常影响心血管合并症负担较低的年轻人，但随着时间的推移，流行病学变化一直在呈现PAH患者的一种已编篡的表型，其特征是血流动力学特征与单纯毛细血管前肺动脉高压（PH）相符，并伴有左心疾病（LHD）的危险因素增加。与属于经典PAH表型的年轻患者相比，患有PAH和LHD表型的患者具有几个独特特征。它们包括：1）PAH诊断时的平均年龄较大；2）特殊的血流动力学特征，表现为平均肺动脉压和肺血管阻力值有降低趋势，而肺动脉楔压值较高；3）临床恶化更严重；4）运动能力受损更明显；5）死亡风险更高；6）对PAH靶向治疗的反应较弱；7）PAH药物停药率更高。医生必须了解PAH的这种特殊表型。为了提供全面的诊断检查，以降低PH误诊风险并提供最合适的决策方法，这是明智的做法。
