Chen Ling-Wei, Chen Ssu-Yuan, Hsu Hsao-Hsun, Wu Yen-Wen, Lai Yu-Mei, Chien Meng-Yueh
School and Graduate Institute of Physical Therapy, College of Medicine, National Taiwan University.
Department of Rehabilitation, Central Clinics and Hospital, Taipei.
Acta Cardiol Sin. 2021 Jan;37(1):74-85. doi: 10.6515/ACS.202101_37(1).20200720A.
Pulmonary arterial hypertension (PAH) is a progressive pulmonary vascular disease with a high mortality rate that can be divided into different groups according to etiology and prognosis. Few studies have investigated differences in the exercise capacity and quality of life (QOL) among the different groups of PAH patients. Therefore, we aimed to (1) compare the hemodynamic exercise responses between patients with idiopathic pulmonary arterial hypertension (IPAH) and PAH associated with other diseases (APAH), and (2) determine the factors associated with exercise capacity in patients with PAH.
Six patients diagnosed with IPAH and eight with APAH [congenital heart disease (CHD)-dominant PAH] were included in this study. The main outcome measures included body composition, exercise capacity, hemodynamic measurements, physical activity levels, fatigue severity, and QOL.
The CHD-dominant PAH group had a significantly lower predicted peak oxygen consumption (VO %), pressure of end-tidal carbon dioxide at the peak and at anaerobic threshold (PETCO and PETCO), and significantly elevated ventilatory equivalent (VE/VCO and VE/VCO) compared with the IPAH group. Multiple regression analysis indicated that PETCO was significantly associated with either VO (β = 0.805, adjusted R = 0.619, p = 0.001) or 6-minute walk distance (β = 0.816, adjusted R = 0.638, p < 0.001).
Patients with CHD-dominant PAH had poor exercise capacity and exercise responses compared to those with IPAH. Evaluating exercise capacity and the patient response to exercise using cardiopulmonary exercise testing is increasingly important in view of the etiology of PAH.
肺动脉高压（PAH）是一种进展性肺血管疾病，死亡率高，可根据病因和预后分为不同组。很少有研究调查不同组PAH患者的运动能力和生活质量（QOL）差异。因此，我们旨在：（1）比较特发性肺动脉高压（IPAH）患者与其他疾病相关PAH（APAH）患者的血流动力学运动反应；（2）确定PAH患者运动能力的相关因素。
本研究纳入6例诊断为IPAH的患者和8例APAH（以先天性心脏病（CHD）为主的PAH）患者。主要观察指标包括身体成分、运动能力、血流动力学测量、身体活动水平、疲劳严重程度和生活质量。
与IPAH组相比，以CHD为主的PAH组预测的峰值耗氧量（VO%）、峰值和无氧阈值时的呼气末二氧化碳分压（PETCO和PETCO）显著降低，通气当量（VE/VCO和VE/VCO）显著升高。多元回归分析表明，PETCO与VO（β = 0.805，调整后R = 0.619，p = 0.001）或6分钟步行距离（β = 0.816，调整后R = 0.638，p < 0.001）显著相关。
与IPAH患者相比，以CHD为主的PAH患者运动能力和运动反应较差。鉴于PAH的病因，使用心肺运动试验评估运动能力和患者对运动的反应越来越重要。
