Segrera Sergio A, Lawler Laurie, Opotowsky Alexander R, Systrom David, Waxman Aaron B
Center for Pulmonary-Heart Diseases, Pulmonary Vascular Disease Program, Pulmonary Critical Care Medicine, Brigham and Women's Hospital, Boston, MA, USA.
Pulm Circ. 2017 Apr-Jun;7(2):531-538. doi: 10.1177/2045893217709024. Epub 2017 May 12.
A growing body of evidence suggests that exercise pulmonary hypertension (ePH) is an early form of pulmonary arterial hypertension (PAH). Identifying the disease at an early, potentially more responsive phase, and initiating treatment may improve functional status and prevent progression to severe forms of PAH. This was a single-center, open-label six-month treatment trial to evaluate the effect of ambrisentan on pulmonary hemodynamics and exercise capacity in ePH utilizing invasive cardiopulmonary exercise testing (iCPET). After six months of treatment with ambrisentan, patients repeated iCPET; exercise capacity, symptoms, and pulmonary hemodynamics were reassessed. Twenty-two of 30 patients completed the treatment phase and repeat iCPET. After six months of treatment there was a significant decline in peak exercise mPAP (-5.2 ± 5.6 mmHg, P = 0.001), TPG (-7.1 ± 8.0 mmHg, P = 0.001), PVR (-0.9 ± 0.7 Woods units, P = 0.0002), and Ca-vO (-1.8 ± 2.3 mL/dL, P = 0.0002), with significant increases in peak PCWP (+2.9 ± 5.6 mmHg, P = 0.02), PVC (+0.8 ± 1.4 mL/mmHg, P = 0.03), and CO (+2.3 ± 1.4 L/min, P = 0.0001). A trend toward increased VOmax (+4.4 ± 2.6% predicted, P = 0.07) was observed. In addition, there were improvements in 6MWD and WHO FC after 24 weeks. Our findings suggest that treatment of ePH with ambrisentan results in improved pulmonary hemodynamics and functional status over a six-month period. Treatment of ePH may prevent the progression of vascular remodeling and development of established PAH.
越来越多的证据表明，运动性肺动脉高压（ePH）是肺动脉高压（PAH）的早期形式。在疾病早期、可能更易反应的阶段识别该疾病并开始治疗，可能会改善功能状态并预防进展为严重形式的PAH。这是一项单中心、开放标签的为期六个月的治疗试验，旨在利用有创心肺运动试验（iCPET）评估安立生坦对ePH患者肺血流动力学和运动能力的影响。在用安立生坦治疗六个月后，患者重复进行iCPET；重新评估运动能力、症状和肺血流动力学。30名患者中有22名完成了治疗阶段并重复进行了iCPET。治疗六个月后，运动峰值平均肺动脉压（mPAP）显著下降（-5.2±5.6mmHg，P = 0.001），跨肺压差（TPG）下降（-7.1±8.0mmHg，P = 0.001），肺血管阻力（PVR）下降（-0.9±0.7伍兹单位，P = 0.0002），动静脉血氧含量差（Ca-vO）下降（-1.8±2.3mL/dL，P = 0.0002），而肺毛细血管楔压（PCWP）峰值显著升高（+2.9±5.6mmHg，P = 0.02），每搏量变异度（PVC）升高（+0.8±1.4mL/mmHg，P = 0.03），心输出量（CO）升高（+2.3±1.4L/min，P = 0.0001）。观察到最大摄氧量（VOmax）有增加趋势（较预测值增加4.4±2.6%，P = 0.07）。此外，24周后6分钟步行距离（6MWD）和世界卫生组织功能分级（WHO FC）有所改善。我们的研究结果表明，用安立生坦治疗ePH可在六个月内改善肺血流动力学和功能状态。治疗ePH可能会预防血管重塑的进展和已确诊PAH的发展。
