EXERCISE REHABILITATION IN PULMONARY ARTERIAL HYPERTENSION

这个子项目是许多研究子项目中利用 资源由NIH/NCRR资助的中心拨款提供。子项目和 调查员(PI)可能从NIH的另一个来源获得了主要资金， 并因此可以在其他清晰的条目中表示。列出的机构是 该中心不一定是调查人员的机构。 本研究的目的是确定运动康复计划对肺动脉高压患者的安全性和有效性。 PAH是一种罕见的疾病，会导致通过肺部的血液流动阻塞。血流阻塞会导致肺部高血压，并导致右心衰竭(右心衰竭)、呼吸急促和过早死亡。PAH患者的平均年龄为32岁，主要影响女性。目前对患者的药物治疗将存活率从2年的约50%提高到5年的约67%。因此，虽然有效，但这一温和的临床益处并不能使患者享有较长的预期寿命。此外，即使接受治疗，大多数患者的日常活动仍然非常有限，仍然呼吸急促。在预期寿命也有限的慢性左心衰竭(充血性心力衰竭，或CHF)患者中，运动康复在提高运动能力、力量和耐力方面取得了成功。我们假设，运动训练也将使因PAH而导致的右心衰竭患者受益。我们还假设，运动训练将使这些患者受益，原因是1)通过肺的血液流动增加，导致肺血管衬里的剪切力增加，血管松弛物质(如一氧化氮)的产生增加，血管收缩因子(如内皮素-1)的产生减少；2)通过肺的血流与向肺的空气输送的匹配得到改善(提高了“呼吸效率”)；3)提高了肌肉的工作能力；以及3)提高了影响生活质量的运动能力。

This subproject is one of many research subprojects utilizing the resources provided by a Center grant funded by NIH/NCRR. The subproject and investigator (PI) may have received primary funding from another NIH source, and thus could be represented in other CRISP entries. The institution listed is for the Center, which is not necessarily the institution for the investigator. The purpose of this study is to determine the safety and efficacy of an exercise rehabilitation program in patients with pulmonary arterial hypertension (PAH). PAH is a rare disease that results in blockage of blood flow through the lungs. The blockage of blood flow results in high blood pressure in the lungs, and causes the right side of their heart to fail (right heart failure), shortness of breath and early death. The average age of patients with PAH is 32 years, and it affects mostly women. Current medical therapy for patients improves survival from about 50% at 2 years to about 67% at 5 years. Thus, while effective, this modest clinical benefit does not enable patients to enjoy a long life expectancy. In addition, even with treatment, most patients are still very limited in their daily activities and remain short of breath. In patients with chronic left heart failure (congestive heart failure, or CHF) whose life expectancy is also limited, exercise rehabilitation has been successful at improving exercise capacity, strength, and endurance. We hypothesize that exercise training will also benefit patients with right heart failure due PAH. We also hypothesize that exercise training will benefit these patients due to 1) the effect of increased blood flow through the lungs resulting in increased shear stress in the lining of the blood vessels of the lungs as well as increased production of blood vessel relaxors such as Nitric Oxide and decreased production of blood vessel constrictors such as endothelin-1; 2) improved matching of blood flow through the lungs with air delivery to the lungs (improved "ventilatory efficiency"); 3) improved ability of the muscles to perform work; and 3) improved exercise capacity affecting quality of life.