Shafazand Shirin, Goldstein Mary K, Doyle Ramona L, Hlatky Mark A, Gould Michael K
The George Washington University, Washington Hospital Center, Washington, DC, USA.
Chest. 2004 Nov;126(5):1452-9. doi: 10.1378/chest.126.5.1452.
Patients with pulmonary arterial hypertension (PAH) often present with dyspnea and severe functional limitations, but their health-related quality of life (HRQOL) has not been studied extensively. This study describes HRQOL in a cohort of patients with PAH.
Cross-sectional study.
A tertiary care, university hospital-based, pulmonary hypertension (PH) clinic.
We studied HRQOL in 53 patients with PAH (mean age, 47 years; median duration of disease, 559 days). Eighty-three percent were women, 53% received epoprostenol, and 72% reported moderate-to-severe functional limitations with a New York Heart Association class 3 or 4 at enrollment.
We examined HRQOL by administering the Nottingham Health Profile, Congestive Heart Failure Questionnaire, and Hospital Anxiety and Depression Scale. We used the Visual Analog Scale and standard gamble (SG) techniques to measure preferences for current health (utilities). Compared with population norms, participants reported moderate-to-severe impairment in multiple domains of HRQOL, including physical mobility, emotional reaction, pain, energy, sleep, and social isolation. Mean SG utilities were 0.71, suggesting that, on average, participants were willing to accept a 29% risk of death in order to be cured of PH.
PAH is a devastating condition that affects predominately young women in the prime of their life. Understanding HRQOL and preferences are important in the care and management of these patients. Compared with population norms, patients with PAH have substantial functional and emotional limitations that adversely affect their HRQOL.
肺动脉高压（PAH）患者常出现呼吸困难和严重的功能受限，但他们与健康相关的生活质量（HRQOL）尚未得到广泛研究。本研究描述了一组PAH患者的HRQOL。
横断面研究。
一家三级医疗、以大学医院为基础的肺动脉高压（PH）诊所。
我们研究了53例PAH患者的HRQOL（平均年龄47岁；疾病中位持续时间559天）。83%为女性，53%接受依前列醇治疗，72%报告在入组时存在中度至重度功能受限，纽约心脏协会心功能分级为3或4级。
我们通过发放诺丁汉健康量表、充血性心力衰竭问卷和医院焦虑抑郁量表来评估HRQOL。我们使用视觉模拟量表和标准博弈（SG）技术来测量对当前健康状况的偏好（效用）。与人群常模相比，参与者报告在HRQOL的多个领域存在中度至重度损害，包括身体活动、情绪反应、疼痛、精力、睡眠和社交隔离。平均SG效用为0.71，这表明，平均而言，参与者愿意接受29%的死亡风险以治愈PH。
PAH是一种致残性疾病，主要影响处于人生黄金时期的年轻女性。了解HRQOL和偏好对于这些患者的护理和管理很重要。与人群常模相比，PAH患者存在严重的功能和情感限制，对其HRQOL产生不利影响。
