背景與目的：原發性肺動脈高壓影響心臟、肺臟及血管系統功能，本研究探討這些患者之運動能力和生活品質，及其相關性和影響因素。方法：徵召受試者18名（16名女性2名男性），平均年齡38.3±10.5歲，罹病診斷時間48.2±29.0月，接受六分鐘行走測試(6-minllte walktest, 6MWT)、Short-Form 36 item Health Survey（簡稱SF-36）和明尼蘇違心臟衰竭生活品質量表(Minnesota living with heart failure questionnaire, MLHF)之評量。採敘述性統計、皮爾森相關係數及部分相關法分析變項間之相關性。結果：受試者平均6MWT距離為479±78公尺（70.1±10.8%預測值），測試後血氧飽和度92.5±6.2%，呼吸困難指數5.0±2.0。SF-36在「生理角色受限」領域的得分最低為44.4±40.7分；MLHF總分為27.5±18.2。6MWT距離之預測百分比值與測試後呼吸困難指數(r=-0.52, p<0.05)呈現負相關；測試後呼吸困難指數且與罹病時間呈正相關(r=0.67, p<0.05)；調整年齡、性別後，6MWT距離與MLHF總分(r=-66, p<0.05)呈現負相關，與SF-36中的身體生理健康領域呈現正相關(r=0.70, p<0.05)。結論：肺高壓患者的6MWT距離介於324-600公尺，運動後血氧飽和度顯著下降，呼吸困難程度為中度，行走距離與生活品質明顯相關。

Background and Purposes: Idiopathic pulmonary arterial hypertension (IPAH) is associated with cardiopulmonary and vascular dysfunction. Relatively little is known about the exercise capacity and quality of life of these patients in Taiwan. The purpose of this study was to describe their exercise capacity and quality of life and to investigate the interrelationships among 6MWT distance, symptoms of dyspnea, and the QOL. Methods: Eighteen patients with IPAH were recruited (16 female and 2 male; mean age, 38.3 years; mean duration of disease, 48.2 months). All patients underwent 6MWT and fìlled in the Short-Form 36 item Health Survey (SF-36) and the Minnesota living with heart failure questionnaire (MLHF). Data were presented as mean±standard deviation. Pearson's correlation coeffìcients and partial correlation after controlling age and gender were used. Results: Distance walked in 6MWT was 479±78m (70.1±10.8% predicted). Oxygen saturation dropped to 92.5±6.2%, Borg scale increased to 5.0±2.0 after 6MWT. Compared with population norms, patients reported impairment in multiple domains of SF-36 and MLHF. The lowest score of SF-36 was reported in the domain of role physical (44.4±40.7). The total score of MLHF was 27.5±18.2 (total score 0-105). The age-and gender predicted percentage of the distance walked in 6MWT was negatively correlated with the Borg scale post 6MWT (r=-0.52, p<0.05). The Borg scale post 6MWT was positively correlated with the duration of disease (r=0.67, p<0.05). After controlling age and gender, the distance walked in 6MWT was negatively correlated with the total score of MLHF (r=-0.66, p<0.05), but positively correlated with the domains of physical health of SF-36 (r=0.70, p<0.05). Conclusions: The 6-minute walking distance in patients with IPAH ranged from 324m to 600m. After walking test, subjects reported moderate dyspnea and their oxygen saturation dropped signifìcantly. The distance walked in 6MWT was moderately correlated with QOL.