医学
心脏病学
内科学
低氧血症
DLCO公司
肺动脉高压
肺功能测试
肺活量
低碳酸血症
肺动脉
血管阻力
血压
心脏指数
扩散能力
心率
肺
呼吸系统
肺功能
高碳酸血症
作者
Stuart Rich,David R. Dantzker,Stephen M. Ayres,Edward H. Bergofsky,Bruce H. Brundage,Katherine M. Detre,Alfred P. Fishman,Roberta M. Goldring,Berton M. Groves,Spencer K. Koerner
标识
DOI:10.7326/0003-4819-107-2-216
摘要
A national registry was begun in 1981 to collect data from 32 centers on patients diagnosed by uniform criteria as having primary pulmonary hypertension. Entered into the registry were 187 patients with a mean age (+/- SD) of 36 +/- 15 years (range, 1 to 81), and a female-to-male ratio of 1.7:1 overall. The mean interval from onset of symptoms to diagnosis was 2 years. The most frequent presenting symptoms included dyspnea (60%), fatigue (19%), and syncope (or near syncope) (13%). Raynaud phenomenon was present in 10% (95% of whom were female) and a positive antinuclear antibody test, in 29% (69% female). Pulmonary function studies showed mild restriction (forced vital capacity [FVC], 82% of predicted) with a reduced diffusing capacity for carbon monoxide (DLCO), and hypoxemia with hypocapnia. The mean (+/- SD) right atrial pressure was 9.7 +/- 6 mm Hg; mean pulmonary artery pressure, 60 +/- 18 mm Hg; cardiac index, 2.3 +/- 0.9 L/min X m2; and pulmonary vascular resistance index, 26 +/- 14 mm Hg/L/min X m2 for the group. Although no deaths or sustained morbid events occurred during the diagnostic evaluation of the patients, the typically long interval from initial symptoms to diagnosis emphasizes the need to develop strategies to make the diagnosis earlier.
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