D'Alonzo G E, Barst R J, Ayres S M, Bergofsky E H, Brundage B H, Detre K M, Fishman A P, Goldring R M, Groves B M, Kernis J T
Division of Lung Diseases, National Heart, Lung, and Blood Institute, Bethesda, MD 20892.
Ann Intern Med. 1991 Sep 1;115(5):343-9. doi: 10.7326/0003-4819-115-5-343.
To characterize mortality in persons diagnosed with primary pulmonary hypertension and to investigate factors associated with survival.
Registry with prospective follow-up.
Thirty-two clinical centers in the United States participating in the Patient Registry for the Characterization of Primary Pulmonary Hypertension supported by the National Heart, Lung, and Blood Institute.
Patients (194) diagnosed at clinical centers between 1 July 1981 and 31 December 1985 and followed through 8 August 1988.
At diagnosis, measurements of hemodynamic variables, pulmonary function, and gas exchange variables were taken in addition to information on demographic variables, medical history, and life-style. Patients were followed for survival at 6-month intervals.
The estimated median survival of these patients was 2.8 years (95% Cl, 1.9 to 3.7 years). Estimated single-year survival rates were as follows: at 1 year, 68% (Cl, 61% to 75%); at 3 years, 48% (Cl, 41% to 55%); and at 5 years, 34% (Cl, 24% to 44%). Variables associated with poor survival included a New York Heart Association (NYHA) functional class of III or IV, presence of Raynaud phenomenon, elevated mean right atrial pressure, elevated mean pulmonary artery pressure, decreased cardiac index, and decreased diffusing capacity for carbon monoxide (DLCO). Drug therapy at entry or discharge was not associated with survival duration.
Mortality was most closely associated with right ventricular hemodynamic function and can be characterized by means of an equation using three variables: mean pulmonary artery pressure, mean right atrial pressure, and cardiac index. Such an equation, once validated prospectively, could be used as an adjunct in planning treatment strategies and allocating medical resources.
描述原发性肺动脉高压患者的死亡率，并调查与生存相关的因素。
前瞻性随访登记。
美国32个临床中心参与了由国家心肺血液研究所支持的原发性肺动脉高压特征患者登记。
194例于1981年7月1日至1985年12月31日在临床中心确诊并随访至1988年8月8日的患者。
诊断时，除了收集人口统计学变量、病史和生活方式信息外，还测量了血流动力学变量、肺功能和气体交换变量。患者每6个月随访一次生存情况。
这些患者的估计中位生存期为2.8年（95%可信区间，1.9至3.7年）。估计的单年生存率如下：1年时为68%（可信区间，61%至75%）；3年时为48%（可信区间，41%至55%）；5年时为34%（可信区间，24%至44%）。与生存不良相关的变量包括纽约心脏协会（NYHA）功能分级为III或IV级、存在雷诺现象、平均右心房压力升高、平均肺动脉压力升高、心脏指数降低以及一氧化碳弥散量（DLCO）降低。入院或出院时的药物治疗与生存时间无关。
死亡率与右心室血流动力学功能密切相关，可通过一个使用三个变量的方程来描述：平均肺动脉压力、平均右心房压力和心脏指数。这样一个方程一旦经过前瞻性验证，可作为规划治疗策略和分配医疗资源的辅助工具。
