Chun K J, Kim S H, An B J, Kim S H, Ha J K, Hong T J, Shin Y W
Department of Internal Medicine, College of Medicine, Pusan National University, Pusan, Korea.
Korean J Intern Med. 2001 Jun;16(2):75-9. doi: 10.3904/kjim.2001.16.2.75.
Primary pulmonary hypertension (PPH) that affects predominantly young and productive people is a progressive fatal disease of unknown cause. The objectives of this study were to characterize mortality in patients with PPH and to investigate the factors associated with their survival.
Thirteen patients with PPH were enrolled between 1988 and 1996 and followed-up through July 1999. Measurements at diagnosis included hemodynamic and pulmonary function variables in addition to information on demographic data and medical history.
Patients with PPH have a poor survival expectancy. In this limited study with a small number of patients, mortality is largely associated with decreased cardiac index.
原发性肺动脉高压(PPH)主要影响年轻且有劳动能力的人群,是一种病因不明的进行性致命疾病。本研究的目的是描述PPH患者的死亡率,并调查与他们生存相关的因素。
1988年至1996年间纳入了13例PPH患者,并随访至1999年7月。诊断时的测量包括血流动力学和肺功能变量,以及人口统计学数据和病史信息。
1)纳入本研究的PPH患者的平均年龄为36.1±9.3岁,女性占多数。2)估计中位生存期为3.4±0.6年。3)心脏指数降低是死亡率的唯一显著预测因素(Cox比例风险模型)。
PPH患者的生存预期较差。在这项患者数量有限的小型研究中,死亡率主要与心脏指数降低有关。