Department of Cardiology, Cardiovascular Institute and Fu Wai Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China ; Department of Cardiology, The First Affiliated Hospital of Jinan University, Guangzhou, China.
Pulm Circ. 2012 Jul;2(3):373-8. doi: 10.4103/2045-8932.101655.
The survival rates of Chinese patients with idiopathic pulmonary arterial hypertension (IPAH) and familial pulmonary arterial hypertension (PAH) on conventional therapy at 1 and 3 years were 68.0% and 38.9%, respectively. Our aim was to update recent knowledge on the demographics, clinical course, hemodynamic features, disease management, and survival of adult patients with IPAH. This retrospective and observational study was conducted at the largest tertiary referral center in China. Ninety patients with IPAH who underwent initial evaluation at Fu Wai Hospital from January 2006 through November 2009 were retrospectively enrolled. The primary outcome was death. Statistical analyses used included independent sample t test, nonparametric test, Kaplan-Meier method, and Cox proportional hazards analysis. Of the 90 patients enrolled, the median age was 32 years with female predominance. The median interval from onset of symptoms to diagnosis was 14 months. Patients exhibited severe exercise limitation and hemodynamic abnormalities at diagnosis. Only 10.6% had a positive vasoreactivity test, while calcium channel blockers were given to 22.2% of patients. Fifty-nine patients (65.6%) received PAH-targeted therapies during follow-up. Our survival rates of 84.1%, 73.7%, and 70.6% at 1-, 2-, and 3-years compared favorably with predicted survival based on the National Institutes of Health equation which showed 1-, 2-, and 3-years survival rates of 67.7%, 55.9%, and 47%, respectively. For the patients receiving conventional therapy solely, the 1- and 3-years survival rates were 67.0% and 49.3%, respectively. Younger age, lower body mass index, presence of pericardial effusion, and absence of PAH-targeted therapy were independently associated with mortality. We concluded that patients with IPAH were still diagnosed too late, and while survival rates have improved in the modern treatment era, there is still room for improvement.
中国特发性肺动脉高压（IPAH）和家族性肺动脉高压（PAH）患者在接受常规治疗后的 1 年和 3 年生存率分别为 68.0%和 38.9%。我们的目的是更新最近关于成人特发性肺动脉高压患者的人口统计学、临床过程、血液动力学特征、疾病管理和生存率的知识。这项回顾性和观察性研究是在中国最大的三级转诊中心进行的。90 例特发性肺动脉高压患者于 2006 年 1 月至 2009 年 11 月在阜外医院首次接受评估，回顾性纳入本研究。主要结局为死亡。统计分析包括独立样本 t 检验、非参数检验、Kaplan-Meier 方法和 Cox 比例风险分析。90 例患者中，中位年龄为 32 岁，女性占优势。从症状出现到诊断的中位间隔为 14 个月。患者在诊断时表现出严重的运动受限和血液动力学异常。仅有 10.6%的患者血管反应性检测阳性，而钙通道阻滞剂则给予 22.2%的患者。59 例（65.6%）患者在随访期间接受了肺动脉高压靶向治疗。我们的 1 年、2 年和 3 年生存率分别为 84.1%、73.7%和 70.6%，与基于美国国立卫生研究院方程预测的生存率相比，分别为 1 年、2 年和 3 年生存率 67.7%、55.9%和 47%，情况较好。对于仅接受常规治疗的患者，1 年和 3 年生存率分别为 67.0%和 49.3%。年龄较小、体重指数较低、存在心包积液和未接受肺动脉高压靶向治疗与死亡率独立相关。我们得出结论，特发性肺动脉高压患者仍被诊断得太晚，尽管在现代治疗时代生存率有所提高，但仍有改进的空间。
