Registry and survival study in chinese patients with idiopathic and familial pulmonary arterial hypertension
Registry and survival study in chinese patients with idiopathic and familial pulmonary arterial hypertension
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DOI：
10.1378/chest.06-2913
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发表时间：
2007-08-01
期刊：
影响因子：
9.6
通讯作者：
Yang, Yue-Jin
中科院分区：
文献类型：
--
作者：
Jing, Zhi-Cheng;Xu, Xi-Qi;Yang, Yue-Jin
背景：评价中国特发性肺动脉高压（PAH）和家族性PAH患者的临床特征和生存资料。方法：选取1999 ~ 2004年特发性PAH和家族性PAH患者72例，按世界卫生组织（WHO）功能分级（I/II和III/IV）分为两组。记录临床和血流动力学数据。结果：72例患者平均年龄35.9岁，女/男比2.4:1。在基线时，两个WHO功能分类组的临床表现存在显著差异。超声心动图显示平均肺收缩压为98 mm Hg。WHO功能分级为III/IV级的患者左室舒张末期直径明显小于I/II级患者。经过平均（+/- SD） 40.1 +/- 20.0个月的随访，1年、2年、3年和5年的生存率分别为68.0%、56.9%、38.9%和20.8%。I/II类组与III/IV类组生存率差异有统计学意义（p = 0.02 [log rank检验]）。结论：本队列研究的基线特征和生存率与20世纪80年代美国国立卫生研究院登记处的基线特征和生存率接近，该登记处患者的1年生存率明显低于2002年至2003年法国登记处的患者。缺乏有效的治疗是本研究中生存率低的主要原因。我们的结果支持
Background: To evaluate the clinical features and survival data of patients with idiopathic pulmonary arterial hypertension (PAH) and familial PAH in Chinese patients.Methods: Seventy-two patients with idiopathic PAH and familial PAH were enrolled in the study from 1999 to 2004 and were classified into two groups according to World Health Organization (WHO) functional class (I/II and III/IV). Clinical and hemodynamic data were recorded. Results: The mean age of the 72 patients was 35.9 years with female patient/male patient ratio of 2.4:1. A significant difference was identified in the clinical presentation between two WHO functional class groups at baseline. Echocardiography showed a mean pulmonary systolic pressure of 98 mm Hg. Left ventricular end-diastolic diameter was significantly smaller in the group of patients in WHO functional class III/IV than in those in class I/II group. After follow-up for a mean (+/- SD) duration of 40.1 +/- 20.0 months, the survival rates at 1, 2, 3, and 5 years were 68.0%, 56.9%, 38.9%, and 20.8%, respectively. A significant difference was identified in survival rate between the class I/II and class III/IV groups (p = 0.02 [log rank test]).Conclusions: The baseline characteristics and survival rates of our cohort study are close to those of the National Institutes of Health Registry in the 1980s, and the I-year survival rate is obviously lower for patients in this registry than for those in the French registry between 2002 to 2003. Lack of effective treatment was the main cause of poor survival in this study. Our results support