The prognostic impact of follow-up assessments in patients with idiopathic pulmonary arterial hypertension
The prognostic impact of follow-up assessments in patients with idiopathic pulmonary arterial hypertension
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DOI：
10.1183/09031936.00092311
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发表时间：
2012-03-01
影响因子：
24.3
通讯作者：
Hoeper, M. M.
中科院分区：
文献类型：
--
作者：
Nickel, N.;Golpon, H.;Hoeper, M. M.
目前特发性肺动脉高压（IPAH）患者的治疗指南建议根据血流动力学、功能和生化变量制定治疗决策。这些参数中的大多数在诊断时已被评估为风险预测因子。本研究的目的是评估靶向治疗开始后这些参数变化的预后影响。一组109例IPAH患者在基线和开始肺动脉高压（PAH）靶向治疗后3-12个月接受了血液动力学、功能和生化评估，为了确定基线和疾病过程中死亡率的预测因素，对这些患者进行了中位38个月的随访。在观察期内，死亡53例（48.6%），肺移植4例（3.7%）。Kaplan-Meier估计的1年、3年和5年无移植生存率分别为92%、67%和51%。在基线变量中，6分钟步行距离、右心房压、心脏指数、混合静脉血氧饱和度（Sv，O-2）和N末端脑钠肽前体（NT-proBNP）是生存的独立预测因子。随访期间，世界卫生组织功能分级、心脏指数、Sv、O-2和NT-proBNP的变化证明是结局的重要预测因素。当分配到预后组时，开始PAH靶向治疗后这些参数的改善和恶化对生存率有很大影响。在随访时获得的测量结果比基线时获得的变量具有更高的预测价值，在疾病过程中确定的预后预测因子的变化为IPAH患者提供了重要的预后信息。
Current guidelines for the treatment of patients with idiopathic pulmonary arterial hypertension (IPAH) recommend basing therapeutic decision-making on haemodynamic, functional and biochemical variables. Most of these parameters have been evaluated as risk predictors at the time of diagnosis. The aim of the present study was to assess the prognostic impact of changes in these parameters after initiation of targeted therapy.A cohort of 109 patients with IPAH who had undergone haemodynamic, functional and biochemical assessments at baseline and 3-12 months after initiation of pulmonary arterial hypertension (PAH)-targeted therapy, were followed for a median 38 months in order to determine predictors of mortality at baseline and during the course of their disease.Within the observation period, 53 (48.6%) patients died and four (3.7%) underwent lung transplantation. Kaplan-Meier estimates for transplantation-free survival were 92%, 67%, and 51% at 1, 3, and 5 yrs, respectively. Among baseline variables, 6-min walk distance, right atrial pressure, cardiac index, mixed-venous oxygen saturation (Sv,O-2) and N-terminal-pro brain natriuretic peptide (NT-proBNP) were independent predictors of survival. During follow-up, changes in World Health Organization functional class, cardiac index, Sv,O-2 and NT-proBNP proved significant predictors of outcome. When assigned to prognostic groups, improvements as well as deteriorations in these parameters after initiation of PAH-targeted therapy had a strong impact on survival. Measurements obtained at follow-up had a higher predictive value than variables obtained at baseline.Changes in established predictors of outcome during the course of the disease provide important prognostic information in patients with IPAH.