Dept of Respiratory Medicine, Hanover Medical School, Hanover, Germany.
Eur Respir J. 2012 Mar;39(3):589-96. doi: 10.1183/09031936.00092311. Epub 2011 Sep 1.
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 (S(v,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, S(v,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.
目前治疗特发性肺动脉高压（IPAH）患者的指南建议根据血流动力学、功能和生化变量来制定治疗决策。这些参数中的大多数在诊断时已被评估为风险预测因子。本研究旨在评估靶向治疗开始后这些参数变化对预后的影响。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 个月进行了血流动力学、功能和生化评估，中位随访 38 个月，以确定基线和疾病过程中死亡的预测因子。在观察期间，53 例（48.6%）患者死亡，4 例（3.7%）患者接受了肺移植。无移植生存的 Kaplan-Meier 估计值分别为 1 年、3 年和 5 年时的 92%、67%和 51%。在基线变量中，6 分钟步行距离、右心房压、心指数、混合静脉血氧饱和度（S(v,O(2)))和 N 末端脑利钠肽前体（NT-proBNP）是生存的独立预测因子。在随访期间，世界卫生组织功能分级、心指数、S(v,O(2))和 NT-proBNP 的变化被证明是预后的显著预测因子。当被分配到预后组时，PAH 靶向治疗开始后这些参数的改善和恶化对生存有很大影响。随访时获得的测量值比基线时获得的变量具有更高的预测价值。在疾病过程中，已确立的预后预测因子的变化为 IPAH 患者提供了重要的预后信息。
