Division of Cardiology, Department of Medicine, Kyorin University School of Medicine, Tokyo, Japan.
Circ J. 2012;76(6):1494-500. doi: 10.1253/circj.cj-11-1223. Epub 2012 Mar 16.
The recent development of various effective drugs, such as epoprostenol, sildenafil, and bosentan, has improved the prognosis for patients with idiopathic pulmonary arterial hypertension (IPAH). This study sought to determine survival rates and to identify predictive prognostic factors in patients with IPAH in the current era of combination therapy with new and more effective vasodilators.
In 65 consecutive IPAH patients treated from 2004 to 2009, hemodynamic parameters were significantly improved and brain natriuretic peptide was significantly decreased by combination therapy (observation period: 35±18 months). The Kaplan-Meier survival curves were determined, and 22 prognostic variables, including 9 hemodynamic variables and 6 biomarkers, were evaluated to obtain the best variables. The 1-year and 3-year survival rates were 98% and 86%, respectively. Only the platelet level was correlated with death (P<0.05), and the platelet level was significantly correlated with mean pulmonary arterial pressure (P<0.01). Patients with a lower platelet level (<20×10(4)/µl (median value)) before treatment had a higher mortality rate compared to the other patients (78% vs. 95% for 3-year survival, P<0.01).
Combination therapy contributed to an improvement in the prognosis of IPAH patients. Platelet level is a significant prognostic predictor in this new treatment era.
近年来，各种有效药物的发展，如依前列醇、西地那非和波生坦，改善了特发性肺动脉高压（IPAH）患者的预后。本研究旨在确定在当前联合使用新型和更有效的血管扩张剂治疗的时代，IPAH 患者的生存率，并确定预测预后的因素。
在 2004 年至 2009 年连续治疗的 65 例 IPAH 患者中，联合治疗显著改善了血流动力学参数，降低了脑利钠肽（观察期：35±18 个月）。绘制 Kaplan-Meier 生存曲线，并评估 22 个预后变量，包括 9 个血流动力学变量和 6 个生物标志物，以获得最佳变量。1 年和 3 年的生存率分别为 98%和 86%。只有血小板计数与死亡相关（P<0.05），且血小板计数与平均肺动脉压显著相关（P<0.01）。治疗前血小板计数较低（<20×10^4/µl（中位数））的患者死亡率高于其他患者（3 年生存率分别为 78%和 95%，P<0.01）。
联合治疗改善了 IPAH 患者的预后。在这个新的治疗时代，血小板计数是一个重要的预后预测指标。
