Effectiveness and Outcome of Pulmonary Arterial Hypertension-Specific Therapy in Japanese Patients With Pulmonary Arterial Hypertension
Effectiveness and Outcome of Pulmonary Arterial Hypertension-Specific Therapy in Japanese Patients With Pulmonary Arterial Hypertension
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DOI：
10.1253/circj.cj-17-0139
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发表时间：
2018-01-01
影响因子：
3.3
通讯作者：
Tatsumi, Koichiro
中科院分区：
文献类型：
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作者：
Tamura, Yuichi;Kumamaru, Hiraku;Tatsumi, Koichiro
背景：肺动脉高压(PAH)的初始治疗策略已从单一疗法转变为一线联合疗法。本研究使用日本公共卫生登记处(JAPHR)的数据分析了日本PAH患者的治疗和结果。JAPHR是日本第一个有组织的PAH多中心登记机构。方法和结果：我们研究了2008年4月至2013年3月在8个肺动脉高压(PH)中心登记的189名连续的PAH患者(108名未接受治疗的患者和81名接受背景治疗的患者)。我们进行了回顾性生存分析，并分析了预先合并和血流动力学改善之间的关系，并根据基线的NYHA分级状态进行了调整。189例患者的1、2、3年生存率分别为97.0%(95%CI：92.1~98.4)、92.6%(95%CI：87.0~95.9)和88.2%(95%CI：81.3~92.7)。在未接受治疗的队列中，33%的患者接受了预先联合治疗。1、2、3年生存率分别为97.6%(95%CI：90.6~99.4)、97.6%(95%CI：90.6~99.4)和95.7%(95%CI：86.9~98.6)。首次随访时接受联合治疗的患者出现血流动力学改善的可能性是单一治疗患者的5.27倍(95%CI：2.68-10.36)。结论：根据JAPHR数据，首次联合治疗与血流动力学状态的改善有关。
Background: The trend of the initial treatment strategy for pulmonary arterial hypertension (PAH) has changed from monotherapies to upfront combination therapies. This study analyzed treatments and outcomes in Japanese patients with PAH, using data from the Japan PH Registry (JAPHR), which is the first organized multicenter registry for PAH in Japan.Methods and Results: We studied 189 consecutive patients (108 treatment-naive and 81 background therapy patients) with PAH in 8 pulmonary hypertension (PH) centers enrolled from April 2008 to March 2013. We performed retrospective survival analyses and analyzed the association between upfront combination and hemodynamic improvement, adjusting for baseline NYHA classification status. Among the 189 patients, 1-, 2-, and 3-year survival rates were 97.0% (95% CI: 92.1-98.4), 92.6% (95% CI: 87.0-95.9), and 88.2% (95% CI: 81.3-92.7), respectively. In the treatment-naive cohort, 33% of the patients received upfront combination therapy. In this cohort, 1-, 2-, and 3-year survival rates were 97.6% (95% CI: 90.6-99.4), 97.6% (95% CI: 90.6-99.4), and 95.7% (95% CI: 86.9-98.6), respectively. Patients on upfront combination therapy were 5.27-fold more likely to show hemodynamic improvement at the first follow-up compared with monotherapy (95% CI: 2.68-10.36).Conclusions: According to JAPHR data, initial upfront combination therapy is associated with improvement in hemodynamic status.