Effectiveness and outcome of pulmonary arterial hypertension-specific therapy in japanese patients with pulmonary arterial hypertension

on behalf of the Japan PH Registry (JAPHR) Network

研究成果: Article査読

25 被引用数 (Scopus)

抄録

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-naïve 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-naïve 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.

本文言語English
ページ(範囲)275-282
ページ数8
ジャーナルCirculation Journal
82
1
DOI
出版ステータスPublished - 2018

ASJC Scopus subject areas

  • 循環器および心血管医学

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