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

Research output: Contribution to journalArticle

17 Citations (Scopus)

Abstract

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.

Original languageEnglish
Pages (from-to)275-282
Number of pages8
JournalCirculation Journal
Volume82
Issue number1
DOIs
Publication statusPublished - 2018 Jan 1

Keywords

  • Multicenter registry
  • Prognosis
  • Pulmonary arterial hypertension
  • Upfront combination therapy

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine

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