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

11 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

Fingerprint

Pulmonary Hypertension
Registries
Japan
Hemodynamics
Therapeutics
Survival Rate
Survival Analysis

Keywords

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

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine

Cite this

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.

In: Circulation Journal, Vol. 82, No. 1, 01.01.2018, p. 275-282.

Research output: Contribution to journalArticle

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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-na{\"i}ve 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-na{\"i}ve 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.",
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author = "{on behalf of the Japan PH Registry (JAPHR) Network} and Yuichi Tamura and Hiraku Kumamaru and Toru Satoh and Hiroaki Miyata and Aiko Ogawa and Nobuhiro Tanabe and Masaru Hatano and Atsushi Yao and Kohtaro Abe and Ichizo Tsujino and Keiichi Fukuda and Hiroshi Kimura and Masataka Kuwana and Hiromi Matsubara and Koichiro Tatsumi",
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T1 - Effectiveness and outcome of pulmonary arterial hypertension-specific therapy in japanese patients with pulmonary arterial hypertension

AU - on behalf of the Japan PH Registry (JAPHR) Network

AU - Tamura, Yuichi

AU - Kumamaru, Hiraku

AU - Satoh, Toru

AU - Miyata, Hiroaki

AU - Ogawa, Aiko

AU - Tanabe, Nobuhiro

AU - Hatano, Masaru

AU - Yao, Atsushi

AU - Abe, Kohtaro

AU - Tsujino, Ichizo

AU - Fukuda, Keiichi

AU - Kimura, Hiroshi

AU - Kuwana, Masataka

AU - Matsubara, Hiromi

AU - Tatsumi, Koichiro

PY - 2018/1/1

Y1 - 2018/1/1

N2 - 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.

AB - 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.

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KW - Prognosis

KW - Pulmonary arterial hypertension

KW - Upfront combination therapy

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