Effectiveness and Outcome of Pulmonary Arterial Hypertension-Specific Therapy in Japanese Patients With Pulmonary Arterial Hypertension

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 fir...

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Published in:	Circulation Journal Vol. 82; no. 1; pp. 275 - 282
Main Authors:	Tamura, Yuichi, Kumamaru, Hiraku, Satoh, Toru, Miyata, Hiroaki, Ogawa, Aiko, Tanabe, Nobuhiro, Hatano, Masaru, Yao, Atsushi, Abe, Kohtaro, Tsujino, Ichizo, Fukuda, Keiichi, Kimura, Hiroshi, Kuwana, Masataka, Matsubara, Hiromi, Tatsumi, Koichiro, on behalf of the Japan PH Registry (JAPHR) Network
Format:	Journal Article
Language:	English
Published:	Japan The Japanese Circulation Society 01-01-2018
Subjects:	Adult Antihypertensive Agents - therapeutic use Drug Therapy, Combination - methods Female Hemodynamics - drug effects Humans Hypertension, Pulmonary - drug therapy Hypertension, Pulmonary - mortality Japan Male Middle Aged Multicenter registry Prognosis Pulmonary arterial hypertension Registries Retrospective Studies Survival Analysis Treatment Outcome Upfront combination therapy Japan Pulmonary arterial hypertension Prognosis Multicenter registry Upfront combination therapy
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Summary:	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.
Bibliography:	ObjectType-Article-1 SourceType-Scholarly Journals-1 ObjectType-Feature-2 content type line 23
ISSN:	1346-9843 1347-4820
DOI:	10.1253/circj.CJ-17-0139