Multi-institutional prospective cohort study of patients with pulmonary hypertension associated with respiratory diseases

Nobuhiro Tanabe, Hiraku Kumamaru, Yuichi Tamura, Hiroyuki Taniguchi, Noriaki Emoto, Yoshihito Yamada, Osamu Nishiyama, Ichizo Tsujino, Hiroshi Kuraishi, Yoshihiro Nishimura, Hiroshi Kimura, Yoshikazu Inoue, Yoshiteru Morio, Yasuto Nakatsumi, Toru Satoh, Masayuki Hanaoka, Kei Kusaka, Mitsuhiro Sumitani, Tomohiro Handa, Seiicihiro SakaoTomoki Kimura, Yasuhiro Kondoh, Kazuhiko Nakayama, Kensuke Tanaka, Hiroshi Ohira, Masaharu Nishimura, Hiroaki Miyata, Koichiro Tatsumi

研究成果: Article査読

3 被引用数 (Scopus)

抄録

Background: There is limited evidence for pulmonary arterial hypertension (PAH)-Targeted therapy in patients with pulmonary hypertension associated with respiratory disease (R-PH). Therefore, we conducted a multicenter prospective study of patients with R-PH to examine real-world characteristics of responders by evaluating demographics, treatment backgrounds, and prognosis. Methods and Results: Among the 281 patients with R-PH included in this study, there was a treatment-naive cohort of 183 patients with normal pulmonary arterial wedge pressure and 1 of 4 major diseases (chronic obstructive pulmonary diseases, interstitial pneumonia [IP], IP with connective tissue disease, or combined pulmonary fibrosis with emphysema); 43% of patients had mild ventilatory impairment (MVI), whereas 52% had a severe form of PH. 68% received PAH-Targeted therapies (mainly phosphodiesterase-5 inhibitors). Among patients with MVI, those treated initially (i.e., within 2 months of the first right heart catheterization) had better survival than patients not treated initially (3-year survival 70.6% vs. 34.2%; P=0.01); there was no significant difference in survival in the group with severe ventilatory impairment (49.6% vs. 32.1%; P=0.38). Responders to PAH-Targeted therapy were more prevalent in the group with MVI. Conclusions: This first Japanese registry of R-PH showed that a high proportion of patients with MVI (PAH phenotype) had better survival if they received initial treatment with PAH-Targeted therapies. Responders were predominant in the group with MVI.

本文言語English
ページ(範囲)333-342
ページ数10
ジャーナルCirculation Journal
85
4
DOI
出版ステータスPublished - 2021
外部発表はい

ASJC Scopus subject areas

  • 循環器および心血管医学

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