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 Sakao; Tomoki Kimura; Yasuhiro Kondoh; Kazuhiko Nakayama; Kensuke Tanaka; Hiroshi Ohira; Masaharu Nishimura; Hiroaki Miyata; Koichiro Tatsumi; for the JRPHS Group

doi:10.1253/circj.CJ-20-0939

Vascular Disease

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 Sakao, Tomoki Kimura, Yasuhiro Kondoh, Kazuhiko Nakayama, Kensuke Tanaka, Hiroshi Ohira, Masaharu Nishimura, Hiroaki Miyata, Koichiro Tatsumi, for the JRPHS Group

Author information

Nobuhiro Tanabe
Department of Respirology, Graduate School of Medicine, Chiba University [Japan]
Pulmonary Hypertension Center, Saiseikai Narashino Hospital [Japan]
Hiraku Kumamaru
Department of Healthcare Quality Assessment, Graduate School of Medicine, The University of Tokyo [Japan]
Yuichi Tamura
Pulmonary Hypertension Center, International University of Health and Welfare Mita Hospital [Japan]
Hiroyuki Taniguchi
Department of Respiratory Medicine and Allergy, Tosei General Hospital [Japan]
Noriaki Emoto
Department of Clinical Pharmacy, Kobe Pharmaceutical University [Japan]
Yoshihito Yamada
Department of Chest Medicine, Japan Railway Tokyo General Hospital [Japan]
Osamu Nishiyama
Department of Respiratory Medicine and Allergology, Faculty of Medicine, Kindai University [Japan]
Ichizo Tsujino
First Department of Medicine, Hokkaido University Hospital [Japan]
Hiroshi Kuraishi
Department of Respiratory Medicine, Nagano Red Cross Hospital [Japan]
Yoshihiro Nishimura
Division of Respiratory Medicine, Department of Internal Medicine, Kobe University Graduate School of Medicine [Japan]
Hiroshi Kimura
Department of Advanced Medicine for Pulmonary Circulation and Respiratory Failure and Department of Respiratory Medicine, Nippon Medical School Graduate School of Medicine [Japan]
Department of Respiratory Medicine, Fukujuji Hospital, Japan Anti-Tuberculosis Association (JATA) [Japan]
Second Department of Internal Medicine, Nara Medical University [Japan]
Yoshikazu Inoue
Clinical Research Center, National Hospital Organization Kinki-Chuo Chest Medical Center [Japan]
Yoshiteru Morio
Department of Respiratory Medicine, Juntendo University Graduate School of Medicine [Japan]
Center for Pulmonary Diseases and Respiratory Disease Division, National Hospital Organization Tokyo National Hospital [Japan]
Yasuto Nakatsumi
Department of Respiratory Medicine, Kanazawa Municipal Hospital [Japan]
Toru Satoh
Division of Cardiology Department of Medicine, Kyorin University Hospital [Japan]
Masayuki Hanaoka
First Department of Medicine, Shinshu University School of Medicine [Japan]
Kei Kusaka
Center for Pulmonary Diseases and Respiratory Disease Division, National Hospital Organization Tokyo National Hospital [Japan]
Mitsuhiro Sumitani
Department of Respiratory Medicine, Osaka City General Hospital [Japan]
Tomohiro Handa
Department of Respiratory Medicine, Graduate School of Medicine, Kyoto University [Japan]
Seiicihiro Sakao
Department of Respirology, Graduate School of Medicine, Chiba University [Japan]
Tomoki Kimura
Department of Respiratory Medicine and Allergy, Tosei General Hospital [Japan]
Yasuhiro Kondoh
Department of Respiratory Medicine and Allergy, Tosei General Hospital [Japan]
Kazuhiko Nakayama
Division of Cardiovascular Medicine, Department of Internal Medicine, Kobe University Graduate School of Medicine [Japan]
Kensuke Tanaka
Department of Chest Medicine, Japan Railway Tokyo General Hospital [Japan]
Hiroshi Ohira
First Department of Medicine, Hokkaido University Hospital [Japan]
Masaharu Nishimura
First Department of Medicine, Hokkaido University Hospital [Japan]
Hiroaki Miyata
Department of Health Policy and Management, Keio University School of Medicine [Japan]
Koichiro Tatsumi
Department of Respirology, Graduate School of Medicine, Chiba University [Japan]
for the JRPHS Group

Keywords: Chronic obstructive pulmonary disease (COPD), Interstitial pneumonia, Pulmonary hypertension, Registry

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Supplementary material

2021 Volume 85 Issue 4 Pages 333-342

DOI https://doi.org/10.1253/circj.CJ-20-0939

Editorial (85-4 pp. 343-344)

Browse “Advance online publication” version

Details

Abstract

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-naïve 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.

Corresponding author

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