Multi-Institutional Prospective Cohort Study of Patients With Pulmonary Hypertension Associated With Respiratory Diseases
| Autor: | Toru Satoh, Hiroshi Ohira, Yoshiteru Morio, Kei Kusaka, Kazuhiko Nakayama, Yoshikazu Inoue, Tomoki Kimura, Yasuto Nakatsumi, Hiroshi Kuraishi, Seiicihiro Sakao, Yoshihiro Nishimura, Nobuhiro Tanabe, Mitsuhiro Sumitani, Kensuke Tanaka, Yasuhiro Kondoh, Tomohiro Handa, Noriaki Emoto, Yuichi Tamura, Koichiro Tatsumi, Masayuki Hanaoka, Ichizo Tsujino, Hiroyuki Taniguchi, Hiroaki Miyata, Masaharu Nishimura, Yoshihito Yamada, Hiroshi Kimura, Hiraku Kumamaru, Osamu Nishiyama |
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| Rok vydání: | 2021 |
| Předmět: |
medicine.medical_specialty
Hypertension Pulmonary 030204 cardiovascular system & hematology 03 medical and health sciences 0302 clinical medicine Japan Internal medicine Normal Pulmonary Arterial Wedge Pressure Pulmonary fibrosis medicine Humans Familial Primary Pulmonary Hypertension 030212 general & internal medicine Prospective Studies Respiratory system Prospective cohort study business.industry Respiratory disease General Medicine medicine.disease Respiration Disorders Pulmonary hypertension Connective tissue disease Cohort Cardiology and Cardiovascular Medicine business Lung Diseases Interstitial |
| Zdroj: | Circulation journal : official journal of the Japanese Circulation Society. 85(4) |
| ISSN: | 1347-4820 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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