著者

Yuichi Tamura Hiraku Kumamaru Shiori Nishimura Yasuo Nakajima Hiromi Matsubara Yu Taniguchi Ichizo Tsujino Ayako Shigeta Koichiro Kinugawa Kazuhiro Kimura Koichiro Tatsumi

出版者

International Heart Journal Association

雑誌

International Heart Journal (ISSN:13492365)

巻号頁・発行日

vol.64, no.4, pp.684-692, 2023-07-29 (Released:2023-07-29)

参考文献数

27

被引用文献数

3

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Upfront combination therapy including intravenous prostaglandin I2 (PGI2-IV) is recognized as the most appropriate treatment for patients with severe pulmonary arterial hypertension (PAH). This retrospective study aimed to determine reasons why this therapy is not used for some patients with severe PAH and describe the hemodynamic and clinical prognoses of patients receiving initial combination treatment with (PGI2-IV+) or without (PGI2-IV−) PGI2-IV.Data for patients with severe PAH (World Health Organization Functional Class III/IV and mean pulmonary arterial pressure [mPAP] ≥ 40 mmHg) were extracted from the Japan Pulmonary Hypertension Registry. Overall, 73 patients were included (PGI2-IV + n = 17; PGI2-IV− n = 56). The PGI2-IV+ cohort was younger than the PGI2-IV− cohort (33.8 ± 10.6 versus 52.6 ± 18.2 years) and had higher mPAP (58.1 ± 12.9 versus 51.8 ± 9.0 mmHg), greater prevalence of idiopathic PAH (88% versus 32%), and less prevalence of connective tissue disease-associated PAH (0% versus 29%). Hemodynamic measures, including mPAP, showed improvement in both cohorts (post-treatment median [interquartile range] 38.5 [17.0-40.0] for the PGI2-IV + cohort and 33.0 [25.0-43.0] mmHg for the PGI2-IV − cohort). Deaths (8/56) and lung transplantation (1/56) occurred only in the PGI2-IV − cohort.These Japanese registry data indicate that older age, lower mPAP, and non-idiopathic PAH may influence clinicians against using upfront combination therapy including PGI2-IV for patients with severe PAH. Early combination therapy including PGI2-IV was associated with improved hemodynamics from baseline, but interpretation is limited by the small sample size.

著者

Masaharu Kataoka Toru Satoh Hiromi Matsubara Koji Yamamoto Tsukasa Inada Kazunari Umezawa Tomohiko Takahashi Atsushi Nakano Keiichi Fukuda

出版者

The Japanese Circulation Society

雑誌

Circulation Reports (ISSN:24340790)

巻号頁・発行日

vol.1, no.6, pp.268-275, 2019-06-10 (Released:2019-06-10)

参考文献数

15

被引用文献数

2

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Background:This retrospective study was conducted to evaluate the safety and efficacy of ambrisentan combination therapy with phosphodiesterase type 5 (PDE5) inhibitors in Japanese patients with pulmonary arterial hypertension (PAH).Methods and Results:PAH patients who received ambrisentan for the first time in combination with a PDE5 inhibitor between January 2013 and the end of August 2015 were included in this study. Adverse drug reaction (ADR) safety analysis, as well as the efficacy analysis focusing on changes in clinical parameters, were investigated for overall cases and cases stratified by patient background. Forty-eight consecutive patients (n=21, 43.8% with idiopathic PAH; male/female, 18/30; average age, 43.3±17.4 years; World Health Organization functional class III/IV, n=22, 45.8%) who were treated with ambrisentan and a PDE5 inhibitor in Japan underwent the safety analysis. A total of 14 ADR occurred in 10 patients (20.8%). ADR included headache (8.3%), face edema (4.2%), angina pectoris (2.1%), hyperemia (2.1%), dyspnea (2.1%), pulmonary hypertension (i.e., worsening of PAH, 2.1%), nausea (2.1%), hepatic function abnormal (2.1%), edema (2.1%), and sudden death (2.1%). On analysis of hemodynamics parameters, there was a significant improvement in the mean pulmonary arterial pressure (−13.5 mmHg, P=0.0001) and pulmonary vascular resistance (−563.53 dyn·s·cm−5, P=0.0033).Conclusions:Ambrisentan combination therapy is safe and effective in hemodynamics improvement.

著者

Yudai Tamura Yuichi Tamura Yu Taniguchi Ichizo Tsujino Takumi Inami Hiromi Matsubara Ayako Shigeta Yoichi Sugiyama Shiro Adachi Kohtaro Abe Yuichi Baba Masaru Hatano Satoshi Ikeda Kenya Kusunose Koichiro Sugimura Soichiro Usui Yasuchika Takeishi Kaoru Dohi Saki Hasegawa-Tamba Koshin Horimoto Noriko Kikuchi Hiraku Kumamaru Koichiro Tatsumi on behalf of the Japan Pulmonary Hypertension Registry Network

出版者

The Japanese Circulation Society

雑誌

Circulation Reports (ISSN:24340790)

巻号頁・発行日

pp.CR-22-0098, (Released:2022-10-08)

参考文献数

21

被引用文献数

3

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Background: Portopulmonary hypertension (PoPH) is one of the major underlying causes of pulmonary arterial hypertension (PAH). However, PoPH, especially treatment strategies, has been poorly studied. Therefore, this study evaluated current treatments for PoPH, their efficacy, and clinical outcomes of patients with PoPH.Methods and Results: Clinical data were collected for patients with PoPH who were enrolled in the Japan Pulmonary Hypertension Registry between 2008 and 2021. Hemodynamic changes, functional class, and clinical outcomes were compared between patients with PoPH treated with monotherapy and those treated with combination therapies. Clinical data were analyzed for 62 patients with PoPH, including 25 treatment-naïve patients, from 21 centers in Japan. In more than half the patients, PAH-specific therapy improved the New York Heart Association functional class by at least one class. The 3- and 5-year survival rates of these patients were 88.5% (95% confidence interval [CI] 76.0–94.7) and 80.2% (95% CI 64.8–89.3), respectively. Forty-one (66.1%) patients received combination therapy. Compared with patients who had received monotherapy, the mean pulmonary arterial pressure, pulmonary vascular resistance, and cardiac index were significantly improved in patients who had undergone combination therapies.Conclusions: Combination therapy was commonly used in patients with PoPH with a favorable prognosis. Combination therapies resulted in significant hemodynamic improvement without an increased risk of side effects.