著者

Toshiyuki Nagai Takayuki Inomata Takashi Kohno Takuma Sato Atsushi Tada Toru Kubo Kazufumi Nakamura Noriko Oyama-Manabe Yoshihiko Ikeda Takeo Fujino Yasuhide Asaumi Takahiro Okumura Toshiyuki Yano Kazuko Tajiri Hiroyuki Matsuura Yuichi Baba Haruki Sunami Shingo Tsujinaga Yasutoshi Ota Keiko Ohta-Ogo Yusuke Ishikawa Hideo Matama Nobutaka Nagano Kimi Sato Kazushi Yasuda Yasushi Sakata Koichiro Kuwahara Tohru Minamino Minoru Ono Toshihisa Anzai on behalf of the Japanese Circulation Society Joint Working Group

出版者

The Japanese Circulation Society

雑誌

Circulation Journal (ISSN:13469843)

巻号頁・発行日

pp.CJ-22-0696, (Released:2023-03-10)

被引用文献数

17

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.

著者

Toru Kubo Takayoshi Hirota Yuichi Baba Yuri Ochi Asa Takahashi Naohito Yamasaki Naohisa Hamashige Katsuhito Yamamoto Fumiaki Kondo Kanji Bando Eisuke Yamada Takashi Furuno Toshikazu Yabe Yoshinori L Doi Hiroaki Kitaoka

出版者

The Japanese Circulation Society

雑誌

Circulation Journal (ISSN:13469843)

巻号頁・発行日

pp.CJ-17-0845, (Released:2018-01-13)

参考文献数

30

被引用文献数

21

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Background:There have been few studies on the clinical course of hypertrophic cardiomyopathy (HCM) in a community-based patient cohort in Japan.Methods and Results:In 2004, we established a cardiomyopathy registration network in Kochi Prefecture (the Kochi RYOMA study) that consisted of 9 hospitals, and finally, 293 patients with HCM were followed. The ages at registration and at diagnosis were 63±14 and 56±16 years, respectively, and 197 patients (67%) were male. HCM-related deaths occurred in 23 patients during a mean follow-up period of 6.1±3.2 years. The HCM-related 5-year survival rate was 94%. In addition, a total of 77 cardiovascular events that were clinically severe occurred in 70 patients, and the HCM-related 5-year event-free rate was 80%. Multivariate Cox proportional hazards model analysis showed that the presence of NYHA class III at registration was a significant predictor of HCM-related deaths and that the presence of atrial fibrillation, lower fractional shortening and presence of left ventricular outflow tract obstruction in addition to NYHA class III were significant predictors of cardiovascular events.Conclusions:In our unselected registry in an aged Japanese community, HCM mortality was favorable, but one-fifth of the patients commonly suffered from HCM-related adverse cardiovascular events during the 5-year follow-up period. Careful management of HCM patients is needed, particularly for those with the above-mentioned clinical determinants.

著者

Yoshihisa Matsumura Jun Takata Hiroaki Kitaoka Toru Kubo Yuichi Baba Eri Hoshikawa Tomoyuki Hamada Makoto Okawa Nobuhiko Hitomi Kyoko Sato Naohito Yamasaki Toshikazu Yabe Takashi Furuno Masanori Nishinaga Yoshinori Doi

出版者

日本循環器学会

雑誌

Circulation Journal (ISSN:13469843)

巻号頁・発行日

vol.70, no.4, pp.376-383, 2006 (Released:2006-03-25)

参考文献数

42

被引用文献数

21 42

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Background Because of their favorable prognostic effects, angiotensin converting enzyme inhibitors (ACEI), angiotensin II receptor blockers (ARB) and β blockers have become background therapy in dilated cardiomyopathy (DCM). However, there are few reports concerning the long-term prognosis of Japanese patients with DCM in relation to these treatments. Methods and Results One hundred and fifty patients with DCM were divided into 2 groups: group A (n=46) (diagnosis: 1982-1989) and group B (n=104) (diagnosis: 1990-2002). During follow-up period of 6.9±4.8 years, 62 patients died and 1 patient had a heart transplant. The survival rate at 5 and 10 years was 60.9% and 34.8%, respectively, in group A patients, and 80.9% and 65.3%, respectively, in group B patients (p=0.0079). In group A patients, ACEI/ARB or β blockers were less frequently used (p<0.0001), whereas antiarrhythmics (class Ia or Ib) were more often used (p<0.0001). The patients treated with ACEI/ARB and β blockers showed a better survival rate than those without (p<0.0001). The patients with antiarrhythmics showed a worse survival rate than those without (p<0.0001). Conclusion The prognosis of Japanese patients with DCM has significantly improved over the past 20 years. This improvement may be explained partly through the increased use of ACEI/ARB and β blockers and a declining use of antiarrhythmics. (Circ J 2006; 70: 376 - 383)