著者

Koichiro Sugimura

出版者

The Japanese Circulation Society

雑誌

Circulation Journal (ISSN:13469843)

巻号頁・発行日

pp.CJ-23-0582, (Released:2023-09-06)

参考文献数

12

著者

Shunsuke Tatebe Satoshi Yasuda Ryo Konno Yasuhiko Sakata Koichiro Sugimura Kimio Satoh Takashi Shiroto Satoshi Miyata Osamu Adachi Masato Kimura Yoshiko Mizuno Junko Enomoto Shigeru Tateno Hiromichi Nakajima Kotaro Oyama Yoshikatsu Saiki Hiroaki Shimokawa

出版者

The Japanese Circulation Society

雑誌

Circulation Journal (ISSN:13469843)

巻号頁・発行日

pp.CJ-23-0383, (Released:2023-09-05)

参考文献数

37

被引用文献数

2

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Background: Little is known about clinical or sociodemographic factors that influence health-related quality of life (HRQoL) in patients with adult congenital heart disease (ACHD).Methods and Results: We conducted a nationwide prospective cross-sectional multicenter study at 4 large ACHD centers in Japan. From November 2016 to June 2018, we enrolled 1,223 ACHD patients; 1,025 patients had an HRQoL score. Patients completed a questionnaire survey, including sociodemographic characteristics, and the 36-Item Short-Form Health Survey (SF-36). To determine factors associated with HRQoL, correlations between 2 SF-36 summary scores (i.e., physical component score [PCS] and mental component score [MCS]) and other clinical or sociodemographic variables were examined using linear regression analysis. In multivariable analysis, poorer PCS was significantly associated with 11 variables, including older age, higher New York Heart Association class, previous cerebral infarction, being unemployed, and limited participation in physical education classes and sports clubs. Poorer MCS was associated with congenital heart disease of great complexity, being part of a non-sports club, current smoking, and social drinking. Student status and a higher number of family members were positively correlated with MCS.Conclusions: This study demonstrates that HRQoL in ACHD patients is associated with various clinical and sociodemographic factors. Further studies are needed to clarify whether some of these factors could be targets for future intervention programs to improve HRQoL outcomes.

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

Saori Yamamoto Tasuku Nagasawa Koichiro Sugimura Atuhiro Kanno Shunsuke Tatebe Tatsuo Aoki Haruka Sato Katsuya Kozu Ryo Konno Kotaro Nochioka Kimio Sato Hiroaki Shimokawa

出版者

The Japanese Society of Internal Medicine

雑誌

Internal Medicine (ISSN:09182918)

巻号頁・発行日

pp.0959-18, (Released:2018-10-17)

参考文献数

16

被引用文献数

8

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Anderson-Fabry disease (AFD) is a rare X-linked disorder caused by deficient activity of the lysosomal enzyme α-galactosidase A (α-GAL A). We herein report 10 cases of AFD in 5 families (3 men and 7 women) that were found to have a specific common mutation in R301Q (G-to-A transition in exon 6 [codon 301] resulting in the replacement of a glutamine with an arginine residue). We evaluated their clinical characteristics, residual enzymatic activity, and plasma concentrations of globotriaosylsphingosine (Lyso-Gb3). Although all 10 cases had cardiac and renal manifestations in common, their clinical manifestations were markedly divergent despite the same genetic abnormality.

著者

Jun Takahashi Kimio Satoh Koji Fukuda Koichiro Sugimura Yasuharu Matsumoto Makoto Nakano Ryuji Tsuburaya Tatsuo Aoki Kiyotaka Hao Kensuke Nishimiya Kenta Ito Yasuhiko Sakata Hiroaki Shimokawa

出版者

日本循環器学会

雑誌

Circulation Journal (ISSN:13469843)

巻号頁・発行日

pp.CJ-16-0644, (Released:2016-07-07)

参考文献数

6

被引用文献数

1

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The 80thAnnual Scientific Meeting of the Japanese Circulation Society was held in Sendai, Japan, on March 18–20, 2016, which coincided with the 5thanniversary of the Great East Japan Earthquake that hit the Tohoku area on March 11, 2011. Thus, the main themes for this meeting were “The Past, Present and Future of Cardiovascular Medicine in Japan” and “The 5thAnniversary of the Great East Japan Earthquake”. Despite the provincial location, approximately 15,000 people attended during the 3-day meeting, and there were in-depth discussions in each of the various sessions on these themes. Especially, to our great pleasure, the Japanese Royals, Emperor Akihito and Empress Michiko, kindly visited the panel exhibition of the Great East Japan Earthquake and spoke words of appreciation to us. The meeting successfully completed and we sincerely appreciate the great cooperation and support from all affiliates.