- 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
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.