Clinical Management and Outcomes of Patients With Portopulmonary Hypertension Enrolled in the Japanese Multicenter Registry

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

doi:10.1253/circrep.CR-22-0098

Pulmonary Circulation

Clinical Management and Outcomes of Patients With Portopulmonary Hypertension Enrolled in the Japanese Multicenter Registry

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

Author information

Yudai Tamura
Cardiovascular Center, International University of Health and Welfare (IUHW) Mita Hospital [Japan]
Yuichi Tamura
Pulmonary Hypertension Center, IUHW Mita Hospital [Japan]
Yu Taniguchi
Division of Cardiovascular Medicine, Department of Internal Medicine, Kobe University Graduate School of Medicine [Japan]
Ichizo Tsujino
Division of Respiratory and Cardiovascular Innovative Research, Faculty of Medicine, Hokkaido University [Japan]
Takumi Inami
Department of Cardiovascular Medicine, Kyorin University School of Medicine [Japan]
Hiromi Matsubara
National Hospital Organization Okayama Medical Center [Japan]
Ayako Shigeta
Department of Respirology, Graduate School of Medicine, Chiba University [Japan]
Yoichi Sugiyama
Division of Cardiovascular Medicine, Department of Internal Medicine, Kurume University School of Medicine [Japan]
Shiro Adachi
Department of Cardiology, Nagoya University Hospital [Japan]
Kohtaro Abe
Department of Cardiovascular Medicine, Kyushu University Graduate School of Medical Sciences [Japan]
Yuichi Baba
Department of Cardiology and Geriatrics, Kochi Medical School, Kochi University [Japan]
Masaru Hatano
Department of Cardiovascular Medicine, Graduate School of Medicine, The University of Tokyo [Japan]
Satoshi Ikeda
Department of Cardiovascular Medicine, Nagasaki University Graduate School of Biomedical Sciences [Japan]
Kenya Kusunose
Department of Cardiovascular Medicine, Tokushima University Hospital [Japan]
Koichiro Sugimura
Department of Cardiology, IUHW, Narita Hospital [Japan]
Soichiro Usui
Department of Cardiovascular Medicine, Kanazawa University Graduate School of Medical Sciences [Japan]
Yasuchika Takeishi
Department of Cardiovascular Medicine, Fukushima Medical University [Japan]
Kaoru Dohi
Department of Cardiology and Nephrology, Mie University Graduate School of Medicine [Japan]
Saki Hasegawa-Tamba
Department of Cardiology, Saitama Medical University International Medical Center [Japan]
Koshin Horimoto
Department of Cardiology, Matsuyama Red Cross Hospital [Japan]
Noriko Kikuchi
Department of Cardiology, Tokyo Women’s Medical University [Japan]
Hiraku Kumamaru
Department of Healthcare Quality Assessment, Graduate School of Medicine, The University of Tokyo [Japan]
Koichiro Tatsumi
Department of Respirology, Graduate School of Medicine, Chiba University [Japan]
on behalf of the Japan Pulmonary Hypertension Registry Network

Keywords: Combination therapy, Multicenter registry, Portopulmonary hypertension, Pulmonary arterial hypertension, Survival

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Supplementary material

2022 Volume 4 Issue 11 Pages 542-549

DOI https://doi.org/10.1253/circrep.CR-22-0098

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Abstract

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.

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