Observational studyBMC pulmonary medicine2021
The clinical characteristics, treatment, and survival of portopulmonary hypertension in Japan.
Observational study in BMC pulmonary medicine, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 7 papers.
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The trial behind it
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Who cites it
7 citing papers in PubMed, 9 citations in OpenAlex.
- Safety of Macitentan for the Treatment of Portopulmonary Hypertension: Real-World Evidence from the Combined OPUS/OrPHeUS Studies.Pulmonary therapy · 2024Article
- Real-world clinical practice of pulmonary arterial hypertension in Japan: Insights from a large administrative database.Pulmonary circulation · 2023Article
- Current Therapy and Liver Transplantation for Portopulmonary Hypertension in Japan.Journal of clinical medicine · 2023Review
- Current clinical understanding and effectiveness of portopulmonary hypertension treatment.Frontiers in medicine · 2023Review
- Clinical Management and Outcomes of Patients With Portopulmonary Hypertension Enrolled in the Japanese Multicenter Registry.Circulation reports · 2022Article
- Treatment pattern and clinical outcomes in portopulmonary hypertension: A database study in Japan.JGH open : an open access journal of gastroenterology and hepatology · 2022Article
- The Different Effects of Direct Bilirubin on Portopulmonary Hypertension and Idiopathic Pulmonary Arterial Hypertension.International journal of clinical practice · 2022Article
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Authors and funding
7 authors at 3 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundPortopulmonary hypertension (PoPH) refers to the simultaneous presentation of pulmonary arterial and portal hypertension. However, few reports have included the characteristics and treatments for patients with PoPH of Asian population; thus, we investigated the clinical characteristics, treatment, and survival of these patients in a Japanese cohort.
methodsPulmonary arterial hypertension (PAH) has been included in the National Research Project on Intractable Disease in Japan; therefore, we extracted data of patients with PoPH from the forms of newly registered cases of the project from 2012 to 2013 (for 2 years), and updated cases of the project in 2013 (Study 1, n = 36 newly registered forms, n = 46 updated forms). Additionally, for Study 2, we performed a retrospective, observational cohort study at Chiba University Hospital (n = 11). We compared the characteristics between patients with PoPH and those with idiopathic/heritable PAH (I/H-PAH).
resultsBoth studies showed higher cardiac outputs (COs) and cardiac indexes (CIs), lower pulmonary vascular resistance (PVR), and less treated with combination therapy in patients with PoPH than those with I/H-PAH. In Study 2, the overall and disease-specific survival between PoPH and I/H-PAH were similar. Conversely, many patients (45%) had to change their PAH-specific medicine because of adverse effects.
conclusionAs seen in western countries, Japanese patients with PoPH showed higher COs and CIs, better exercise tolerance, and lower PVRs than patients with I/H-PAH. Further studies are needed to improve PoPH treatments.
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