The clinical characteristics, treatment, and survival of portopulmonary hypertension in Japan

Portopulmonary 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, a...

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Bibliographic Details
Published in:BMC pulmonary medicine 2021-03, Vol.21 (1), p.89-89, Article 89
Main Authors: Takahashi, Yukiko, Yamamoto, Keiko, Sakao, Seiichiro, Takeuchi, Takao, Suda, Rika, Tanabe, Nobuhiro, Tatsumi, Koichiro
Format: Article
Language:English
Subjects:
Care and treatment
Classification
Disease
Heart
Heart failure
Hemodynamics
Hospitals
Hypertension
Mortality
Patient outcomes
Patients
PoPH
Portal hypertension
Portopulmonary hypertension
Pulmonary arterial hypertension
Pulmonary arteries
Pulmonary hypertension
Pulmonology
Registration
Survival
Veins & arteries
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Summary:Portopulmonary 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. Pulmonary 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). Both 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. As 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.
ISSN:1471-2466
1471-2466
DOI:10.1186/s12890-021-01452-3