Changes in the Characteristics and Initial Treatments of Pulmonary Hypertension Between 2008 and 2020 in Japan.
JACC Asia
; 2(3): 273-284, 2022 Jun.
Article
em En
| MEDLINE
| ID: mdl-36338395
ABSTRACT
Background:
Pulmonary arterial hypertension (PAH) is a rare, progressive disease. The treatment landscape for PAH in Japan has evolved considerably in recent years, but there is limited knowledge of the changes in treatment practices or patient characteristics.Objectives:
The aim of this study was to evaluate the changes in characteristics and initial treatments for PAH in Japan over time.Methods:
This study used data from the Japan Pulmonary Hypertension Registry (JAPHR) to compare patient characteristics and treatment practices between 2008-2015 (n = 316) and 2016-2020 (n = 315).Results:
The mean ± standard deviation age at diagnosis increased from 47.9 ± 16.7 years in 2008-2015 to 52.7 ± 16.9 years in 2016-2020. The mean pulmonary arterial pressure decreased from 45.4 ± 15.0 to 38.6 ± 13.1 mm Hg. Idiopathic/hereditary PAH was the most common etiology in both periods (50.0% and 51.1%, respectively). The proportion of patients prescribed oral/inhaled combination therapies increased from 47.8% to 57.5%. Oral/inhaled combination therapies were frequently prescribed to patients with congenital heart disease-related PAH (81.8%). There was no significant trend in prescribing practices based on French low-risk criteria among patients with 0, 1, 2, 3, or 4 criteria, 53.8%, 68.8%, 52.8%, 66.7%, and 39.4% were prescribed oral/inhaled combination therapies, and 0%, 16.7%, 27.0%, 17.3%, and 15.2% were prescribed oral/inhaled monotherapies. Macitentan, tadalafil, selexipag, and epoprostenol were the most frequently prescribed drugs.Conclusions:
The severity of PAH decreased over time in Japan. Oral/inhaled combination therapies were generally preferred. Physicians generally prescribed therapies after considering the patients' hemodynamics and clinical severity. (Japan Pulmonary Hypertension Registry [JAPHR]; UMIN000026680).
6MWD, 6-minute walk distance; AMED, Agency for Medical Research and Development; BNP, brain natriuretic peptide; CHD-PAH, congenital heart diseaserelated pulmonary arterial hypertension; CTD-PAH, connective tissue diseaserelated pulmonary arterial hypertension; ERA, endothelin receptor antagonist; HPAH, hereditary pulmonary arterial hypertension; IPAH, idiopathic pulmonary arterial hypertension; JAPHR, Japan Pulmonary Hypertension Registry; Japan; NO, nitric oxide; NYHA, New York Heart Association; PAH, pulmonary arterial hypertension; PGI2, prostacyclin; PH, pulmonary hypertension; PVR, pulmonary vascular resistance; PoPH, portopulmonary hypertension; mPAP, mean pulmonary arterial pressure; mRAP, mean right arterial pressure; pulmonary arterial hypertension; risk criteria; treatment
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Idioma:
En
Revista:
JACC Asia
Ano de publicação:
2022
Tipo de documento:
Article
País de afiliação:
Japão