Epidemiology and Disease Management of Pulmonary Hypertension During The Last Two Decades in Japan

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 To evaluate the changes in characteristics and initial treatments for PAH in Japan over time. Methods We used data from the Japan Pulmonary Hypertension Registry (JAPHR) to compare patient characteristics and treatment practices between 2008–2015 (316 patients) and 2016–2020 (315 patients). Results The age (mean ± standard deviation) 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 mmHg (mean ± standard deviation). Idiopathic/hereditary PAH was the commonest etiology in both periods (50.0% and 51.1%). 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, respectively. 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. 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. To evaluate the changes in characteristics and initial treatments for PAH in Japan over time. We used data from the Japan Pulmonary Hypertension Registry (JAPHR) to compare patient characteristics and treatment practices between 2008–2015 (316 patients) and 2016–2020 (315 patients). The age (mean ± standard deviation) 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 mmHg (mean ± standard deviation). Idiopathic/hereditary PAH was the commonest etiology in both periods (50.0% and 51.1%). 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, respectively. Macitentan, tadalafil, selexipag, and epoprostenol were the most frequently prescribed drugs. 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.