Regional Disparities in Pulmonary Arterial Hypertension Care in Japan ― Beyond the Availability of Targeted Therapies ―

The authors defined PH specialized facilities as those participating in the Japan Pulmonary Hypertension Registry (JAPHR).As shown in the Figure, 13 prefectures do not have participating PH specialized facilities, and this uneven geographic distribution would contribute directly to disparities in referral patterns and treatment strategies. Pulmonary hypertension (PH) is a life-threatening disease when diagnosis and therapeutic intervention are delayed, 1,2 so accurate diagnosis and early initiation of appropriate therapy by PH specialists are essential for improving hemodynamics, exercise capacity, and longterm prognosis.When echocardiography suggests a high probability of PH, current PH guidelines 3 recommend referral to specialized PH centers, where PH experts investigate the etiology and classify PH according to established clinical classifications.Pulmonary arterial hypertension (PAH) is characterized by pulmonary artery stenosis and occlusion caused by vasoconstriction, vascular remodeling, and thrombosis.With the development of PAH-targeted therapies, contemporary guidelines propose treatment algorithms incorporating these agents.4 After excluding significant cardiac or respiratory comorbidities, pulmonary vasoreactivity testing is recommended.In patients without vasoreactivity, comprehensive risk is assessed for initial therapy.Current guidelines recommend initial combination therapy with endothelin receptor antagonists and phosphodiesterase type 5 inhibitors for low-and intermediate-risk patients, and triple combination therapy including parenteral prostacyclin for high-risk patients.Reassessment is recommended after approximately 3 months, with treatment escalation aimed at achieving a lower risk profile.In this issue of the Journal, Iwahori et al. 5 present their investigation of access to PH specialized facilities and initial treatment strategies for PAH in Japan using the National Database of Health Insurance Claims.They demonstrate that, in real-world clinical practice, most PAH patients are diagnosed by echocardiography and initially treated with oral monotherapy at non-specialized facilities.Notably, patients aged ≥70 years were less likely to visit PH specialized facilities and less likely to receive combination therapy.Marked regional disparities were observed in both access to specialized care and treatment strategies.These findings raise an important question: why do such disparities persist despite the widespread availability of PAH-targeted Article p ????