Introduction: Guideline consensus regarding the use of PAH-specific therapies for the treatment of pulmonary hypertension (PH) secondary to left-heart disease (WHO group 2) or chronic lung disease (WHO group 3) has moved from potential benefits, to limited use by expert centers, to recommendations against use. We sought to understand the trends in the real world use of PAH-specific therapies among a cohort of patients with PH.
Methods: We identified patients with incident PH from the Veterans Affairs healthcare system from 2010 to 2016 using a validated claims-based algorithm. Patients were classified by etiology of PH: WHO group 1 (PAH), group 2, group 3, group 4 (CTEPH), or unknown. Patients could belong to more than one group, with the exception of group 1. The use in PAH-specific pharmacotherapies in the first year after diagnosis and trends in the rates of use of pharmacotherapies from 2010 to 2016 were determined.
Results: Among 111,356 incident cases of PH, 7,799 (7.0%) had group 1 PH, 71,615 (64.3%) had group 2 PH, 43,115 (38.7%) had group 3 PH, 4,084 (3.7%) had group 4, and 16,883 (15.2%) were unknown. A PAH-specific medication was use in 1,358 (17.4%) of group 1, 7,616 (10.6%) of group 2, 5,948 (13.8%) of group 3, 499 (12.2%) of group 4, and 2,395 (14.2%) of the unknown group. From 2010 to 2016, there was no increase in pharmacotherapy use (P Value = 0.86) among group 1 patients while there was an increase in use among group 2 and group 3 patients (P < 0.001).
Conclusions: We found increasing use of PAH-specific pharmacotherapies among patients with group 2 and group 3 PH despite guidelines evolving to recommend against use over a similar time period. A greater understanding of the reasons for potentially inappropriate use would allow targeted interventions aimed at reducing misuse.