Abstract
Patients with pulmonary arterial hypertension (PAH) experience long delays in diagnosis; however, little is known about the time between diagnosis and initiation of guideline-recommended optimal treatment. Immediate therapy is imperative for optimizing patient outcomes, as much of the pulmonary vasculature has already deteriorated by the time of diagnosis. This study used real-world U.S. data to investigate the time from the most recent right heart catheterization (RHC) to initiation of the first PAH-specific treatment. Adults with PAH were identified in the Komodo Health database using claims for RHC, pulmonary hypertension diagnostic codes, and PAH medications (1/1/2016-4/1/2024). Time from RHC to medication dispensing was examined and categorized by initial treatment regimen (monotherapy, dual therapy, and triple therapy). Baseline characteristics and treatment patterns were assessed. Among 7952 patients, the median time from most recent RHC to first PAH medication was 43.0 days. Most patients (77.7%) initially received monotherapy, with 20.0% and 2.3% receiving upfront dual and triple therapy, respectively. Cardiac comorbidities were present in most patients (85.8%). Among 2965 (48.0%) upfront monotherapy users who added another treatment during follow-up, the median time to dual therapy was 39 days. Of those who escalated to dual therapy, 567 (19.1%) eventually escalated to triple therapy, with a median time from dual therapy to triple therapy of 62 days. In real-world practice, delays between PAH diagnosis and initiation of optimal therapy remain significant, and barriers to access should be addressed.