Abstract
<h3>Purpose</h3> The relationship between initial treatment strategy and survival in pulmonary arterial hypertension (PAH) is uncertain, although early combination therapy is proposed by current guidelines for majority of patients. <h3>Methods</h3> Data were extracted from the Pulmonary Hypertension Society of Australia and New Zealand registry for all patients with a diagnosis with idiopathic/heritable/toxin-induced (I/H/D-PAH) with survival, treatment response and medication data. Patients were divided into prevalent (diagnosed < 2011), early incident (diagnosed 2011-2015) and recent incident (diagnosed 2016-2019) cohort. Early combination therapy was defined as the use of 2 or more drugs within 3 months of treatment initiation. <h3>Results</h3> A total of 489 patients diagnosed between 2006 and 2019 were included with 318 (65%) started on monotherapy, 156 (32%) on early double combination therapy and 13 (3%) on early triple therapy. All patients on triple therapy were on parenteral prostacyclin, as well as 20 (13%) patients of double therapy and 2 (0.6%) receiving monotherapy. Rates of early combination therapy (double or triple) were 20%, 28% and 59% across the 3 treatment eras. In the entire population, there was no significant differences in transplant-free survival according to upfront treatment strategy (log rank test p=0.66). After adjustment for baseline risk according to REVEAL 2.0 score, HR for monotherapy compared to early combination therapy (2 or 3 agents) was 1.11 [0.79-1.62]. With analysis restricted to incident cases only, the adjusted HRs for monotherapy compared to early combination were 1.57 [0.86-2.84] for the period 2011-2015, and 0.78 [0.25-2.40] for period 2016-2019. Change in 6MWD was higher in the early combination group versus monotherapy group (51±86m vs 74±113m, p=0.017). <h3>Conclusion</h3> In a real-world registry where early combination therapy consisted mainly of dual oral agents, a survival benefit over monotherapy was not observed. Further studies are needed to determine impact of specific drug combinations (such as inclusion of parenteral prostacyclin) on long term outcomes.
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Disclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.