Anticoagulation in pulmonary arterial hypertension - association with mortality, healthcare utilization, and quality of life: The Pulmonary Hypertension Association Registry (PHAR)
Section snippets
The pulmonary hypertension association registry (PHAR)
Characteristics of the PHAR, including rationale, criteria for enrollment, data collection procedures, and organizational structure have been described previously.20 The PHAR IRB approval is maintained at The University of Pennsylvania (Protocol Number 822830), which serves as single IRB. All patients provided informed consent for enrollment. Patients new to care at PHAR-associated pulmonary hypertension (PH) centers were non-consecutively enrolled beginning in 2015. The dataset analyzed in
Patient characteristics
There were 1175 patients that met inclusion criteria, with 316 patients anticoagulated during enrollment (Figure 1). The median duration of follow up was 839 days. Of the 540 patients with IPAH/HPAH, 176 (32.6%) were treated with anticoagulation and of the 389 patients with CTD-PAH, 91 (25.7%) were treated with anticoagulation. There were 638 patients (54.3%) that were diagnosed with PAH within 6 months of enrollment and therefore considered incident cases.
In the overall cohort, patients who
Discussion
In a large, multicenter, contemporary cohort, we found no association between mortality and anticoagulation treatment. Anticoagulation was consistently associated with increased healthcare utilization for the overall cohort, IPAH/HPAH subgroup, and the CTD-PAH subgroup in our primary analysis. These findings were durable on sensitivity analysis by anticoagulation definition for the overall cohort and CTD-PAH subgroup, but inconsistent for the IPAH/HPAH subgroup. This inconsistency suggests that
Conclusions
Anticoagulation may not have a role as routine therapeutic intervention in PAH. Anticoagulation does not reduce mortality and is associated with increased healthcare utilization with a signal to suggest a neutral or negative effect on QoL. Although early cohorts of PAH patients previously reported clinical mortality benefit to anticoagulation with a plausible physiologic hypothesis to support the findings, modern clinical cohorts such as the PHAR have not borne out a clear benefit.
Author contributions
JDG, NAK, and TDM conceived of the study. JDG, NAK, RK, TT, AH, DG, TB, MC, EH, MAS, and TDM participated in study design. JDG and RK performed statistical analysis. JDG wrote the first draft of the manuscript and NAK, RK, TT, AH, DG, TB, MC, EH, MAS, and TDM contributed to revising the manuscript. All authors have approved the final draft of the manuscript.
Financial Disclosure Statement
N.A.K. reports consulting fees from United Therapeutics, Janssen, and Acceleron and serves on advisory board for United Therapeutics, Janssen, and Bayer. R.K. reports grant support from the Pulmonary Hypertension Association. T.T. reports grant support from United Therapeutics, Aria CV, Acceleron Pharma, and Tenax Therapeutics, consulting fees from Acceleron Pharma, Altavant science, United Therapeutics, and Actelion, and serves on advisory board for Acceleron Pharma, Altavant science, United
Funding Sources
None
Acknowledgments
The Pulmonary Hypertension Association Registry (PHAR) is supported by Pulmonary Hypertension Care Centers, Inc., a supporting organization of the Pulmonary Hypertension Association. The authors thank the other investigators, the staff, and particularly participants of the PHAR for their valuable contributions. A full list of participating PHAR sites and institutions can be found at www.PHAssociation.org/PHAR.
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