The Journal of Heart and Lung Transplantation
International Society for Heart and Lung Transplantation.

Anticoagulation in pulmonary arterial hypertension - association with mortality, healthcare utilization, and quality of life: The Pulmonary Hypertension Association Registry (PHAR)


      Routine long-term anticoagulation in pulmonary arterial hypertension (PAH) is controversial. To date, anticoagulation has been found to be beneficial or neutral in idiopathic disease (IPAH) and neutral-to-harmful in connective tissue disease (CTD-PAH). We sought to examine the association between anticoagulation and mortality, healthcare utilization, and quality of life (QoL) in PAH.


      The PHAR is a prospective registry of PAH patients referred to 58 pulmonary hypertension care centers in the United States. We compared patients who received anticoagulation during enrollment (questionnaire documented) to those who did not. Cox proportional hazard models were used for mortality, Poisson multivariate regression models for healthcare utilization, and generalized estimating equations for QOL


      Of 1175 patients included, 316 patients were treated with anticoagulation. IPAH/hereditary PAH (HPAH) comprised 46% of the cohort and CTD-PAH comprised 33%. After adjustment for demographics, clinical characteristics, site and disease severity, anticoagulation was not associated with mortality in the overall population (HR, 1.00; 95% CI, 0.72-1.36), IPAH/HPAH (HR, 1.19; 95% CI, 0.74-1.94), or CTD-PAH (HR 0.87; 95% CI, 0.53-1.42). Anticoagulation was associated with an increased rate of emergency department visits (IRR: 1.41), hospitalizations (IRR: 1.30), and hospital days (IRR 1.33). QOL measured by emPHasis-10 score was worse in patients receiving anticoagulation (mean difference 1.74; 95% CI 0.40-3.09).


      Anticoagulation is not associated with higher mortality, but is associated with increased healthcare utilization in the PHAR. PAH-specific QoL may be worse in patients receiving anticoagulation. The risks and benefits surrounding routine prescription of anticoagulation for PAH should be carefully considered.



      PH (Pulmonary Hypertension), PHAR (Pulmonary Hypertension Association Registry), PAH (Pulmonary arterial hypertension), CTD-PAH (Connective Tissue Disease associated pulmonary arterial hypertension), IPAH (Idiopathic pulmonary arterial hypertension), HPAH (Hereditary pulmonary arterial hypertension), QoL (Quality of life), RVSWI (Right ventricular stroke work index), PAPi (Pulmonary Artery Pulsatility Index), PAWP (Pulmonary rtery Wedge Pressure), PDE5i (Phosphodiesterase type 5 inhibitor), ERA (Endothelin receptor antagonist), SSc-PAH (Systemic Sclerosis associated pulmonary arterial hypertension), WHO FC (World Health Organization functional class)
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