Anticoagulation Improves Outcomes in Idiopathic PAH, but Not CTD-PAH

Pulmonary arterial hypertension (PAH). CT scan of the thorax
Systemic anticoagulation was found to improve quality-adjusted life years among patients with idiopathic pulmonary arterial hypertension but not among those with connective tissue disease-associated pulmonary arterial hypertension.

Systemic anticoagulation improves quality-adjusted life years (QALYs) among patients with idiopathic pulmonary arterial hypertension (PAH), a decision analysis in Pulmonology Circulation suggests.

However, researchers also found that in a model of patients with connective tissue disease-associated PAH (CTD-PAH), anticoagulation with vitamin K antagonists (VKAs) may worsen quality-adjusted survival.

A 31-state Markov simulation decision analytic model was used to examine outcomes associated with anticoagulation with VKA therapy vs no anticoagulation with VKA therapy in a model of patients with PAH. Patients could develop atrial fibrillation, stroke, deep vein thrombosis, or bleeding during each 1-month cycle in the simulation. The model included several different patient characteristics in the analysis, including sex, use of central catheter-based PAH therapy, type of PAH (eg, idiopathic or CTD-PAH), and use of oral contraceptive medication among women.

All cases of idiopathic PAH favored anticoagulation with VKAs, as represented by a gain of 0.43 to 0.51 QALY. Conversely, anticoagulation was considered detrimental in all cases of CTD-PAH, as indicated by a loss of 0.66 to 1.89 QALYs. For anticoagulation to be better favored in cases of CTD-PAH, the researchers suggest that the therapy would need a hazard ratio for PAH mortality of ≥0.95.

According to probabilistic sensitivity analyses of CTD-PAH in both women and men, the choice to not use anticoagulation was favored in 95.5% to 99.0% of 10,000 second-order Monte Carlo iterations, whereas anticoagulation was favored in 75.7% to 79.5% of simulations in men with idiopathic PAH and 97.0% to 98.3% of simulations in women with idiopathic PAH.

A limitation of the study included the modeling of only the effects of VKAs rather than other frequently used anticoagulation therapies.

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The finding that systemic anticoagulation with VKA is beneficial for improving quality of life and outcomes in patients with idiopathic PAH provides “the basis of future shared decision-making tools for clinical use in determining PAH therapeutic strategies at the bedside.”


Jose A, Eckman MH, Elwing JM. Anticoagulation in pulmonary arterial hypertension: a decision analysis. Pulm Circ. 2019;9(4):1-12.

This article originally appeared on Pulmonology Advisor