Abstract
The use of anticoagulant therapy in patients with pulmonary arterial hypertension (PAH) has been controversial for decades. Recommendations for anticoagulation in these patients are often derived from small, retrospective, and single centre studies without any placebo-controlled randomized study. Furthermore, uncertainties exist regarding a number of issues such as patient selection, risk stratification for bleeding, the intensity of anticoagulation, appropriateness of anticoagulation in different types of PAH, and the potential use of new oral anticoagulants.Recently, the database of the Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension (COMPERA) has been analyzed to assess the effect of anticoagulation on the long-term outcome of patients with various forms of PAH. This analysis is the largest to date to assess anticoagulant therapy in PAH patients in a prospective design with long observation period. The results of COMPERA lend support to current recommendations for the use of anticoagulant therapy in patients with idiopathic PAH, but not in other forms of PAH. Also, the study confirmed the previously reported concern that anticoagulant therapy may be harmful in patients with scleroderma-associated PAH.
Highlights
The exact role of chronic thrombosis in the pulmonary arteries in patients with pulmonary arterial hypertension (PAH) is controversial
In two retrospective studies evaluating histopathology in idiopathic PAH patients, the prevalence rates for chronic organized pulmonary vascular thromboses were 56% and 57%.3 – 4 (2) Evidence that PAH is associated with prothrombotic abnormalities, causing in situ thrombosis
Results of the COMPERA registry open the gate for several unanswered questions related to criteria that should be used to select patients for anticoagulant therapy; risk stratification for bleeding; the optimum target international normalized ratio (INR); the potential role of new oral anticoagulants; and the need for further randomized controlled trials
Summary
The exact role of chronic thrombosis in the pulmonary arteries in patients with pulmonary arterial hypertension (PAH) is controversial. In two retrospective studies evaluating histopathology in idiopathic PAH patients (formerly called primary pulmonary hypertension), the prevalence rates for chronic organized pulmonary vascular thromboses were 56% and 57%.3 – 4 (2) Evidence that PAH is associated with prothrombotic abnormalities, causing in situ thrombosis.
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