Use of Anticoagulants in Patients with Pulmonary Hypertension

Abstract

Since the earliest works on the understanding of different forms of pulmonary hypertension, thrombosis has been involved in the pathophysiology of the disease, both in pulmonary arterial hypertension (PAH) and in chronic thromboembolic pulmonary hypertension (CTEPH). Autopsy and then pathophysiological data paved the way for the use of anticoagulants as a treatment for PAH and CTEPH. In PAH their role has diminished with the advent of specific targeted therapies, but they are still prescribed in more than half of PAH patients, because of concomitant venous thromboembolism or atrial fibrillation. In CTEPH long-term anticoagulant therapy is the cornerstone of the management. The recent development of direct oral anticoagulants (DOACs) raises the question of the best anticoagulation strategy, both in patients with PAH and in patients with CTEPH. In this review, we present an overview of the history of anticoagulants in the management of patients suffering from PAH or CTEPH, an update of the available data on the underlying rationale of their use in these subjects, an alert on the potential risks of using DOACs in these poorly explored situations, and the setting up of dedicated trials to evaluate the best anticoagulant treatment strategies in patients suffering from PAH or CTEPH.

2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension

“…In patients with very high Hb levels (>20 mg/dL), standard international normalized ratio measures are less accurate, and citrate-adjusted blood bottles must be used. Regarding using novel oral anticoagulants (NOACs), a large, nationwide, German, adult CHD database (including 106 NOAC-treated patients with Eisenmenger syndrome) showed that NOAC users had higher long-term risk of bleeding, major adverse cardiovascular events, and mortality compared with those on VKAs, suggesting that initiating NOACs should be reserved for experienced adult CHD centres, carefully weighing potential benefits and risks [572,573].…”

Sex and gender in pulmonary arterial hypertension

“…Anticoagulation with vitamin K antagonists (VKA) is sometimes used as an adjunctive treatment in PAH, with observational data suggesting better survival, particularly in patients with iPAH [140,141]. Currently, there are no strong recommendations on anticoagulant therapy in group 1 PAH and no data available for direct oral anticoagulants (DOACs) [142].…”

Epidemiology and Management of Chronic Thromboembolic Pulmonary Hypertension in Greece. Real-World Data from the Hellenic Pulmonary Hypertension Registry (HOPE)

“…Data suggestive of the appropriate form of anticoagulation therapy in CTEPH are lacking. Traditional anticoagulation with oral vitamin K antagonists is currently recommended and whether the new oral anticoagulants are adequate in CTEPH is currently unknown [18,24].…”