Abstract: Pulmonary hypertension (PH) is a rare and life-threatening disease. Over past two decades, rapid advancements in treatment techniques have significantly improved the prognosis of two major subgroups of PH entities: pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH). Given the underlying pathological feature of in situ thrombosis of small arteries, anti-coagulation was previously considered to be a supportive therapy for PAH to improve prognosis. However, due to a lack of robust evidence, it is not recommended by the current guidelines. In contrast, lifelong anticoagulation is recommended for CTEPH patients to prevent thrombus recurrence and in situ thrombosis, based on the thromboembolic etiology. Furthermore, with the advent of direct oral anticoagulants, there are now more options for anti-coagulation therapy in PH. Nevertheless, due to the complexity of PH etiology and the heterogeneity of treatment approaches, anti-coagulation management remains challenging. This article reviews and evaluates the current status and safety issue of anti-coagulation therapy for PH patients, providing guidance and insights for clinical practice and research in this field.

Key words: pulmonary hypertension, chronic thromboembolic pulmonary hypertension, anti-coagulation, targeted drug, drug interactions