How should a physician approach the pharmacological management of chronic thromboembolic pulmonary hypertension?

Chronic thromboembolic pulmonary hypertension (CTEPH) is characterized by the presence of organized thromboembolic material and proliferative fibrous intima occluding varying degrees of the pulmonary arteries, and is also accompanied by small vessel vasculopathy in occluded and non-occluded pulmonary vasculature. The similarity in hemodynamics and pathophysiology between CTEPH and group 1 pulmonary arterial hypertension provides the rationale for clinical use of pulmonary arterial hypertension (PAH)-specific therapy.The authors present the current knowledge concerning the updated therapeutic strategies in CTEPH, and try to illustrate the established and uncertain role of PAH-specific therapy and anticoagulation therapy. The real-world observational registries and landmark randomized controlled trials of PAH-specific drugs in CTEPH are emphasized in the manuscript.Despite surgical and interventional therapies, which could provide effective and potential curable treatments, medical therapies are still the substantial and irreplaceable option for patients with CTEPH. More and more PAH-specific drugs have demonstrated favorable efficacy and safety profiles in patients with CTEPH. Additionally, anticoagulation therapy is also a substantial medical treatment in all CTEPH patients without contradiction. However, the benefit-to-risk balance in life-long anticoagulation and whether more individualized anticoagulation strategies are needed warrants further investigation.