On the way to optimization approach for the treatment of chronic thromboembolic pulmonary hypertension

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Abstract

Chronic thromboembolic pulmonary hypertension (CTEPH) is unique and potentially curable pathology. However, if surgery is not possible in patients with persistent or residual CTEPH after thromboendarterectomy the specific therapy that is usually used in pulmonary arterial hypertension (PAH) can be prescribed. The first drug from the soluble guanylate cyclase promoters known as riociguat was approved in our country for the treatment of patients with inoperable and residual CTEPH in 2014. Riociguat is discussed as the drug of first choice in the Russian recommendations for the diagnosis and treatment of CTEPH 2015. Inhaled iloprost is recommended as 2nd-line permanent treatment in patients with inoperable CTEPH. Endothelin receptor antagonists and phosphodiesterase type 5 inhibitors are the third-line drugs. Common arrangements and standard treatment in case of CTEPH patients do not differ from the treatment approaches in PAH patients. CTEPH patients should take oral anticoagulants for the rest of life to maintain a target INR of 2.5-3.5. Drug of choice is warfarin - vitamin K antagonist. New anticoagulants are not recommended due to absence of statistical significance. Low molecular weight heparins are recommended as an alternative in case of intolerance to warfarin or increased risk of developing bleeding.

About the authors

T. V Martyniuk

Russian Cardiological Scientific-Industrial Complex of the Ministry of Health of the Russian Federation

Email: trukhiniv@mail.ru
д-р мед. наук, рук. лаб. легочной гипертензии отд. гипертонии ИКК им. А.Л.Мясникова ФГБУ РКНПК 121552, Russian Federation, Moscow, ul. 3-ia Cherepkovskaia, d. 15a

S. N Nakonechnikov

Russian Cardiological Scientific-Industrial Complex of the Ministry of Health of the Russian Federation

д-р мед. наук, проф., первый зам. ген. дир. ФГБУ РКНПК 121552, Russian Federation, Moscow, ul. 3-ia Cherepkovskaia, d. 15a

I. E Chazova

Russian Cardiological Scientific-Industrial Complex of the Ministry of Health of the Russian Federation

чл.-кор. РАН, д-р мед. наук, проф., и.о. ген. дир. ФГБУ РКНПК, рук. отд. гипертонии, дир. ИКК им. А.Л.Мясникова ФГБУ РКНПК 121552, Russian Federation, Moscow, ul. 3-ia Cherepkovskaia, d. 15a

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