A systematic review as a method of gathering scientific evidence into clinical guidelines: CHEST-2019 guideline for the therapy pulmonary arterial hypertension in adults

Racionalʹnaâ farmakoterapiâ v kardiologii

2022

Pulmonary hypertension is a severe and often rapidly progressive disease with a fatal outcome. Approved specific therapy with five classes of drugs – prostanoids, endothelin receptor antagonists, phosphodiesterase-5 inhibitors, soluble guanylate cyclase stimulators and prostacyclin receptor agonists has significantly improved the prognosis of patients. An accessible and reproducible criterion of exercise tolerance – a distance walked in six-minute walk test (6MXD) – occupies one of the central places among the performance indicators (clinical, echocardiographic and hemodynamic) of specific pulmonary vasodilators. Reflecting the clinical severity of pulmonary hypertension and, according to some data, its long-term prognosis, the dynamics of 6MXD is an integral component of the combined primary endpoints of randomized controlled trials. In the CHEST-2019 recommendations, the dynamics of the 6MXD was used to standardize the randomized controlled trials. The 6MXD is indispensable for dynamic observation and decisionmaking on further treatment tactics

Section: динамика дистанции 6мх как составляющая комбинированных перв...unclassified

Section: динамика дистанции 6мх в качестве стандартизирующего рки пок...unclassified

Dynamics of 6-Minute Walk Distance as a Predictor of Efficiency Specific Pulmonary Vasodilators

Racionalʹnaâ farmakoterapiâ v kardiologii

2022

“…Стратегической целью лечения больных ЛГ согласно российским, евразийским и европейским рекомендациям является достижение и длительное сохранение статуса низкого риска [2-6, [31][32][33][34][35]. При этом традиционный подход к повышению эффективности специфической терапии при прогрессировании легочной сосудистой болезни -добавление новых препаратов с сохранением предыдущих (так называемая последовательная комбинированная терапия)в наши дни пересматривается [31,[34][35][36][37][38][39].…”

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Optimization of specific therapy for pulmonary hypertension: the possibilities of riociguat

Martynyuk

Витальевна²,

Terapevticheskii arkhiv

et al. 2021

Pulmonary hypertension (PH) is a severe and often rapidly progressive disease with fatal outcome. Endothelial dysfunction in PH is associated with decreased nitric oxide production. After reviewing the mechanisms of action and the evidence base for specific therapy with phosphodiesterase 5 inhibitors (PDE-5) and soluble guanylate cyclase stimulators, a reseach review on switching from PDE-5 to riociguat is conducted. A potential advantage of riociguat is its independence from endogenous nitric oxide and from the other (besides PDE-5) isoenzymes of phosphodiesterases. The favorable efficacy profile of sildenafil has been proven for the main forms of pulmonary arterial hypertension, of riociguat for the main forms of pulmonary arterial hypertension and chronic thromboembolic PH. The clinical efficacy of replacing PDE-5 with riociguat has been demonstrated in uncontrolled trials and in the randomized controlled study REPLACE. The possibility of therapy optimization by switching from IFDE-5 to riociguat is fixed in the Russian (class and level of evidence B-3) and Eurasian (class and level of evidence IIb-B) clinical guidelines, as well as in the materials of the Cologne Expert Consensus. An additional argument for switching is the lower cost as compared to combination therapy in the Russian Federation. According to the Russian and Eurasian guidelines for PH and the Russian instructions for the use of riociguat, the drug should be taken at least 24 hours after sildenafil discontinuation.

Advantages and limitations of initial combination therapy in pulmonary arterial hypertension patients in Russia

Terapevticheskii arkhiv

Горбачевский

2020

Pulmonary arterial hypertension (PAH) is severe and often times rapidly progressive disease with fatal outcome. The concept of initial combination of PAH-specific therapies in high risk patients at baseline was first described in the European guidelines on pulmonary hypertension (PH) in 2009, and in low or intermediate risk patients at baseline in 2015. Interestingly, that in Cologne Experts Consensus, and then in the 6th World Symposium on PH medical community started considering initial combination therapy as one of the most important pillars in PAH treatment algorithms in 2018. As of August 2020, as many as 8 formulations of 7 reference PAH-specific drugs are licensed for medical use in the Russian Federation. On top of that, 6 abbreviated drugs (generics) have also become available few years ago. Unfortunately, intravenous and subcutaneous prostacyclin analogs (PCA) and tadalafil are not approved for PH patients treatment in the Russian Federation. In this narrative review paper we attempted to describe studies on initial dual combination therapy with PAH-specific drugs registered in Russia, i.e. ambrisentan and riociguat, macitentan and riociguat, macitentan and sildenafil in low or intermediate risk patients at baseline, as well as iloprost inhaled and sildenafil, iloprost inhaled and bosentan in high risk patients. Some beneficial pharmacological effects due to the synergy between ambrisentan plus riociguat, and inhaled iloprost plus sildenafil appear to be interesting and require further clinical confirmation. Other initial combinations of PAH-specific agents require large-scale clinical trials as well.