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.
The aim of the work is to compare vectorcardiographic (VCG) variables - spatial QRS-T angle and electrocardiographic ventricular gradient (VG) with echocardiography (EchoCG) data in patients with idiopathic pulmonary hypertension (IPH) and chronic thromboembolic pulmonary hypertension (CTEPH). Materials and methods. In 40 patients with IPH and 40 patients with CTEPH at the age of 45±12 years, systolic pulmonary artery pressure (SPAP); the sizes of heart chambers, parameters of RV systolic and diastolic function were evaluated with EchoCG. The QRS-T and VG angles were calculated on the VCG, derived from 12-lead digital ECG. Results. In all patients SPAP was greater than 40 mm Hg (mean 83±18 mm Hg), EchoCG data indicated hypertrophy and dilatation of RV, its systolic and diastolic function; dilatation of the right atrium (RA). Prognostically unfavorable changes in EchoCG were observed: the presence of pericardial effusion in 35 (44%) patients, RA area greater than 26 cm2 in 18 (23%) patients; TAPSE less than 1.5 cm in 37 (46%) patients. EchoCG and VCG variables had statistically significant differences in patients with III-IV functional class in comparison with I-II functional class. Statistically significant moderate correlations between VCG and EchoCG variables were revealed. VCG variables allowed to separate patient groups with the presence and absence of prognostically unfavorable changes in EchoCG with sensitivity from 54 to 78% and specificity from 66 to 87%. Conclusion. In patients with IPH and CTEPH, changes of QRS-T angle and VG correlate with SPAP, the size of RV and RA, parameters of RV systolic and diastolic function. The possibility of the use of QRS-T angle and VG for the detection of patients with prognostically unfavorable echocardiographic changes in the general group of patients with IPH and CTEPH has been shown.
Aim. To conduct a comprehensive analysis of the clinical, functional, hemodynamic profile of patients with pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) and Eisenmenger syndrome, as compared with the group of idiopathic pulmonary hypertension (IPAH) according to the Russian Registry, and also to study the features of drug therapy. Materials and methods. The study involved 20 patients with PAH-CHD and Eisenmenger syndrome and 20 patients with IPAH over 18 years old. All patients had been entered in the Russian registry (NCT03707561). A comparative analysis of clinical, functional, hemodynamic parameters was held. The diagnosis was established in accordance with the algorithm proposed in the Russian guidelines the diagnosis and treatment of PH (2016). Results. In this study, 20 patients with PAH-CHD (16 women and 4 men) and Eisenmenger syndrome and 20 patients with IPAH (18 women and 2 men) were analyzed. Patients were comparable in age and sex. The time from the onset of symptoms to the final diagnosis in the PAH-CHD group was significantly longer: on average, it took three years to establish the diagnosis, whereas in patients with IPAH, on average, after 10.5 months, the correct diagnosis was made (p=0,0006). In the group of patients with Eisenmenger syndrome, there was a significant decrease in SpO2 both at rest and under exertion and averaged 86.5±9.7% at rest and 70±12.3% after exercise (p=0,0002). Conclusions. Patients with Eisenmenger syndrome compared with the IPAH group are characterized by a longer duration of symptoms until the diagnosis, and lower oxygen saturation. When estimating the parameters of pressure in the pulmonary artery (sPAP, dPAP, mPAP), according to the data of right heart catheterization and echo, significantly higher values of pressure in the pulmonary artery and lower values of SpO2 were detected.
Background. Pulmonary hypertension (PH) is a severe pathology that often leads a patient to death or disability. Recently, the development of PH associated with activation of the sympathetic nervous system (SNS) has been of interest. Some results obtained in an acute experiment on rats showed that activation of the carotid bodies of one external carotid artery is a sufficient stimulus to increase the tone of pulmonary arteries. Obviously, this effect is mediated by the sympathetic nervous system. However, the long-term effects of unilateral hypoxia of the carotid bodies on the morphofunctional state of the pulmonary arteries are not described in the literature. Aim. Effect assessment of common carotid artery bifurcation region ischemia due to the atherosclerotic process on the pulmonary arteries in patients. Materials and methods. The retrospective study was conducted in the Burdenko Neurosurgical Center and in the National Medical Research Center for Cardiology. A total of 60 case histories were analyzed in detail. All patients underwent Duplex scanning of the brachiocephalic arteries, as well as transthoracic echocardiography. The study included patients with atherosclerotic plaque in the area of the common carotid artery bifurcation on either one side or both. Exclusion criteria consisted of diseases that lead to the development of PH. The relationship between the presence of hemodynamically significant atheroma and the development of PH was evaluated. Results. Patients were divided into two groups those with hemodynamically significant atherosclerotic plaque in the common carotid artery bifurcation region at least on one side (more than 75% of the vascular obstruction) and hemodynamically insignificant atheromas in the common carotid artery bifurcation region on one or both sides (less than 45%). Among patients from the first group, 52.8% of the patients had signs of PH. Among patients from the second group, only 16.7% of the patients had signs of PH. The difference in the frequency of PH occurrence between the two selected groups is statistically significant (p=0.005). There were no differences in red blood cells number, platelets number, glucose concentration and lipid composition of blood plasma. Conclusion. Activation of SNS due to hemodynamically significant atherosclerotic plaque in the area of the bifurcation of the common carotid artery may be an independent mechanism for the development of PH. Key words: pulmonary hypertension, carotid bodies, atherosclerotic plaque, sympathectomy of pulmonary arteries.
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