["Guiding lights" for the diagnosis of chronic thromboembolic pulmonary hypertension in the flow of patients with pulmonary embolism].

On December 13, 2021, an expert council was held to determine the position of experts of different specialties regarding the reasons for the low level of diagnosis of chronic thromboembolic pulmonary hypertension (CTEPH) in real clinical practice in a pandemic of a new coronavirus infection and possible ways to improve detection in patients with pulmonary embolism (PE) ) in history. The reasons for the low level of diagnosis of CTEPH are the insufficient level of knowledge of specialists, especially primary care physicians; lack of clear regulatory documents and expert centers for the management of this category of patients. Primary diagnosis of CTEPH in a pandemic can be strengthened through the widespread use of telemedicine for consultations of primary care physicians with specialists from expert centers; to maximize the role of echocardiography and computed tomography (CT) as differential diagnostic tools for dyspnea, in particular in patients with COVID-19. To increase the detection rate of CTEPH, diagnostic vigilance is required in patients with risk factors and episodes of venous thromboembolism. To improve the screening of CTEPH, it is necessary to create an algorithm for monitoring patients who have had PE; provide educational activities, including through the media; create materials for patients with accessible information. The regulatory documents should designate the circle of responsible specialists who will be engaged in long-term monitoring of patients with PE. Educational programs are needed for primary care physicians, cardiologists, and other physicians who come into the field of view of patients with CTEPH; introduction of a program to create expert centers for monitoring and managing patients with the possibility of performing ventilation-perfusion lung scintigraphy, cardiopulmonary stress test, CT, right heart catheterization. It seems important to build cooperation with the Ministry of Health of Russia in order to create special protocols, procedures for managing patients with PE and CTEPH.

[Rehabilitation in patients with chronic thromboembolic pulmonary hypertension].

For patients with pulmonary hypertension (PH) there have been no recommendations for physical rehabilitation for a long time, and in fact physical activity was discouraged. Nowadays, the position regarding exercise in patients with PH has changed studies have shown the safety and effectiveness of exercise in these patients. Most of the studies published to date on rehabilitation in patients with PH have assessed its effect on exercise tolerance. This review provides an overview of published studies and their conclusions on the effectiveness of rehabilitation in patients with chronic thromboembolic hypertension being as a complex therapy using medical and surgical methods of treatment and rehabilitation. The inclusion of rehabilitation in complex treatment allows to achieve patients highest possible tolerance to physical activity, increase in the functional class, quality of life and restore working capacity.

[Russian Medical Society for Arterial Hypertension expert consensus. Resistant hypertension: detection and management].

The diagnosis of resistant arterial hypertension allows us to single out a separate group of patients in whom it is necessary to use special diagnostic methods and approaches to treatment. Elimination of reversible factors leading to the development of resistant arterial hypertension, such as non-adherence to therapy, inappropriate therapy, secondary forms of arterial hypertension, leads to an improvement in the patient's prognosis. Most patients with resistant hypertension should be evaluated to rule out primary aldosteronism, renal artery stenosis, chronic kidney disease, and obstructive sleep apnea. The algorithm for examining patients, recommendations for lifestyle changes and a step-by-step therapy plan can improve blood pressure control. It is optative to use the most simplified treatment regimen and long-acting combined drugs. For a separate category of patients, it is advisable to perform radiofrequency denervation of the renal arteries.

[The clinical case of successful combined treatment of refractory arterial hypertension. Case report].

In recent years, there has been an increase of patients with arterial hypertension, one of the variants of which is refractory arterial hypertension. This unfavorable clinical variant of the course of hypertension worries clinicians, due to the higher risk of developing cardiovascular complications, realizing the need for a better control of blood pressure. The presented clinical case demonstrates the successful combined treatment of refractory hypertension using antihypertensive therapy and renal denervation.

[New opportunities of renal denervation].

Radiofrequency renal artery denervation (RND) was introduced as a method of interventional treatment of resistant hypertension almost ten years ago. The first studies demonstrated the effectiveness of this procedure. This has led to growing interest in this area and the rapid development of the method. However, the results of the first randomized trial of SYMPLICITY HTN-3 questioned the role of RND in the treatment of resistant hypertension, this fact conducted to the decrease in the recommendation class and level of evidence in the Guidelines of the European Society of Cardiology and the European Society of Hypertension in 2018. To date, the medical community is actively discussing the results of recently published trials such as SPYRAL HTN-OFF MED, SPYRAL HTN-ON MED, RADIANCE-HTN SOLO and RADIOSOUND-HTN.

[Possibility of complex medicamental and endovascular treatment of pulmonary hypertension in Takayasu arteritis with predominant pulmonary arteries' lesion].

Takayasu arteritis (TA) is a systemic vasculitis with predominatly lesions of aorta and its large branches. In some cases pulmonary arteries (PA) are involved in the pathological inflammatory process and lead to the formation of pulmonary hypertension and significantly worse the prognosis. Timely development of lesion of PA, appointment of adequate therapy and surgical treatment can prevent irreversible damage of blood vessels and improve the prognosis. Perioperative administration of interleukin-6 inhibitor inhibitor (tocilizumab) in at patients with indications for vascular surgery, including angioplasty PA, should be considered as a promising approach to control the inflammatory activity of TA, reduce the dose of glucocorticoids and the risk of postoperative complications. We present the clinical experience of significant improvement in the patients condition was achieved by using two-stage balloon angioplasty on the background of control of the disease activity with interleukin-6 tocilizumab intravenously and specific therapy with riociguat and iloprost.

Balloon pulmonary angioplasty for patients with inoperable chronic thromboembolic pulmonary hypertension.

AIM: To evaluate the effectiveness of balloon pulmonary angioplasty (BPA) in patients with inoperable chronic thromboembolic pulmonary hypertension (CTEPH). MATERIALS AND METHODS: Forty patients with inoperable CTEPH were enrolled in this study. The indications were determined by multidisciplinary team. The average age of patients was 53.5 [43; 63] years. In 65% of cases patients had functional class III (according to WHO); the distance in the 6-minute walk test (6MWD) was 327 [280; 400] m; catheterization of the right heart revealed systolic pulmonary artery pressure (SPAP) 82 [64; 100] mm Hg, mean pulmonary artery (mPAP) 48.5 [38; 56] mm Hg, pulmonary vascular resistance (PVR) 784 [525; 1257] dyn·s/cm-5. Each patient underwent 6 BPA. RESULTS: The effectiveness of BPA was assessed 2 months after the last session. According to the data of right heart catheterization SPAP decreased by 27.3%, mPAP by 26%, PVR by 34.5% from baseline. After all series of BPA echocardiography and magnetic resonance imaging demonstrated reverse remodeling of the right heart. Also significant decrease in the level of BNP by 62%, increasing in 6MWD distance by 39% and improvement of the functional class up to I in 60% cases and up to II in 40% cases were noted. CONCLUSION: The results of the present study demonstrated a high efficacy of BPA allowing to normalize hemodynamic and clinical parameters, increasing the physical activity. Balloon pulmonary angioplasty is a new highly effective, safe method for treating patients with inoperable CTEPH.

[Evolution of pathogenetic therapy of pulmonary arterial hypertension].

Pulmonary arterial hypertension (PAH) is a severe, disabling disease characterized by an increase pressure in the pulmonary artery (PA), an increase pressure in the right atrium, and a decrease of the cardiac output. It combines several diseases: idiopathic pulmonary hypertension, inherited pulmonary hypertension, PAH induced by medication and toxins, PAH associated with systemic diseases of connective tissue, HIV infection, portal hypertension, congenital heart defects, schistosomiasis. In the absence of treatment, PAH quickly leads to insufficiency of the right heart and premature death. An effective PAH therapy did not exist for a long time. However, in 1987 there was established a positive effect of taking large doses of calcium channel blockers in patients, who "responded" to their prescription in the short term, and in recently several groups of specific drugs have been developed and approved for the treatment of this pathology: prostacyclin analogues and prostacyclin receptors agonists, endothelin receptor antagonists, phosphodiesterase type 5 inhibitors and soluble guanylate cyclase stimulators. Modern studies of treatment of PAH are based on the latest data of the molecular transmission mechanisms of intracellular and intercellular signals, the action of hormones and tissue enzymes. The available results of these studies allow to suggest the inclusion to clinical guidelines several new drugs for the pathogenetic treatment of PAH in the near future: receptor tyrosine kinase inhibitors, Rho - kinase inhibitors, immunosuppressants and type 2 activin receptor agonists, protein kinase C inhibitors, aromatase inhibitors and estrogen receptor antagonists, poly-(ADP-ribose)-polymerase inhibitors and bromodomain protein 4, elastase inhibitors. Some of the drugs have already passed the III phase of clinical trials (imatinib), others are at the preclinical stage or at the I-II phase tests (olaparib, enzastaurin, elafin).

[The State of Vascular Bed, Parenchyma and Perfusion of Lungs in Chronic Thromboembolic Pulmonary Hypertension According to Data of Subtraction Pulmonary Angiography].

AIM: to assess the state of vascular bed, parenchyma, and perfusion of lungs in patients with chronic thromboembolic pulmonary hypertension (CTEPH) using the method of subtraction computed tomography (CT). METHODS: CT pulmonary angiography (CTPA) was performed in 45 patients with verified CTEPH (18 men, 27 women, age 26-79 years) by CT scanner using the "Lung subtraction" standard protocol. Parameters analyzed were characteristics of the state of main pulmonary artery (MPA) and the right ventricle (RV), and calculated CT angiographic (CTA) obstruction and perfusion defect scores. RESULTS: Significant correlation was found between CTA obstruction score and perfusion defect score (r=0.34, p=0.02). Mean pulmonary arterial pressure (mPAP) correlated with MPA diameter (r=0.4, p=0.02), RV wall thickness (r=0.6, p=0.0003) and the ratio of MPA diameter to ascending aortic diameter (r=0.5, p=0.002). Significant correlation was also found between RV wall thickness and pulmonary vascular resistance (PVR) (r=0.4, p=0.04). Neither CTA obstruction score nor perfusion defect score correlated with PVR and mPAP. The data of CT did not correlate with results of 6-minute walk test. CONCLUSION: In patients with CTEPH subtraction CTPA allows carrying out complex diagnostics of the state of vascular bed, parenchyma and perfusion of the lungs.

[Chronic thromboembolic pulmonary hypertension developed in a female patient with lupus erythematosus and secondary antiphospholipid syndrome]. / Khronicheskaia tromboémbolicheskaia legochnaia gipertenziia, sformirovavshaiasia u bol'noi sistemnoi krasnoi volchankoi c vtorichnym antifosfolipidnym sindromom.

Chronic thromboembolic pulmonary hypertension (CTEPH) is one of the potentially curable forms of pulmonary hypertension, in which pulmonary thromboendarterectomy is the gold standard treatment. However, over the last decade, great attention has been given to a combined therapeutic approach including both drug therapy and surgical treatment and the application of endovascular technologies. This clinical case demonstrates the diagnostic difficulties of CTEPH and the opportunities of a comprehensive approach to therapy for the disease with mandatory assessment of preoperative surgical and medical treatment in order to improve the patient status and to prepare for surgery.

[The First Experience of Using Very Long Stents Covered With Sirolimus (4060 mm) in the Treatment of Patients With Extensive and Diffuse Lesions of the Coronary Arteries].

OBJECTIVES: To assess the clinical outcome of one year follow-up in patients with diffuse long lesions treated with extra-long sirolimus-eluting stents. METHODS AND RESULTS: 85 patients underwent implantation of very long BioMime stents during the period from March 2014 to February 2016. RESULTS: The angiographic success rate was 100%, clinical success was observed in 98.8% cases (one patient developed periprocedural MI). The additional back-up support for stent delivery was used in 11 (12.9%) patients (the "buddy wire" technique in 6 pts and Guidzilla catheter in 2 pts.). Cumulative MACE rate at 12 months was 9.4% with 1.1% myocardial infarction and 5.9% TLR. One patient died 6 month after stent implantation from massive pulmonary thromboembolism. None of the patients had subacute or late stent thrombosis. Follow-up angiography was performed in 48 (68.5%) patients, 5 (10.4%) patients demonstrated restenoses. CONCLUSION: The use of extra-long sirolimus-eluting stents is associated with good procedural and one-year clinical outcomes in complex patients with long and diffuse lesions.

[Subtraction CT angiopulmonographic assessment of the vascular bed and lung perfusion in patients with chronic thromboembolic pulmonary hypertension: A pilot study]. / Otsenka sosudistogo rusla i perfuzii legkikh u bol'nykh khronicheskoi tromboémbolicheskoi legochnoi gipertenziei s pomoshch'iu subtraktsionnoi KT-angiopul'monografii: rezul'taty predvaritel'nogo issledovaniia.

AIM: To evaluate the vascular bed and lung perfusion in patients with chronic thromboembolic pulmonary hypertension (CTEPH) by computed tomography (CT) and to compare the severity of pulmonary arterial (PA) thrombotic lesions concurrent with parenchymal perfusion disorders with angiopulmonographic findings and clinical and hemodynamic characteristics. SUBJECTS AND METHODS: In the period from November 2015 to May 2016, 22 patients (7 men, 15 women) aged 27 to 67 years with a verified diagnosis of CTEPH were examined using an Aquilion ONE VISION Edition 640 (Toshiba Medical Systems, Japan) CT scanner with a 320-row detector. Perfusion defect in this study was evaluated with the new software allowing one to combine contrast and contrast-free images by a subtraction method. CT data analysis included visual assessment of the vascular bed and lung parenchyma and quantitative assessment with perfusion map construction and semi-automatic determination of the obstruction index (OI) and perfusion defect index (PDI). OI was compared with PDI and mean LA pressure according to the data of right heart catheterization and 6-minute walk test. RESULTS: A statistically significant correlation was found between OI and PDI in patients with CTEPH (Pearson r=0.56; p=0.0065). There were no relationships between mean LA pressure and vascular perfusion indices (OI and PDI) or between vascular perfusion parameters and 6-minute walk test results. CONCLUSION: CT angiopulmonography in conjunction with pulmonary perfusion assessment (within one study) allows evaluation of the severity of vascular lesions and perfusion disorders and determination of the efficiency of treatment in patients with CTEPH.

[Possibilities of using levosimendan in patients with idiopathic pulmonary hypertension].

AIM: To evaluate the efficiency and safety of intravenous infusion of levosimendan in patients with idiopathic pulmonary hypertension (IPH) and its clinical, hemodynamic, and neurohumoral effects. SUBJECTS AND METHODS: The study included 9 patients (mean age 31.8 +/- 8.7 years) with Functional Class III and IV IPH and severe right ventricular failure (RVF). Levosimendan was administered as dropwise intravenous infusion. The patients' general condition, blood pressure, heart rate, and 6-minute walk test results were assessed at baseline and 24 hours after levosimendan infusion, and then following 12 weeks. The time course of changes in blood neurohumoral mediators was determined and transthoracic echocardiography performed. RESULTS: At 24 hours after initiation of levosimendan infusion, there were substantial reductions in pulmonary artery systolic pressure, mean pulmonary artery pressure, mean right atrial pressure, and pulmonary vascular resistance. There was a significant increase in exercise tolerance. The blood exhibited lower norepinephrine and NT-proBNP and higher epinephrine concentrations. At 12 weeks after termination of levosimendan infusion, the improved functional status was preserved in the patients with IPH. There were no noticeable changes in the achieved key hemodynamic parameters. Only one patient needed re-administration of the drug 12 weeks later. The achieved changes in vasoactive mediators were retained 12 weeks following levosimendan infusion. Levosimendan treatment was not followed by clinically relevant adverse reactions requiring infusion termination and therapy discontinuation. CONCLUSION: 24-hour intravenous levosimendan infusion in patents with FC III-IV IPH and severe RVF causes positive changes in the key hemodynamic parameters and neurohumoral status of the patients and promotes stabilization in those with IPH at 12-week follow-up.

[Endovascular radiofrequency denervation of renal arteries as an innovation method of treatment of refractory arterial hypertension. First experience in Russia].

Excessive activation of the sympathetic nervous system forms the basis of pathogenesis of essential arterial hypertension (AH). The present work was aimed at evaluating efficacy and safety of endovascular radiofrequency denervation of renal arteries in patients with AH refractory AH based on the initial first experience in with using this methodology in the Russian Federation. The interventions were carried out on December 14-15th, 2011 in the first five patients presenting with AH refractory to antihypertensive therapy consisting of three and more drugs in therapeutic doses, one of which was a diuretic. The selection criteria were systolic arterial pressure (SAP) ≥160 mm Hg or ≥150 mm Hg in the presence of type 2 diabetes mellitus. The obligatory conditions for selection were the preserved renal function [glomerular filtration rate (GFR) ≥45 ml/min] and the absence of the secondary form of AH. The procedure of denervation was performed in the conditions of roentgen-operating room using special Medtronic Ardian Simplicity Catheter System™. In all cases we managed to perform bilateral denervation of renal arteries with the radiofrequency effect in not less than 4 zones of each of vessels. Efficacy of each of the effect was registered with due regard for reaching certain temperature and values of impedance. The interventions were not accompanied by the development of any complications either in the area of manipulations or the site of puncture. Neither were there any complications from the side of the cardiovascular or excretory systems of the body. Diurnal monitoring of AP (DMAP) registered a significant decrease in SAP averagely from 174±12 to 145±10 mm Hg three days after the intervention. A persistent antihypertensive effect was confirmed by the DMAP findings one month after denervation - the SAP level averagely amounted to 131±6 mm Hg. Endovascular radiofrequency denervation of renal arteries is a safe and efficient method of treatment of AH resistant to multicomponent antihypertensive therapy.

[Using nonselective endothelin receptor antagonist in idiopathic pulmonary hypertension].

Idiopathic (primary) pulmonary hypertension (IPH) is a rare disease of unknown etiology, which is characterized by elevated pulmonary artery pressure, increased total pulmonary vascular resistance, frequently a malignant course with evolving right ventricular decompensation, and a fatal outcome. The diagnosis of IPH is established on the increments in the mean resting and exercise pulmonary artery trunk pressure by more than 25 and more than 30 mm Hg at rest and during exercise, respectively, with a normal pulmonary artery wedge pressure. Endothelin receptor antagonists (ERA) are one of the effective classes of drugs for the treatment of patients with IPH. Bosentan is the first drug from the ERA class that blocks the receptors of both types and that has been recommended by the WHO to treat patients with functional class II-IV pulmonary hypertension. The described case demonstrates the possibility of concomitantly using bosentan in a female patient with IPH shortly after ineffective treatment with a calcium antagonist.

[Coronary arterial perforation during endovascular intervention].

The paper deals with the timely detection and treatment of a menacing complication during endovascular interventions, such as coronary arterial perforation. It describes the causes of perforations, their classification, and an algorithm of actions to eliminate this complication. A clinical example demonstrates the sequence of operations for the detection and correction of coronary arterial perforation.

[Long term results of endovascular treatment of ischemic heart disease in arterial hypertension patients]. / Otdalennye rezul'taty éndovaskuliarnogo lecheniia ishemicheskoi bolezni serdtsa u bol'nykh arterial'noi gipertoniei.

AIM: To ascertain long-term prognosis after endovascular treatment of ischemic heart disease (IHD) in patients with arterial hypertension (AH). MATERIAL AND METHODS: The trial enrolled 87 IHD patients (mean age 54 +/- 8 years) who have undergone successful transluminal balloon coronary angioplasty (TBCA) or stenting. The patients were divided into two groups. Group 1 consisted of IHD patients with mild or moderate AH, group 2--of IHD patients without AH. The repeat examination was made 1-72 months (34 +/- 12) after TBCA, control coronarography was performed in 42 (48%) patients. RESULTS: The groups did not differ much by clinical and angiographic characteristics. Within 72 months the number of unfavourable clinical outcomes (UCO) in group 1 was significantly higher (68 and 19%, respectively; p = 0.02) as well as frequency of repeated TBCA (43 and 19%, respectively, p = 0.03). In both groups TBCA were repeated more frequently because of restenosis than of fresh lesions (91 and 33%, p < 0.0001; 70 and 20%, p = 0.04, respectively). The probability of no need in repeated TBCA was significantly less in group 1 than group 2 (41 and 72%, respectively; p = 0.007), the probability regressing more actively within 8 months after the intervention. CONCLUSION: Total incidence of UCO seventy two months after the endovascular treatment was higher in AH patients primarily due to repeated TBCA. Most of TBCAs were conducted within 8 months after the intervention for restenosis.

[X-ray morphology of coronary arteries in patients with arterial hypertension in late periods after endovascular treatment]. / Rentgenomorfologiia koronarnykh arterii u bol'nykh arterial'noi gipertoniei v otdalennye sroki posle éndovaskuliarnogo lecheniia.

An association of arterial hypertension (AH) and late angiographic outcomes of endovascular interventions was studied in coronary heart disease. Forty two patients with or without AH underwent repeated coronary angiography within 2 months after the procedure. The total number of restenoses was found to be significantly greater in patients with AH than in those without it. The diameter of restenoses was significantly smaller (70.2 +/- 24.7 vs 48.2 +/- 29.2%) and their area is significantly larger (82.6 +/- 19.6 vs 66.9 +/- 28.8). Absence probability of restenosis was significantly smaller in patients with AH (7 vs 27%); at the same time there was the greatest reduction in this probability in the first 8 months following the intervention.