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Disclaimer. The EAC Guidelines represent the views of the EAC, and were produced after careful consideration of the scientific and medical knowledge, and the evidence available at the time of their publication. The EAC is not responsible in the event of any contradiction, discrepancy, and/or ambiguity between the EAC Guidelines and any other official recommendations or guidelines issued by the relevant public health authorities, in particular in relation to good use of healthcare or therapeutic strategies. Health professionals are encouraged to take the EAC Guidelines fully into account when exercising their clinical judgment, as well as in the determination and the implementation of preventive, diagnostic, or therapeutic medical strategies; however, the EAC Guidelines do not override, in any way whatsoever, the individual responsibility of health professionals to make appropriate and accurate decisions in consideration of each patient’s health condition and in consultation with that patient and, where appropriate and/or necessary, the patient’s caregiver. Nor do the EAC Guidelines exempt health professionals from taking into full and careful consideration the relevant official updated recommendations or guidelines issued by the competent public health authorities, in order to manage each patient’s case in light of the scientifically accepted data pursuant to their respective ethical and professional obligations. It is also the health professional’s responsibility to verify the applicable rules and regulations relating to drugs and medical devices at the time of prescription.

Modern pathogenetic therapy of idiopathic pulmonary arterial hypertension (IPAH), a severe life-threatening cardiovascular disease of unknown etiology, leads to a positive clinical effect due to reverse remodeling of the vessels of the microvasculature of the lungs. Highly effective drugs of specific therapy that act on the main targets of pathogenesis have now been introduced into clinical practice.The presented clinical case of a patient with diagnosed in 2014 IPAH with an initial functional class III according to the WHO classification demonstrates high long-term efficacy and safety of specific therapy based on the use of the soluble guanylate cyclase stimulator riociguat for 5 years after replacing previous therapy with sildenafil with further implementation of the strategy of sequential combination therapy due to the addition of ambrisentan and selexipag.

Objective. To determine the significance of renal artery stenosis according to different methods of assessing the functional significance of stenosis and to evaluate the safety and efficacy of stenting of unilateral renal artery stenosis after 6 months and 1 year according to office BP and ABPM.Materials and methods. The study included 44 patients with resistant arterial hypertension and renal artery stenosis (mean age 69 ± 11 years), while receiving multicomponent antihypertensive therapy, including diuretic. After assessing the degree of RA stenosis according to selective angiography and additional methods of assessing the functional significance of RA stenosis (iFR, Pd/Pa, translesional gradient and FFR) the patients underwent RA stenting. The safety of the procedure was assessed by creatinine and glomerular filtration rate dynamics (MDRD equation). The effectiveness of the procedure was study according to office blood pressure (BP) and ambulatory BP monitoring (ABPM).Results. According to the results of angiography, the patients were divided into two groups: group A – patients with subtotal PA stenosis (> 90%) and group B – patients with moderate stenosis (60-90%), who underwent additional assessment of the functional significance of stenosis. The immediate angiographic result in both groups was optimal (the degree of residual stenosis was less than 5%). In group B, after stent implantation in the PA, there was a decrease of the translesional gradient by 38 ± 10 mm Hg (p < 0,0001), FFR > 0,8(p < 0.0001). iFR > 0.89, Pd/Pa>0.9 (p = 0,093). In both groups, there was a significantly significant reduction in arterial blood pressure at distant times, according to clinical BP measurement and ABPM.Conclusion. The measurement of such parameters as translesional gradient and fractional reserve is the most reliable in assessing the hemodynamic significance of PA stenosis in comparison with iFR and Pd/Pa measurements in patients with moderate PA stenosis. PA stenting in patients with uncontrolled resistant AH and severe renal artery lesions, as well as moderate stenosis confirmed by additional methods of stenosis functional significance assessment is reasonable to consider in order to enhance the antihypertensive effect.

Aim. To study the frequency of clinical and radiological signs of reperfusion pulmonary edema and compare them with the volume of endovascular intervention after balloon pulmonary angioplasty (BPA) in patients with chronic thromboembolic pulmonary hypertension (CTEPH).Materials and methods. The study included 50 patients with CTEPH, who underwent stage 1 PLA. To prevent severe reperfusion edema, the number of segmental arteries planned for angioplasty was taken into account, the Pulmonary Edema Predictive Scoring Index (PEPSI) was determined, and after the intervention, noninvasive ventilation was performed in the Continuous positive airway pressure (СPAP) mode for 24 hours. Clinical manifestations of reperfusion edema were assessed 1, 24, 48 and 72 hours after BPA. Radiological signs of edema were considered based on the results of multislice computed tomography (MSCT) or chest x-ray.Results. Angioplasty was performed on 97 segmental, 6 lobar pulmonary arteries, incl. by 45 (46,4%) – with occlusive lesions. The number of arteries undergoing angioplasty ranged from 1 to 7 in each patient, on average 1,9 ± 1,4, change in pulmonary flow grade (ΔPFG) – 4,3 ± 3,4, PEPSI 58,4 ± 51,0, which exceeded the recommended value of 35,5. During the 72-hour observation period, 28 (56%) patients had grade 1 edema, grade 2 reperfusion edema was observed in 15 (30%), grade 3 – in 5 (10%), grade 4 – in 2 (4%) patients. Patients with grade 2-4 edema had higher baseline mPAP (p = 0,015) and PEPSI (p = 0,046). All manifestations of reperfusion edema of 2-4 degrees were stopped due to the prolonged regimen of CPAP therapy for 3 ± 2 days. None of the patients reached grade 5 edema, and there were no deaths. Clinical manifestations of reperfusion injury were observed in 25 (50%) patients, their highest frequency was observed after 24 hoursConclusion. Balloon pulmonary angioplasty of the pulmonary arteries is a safe method for the treatment of inoperable patients with chronic thromboembolic pulmonary hypertension, provided that recommendations for the prevention of reperfusion pulmonary edema are observed. CPAP therapy can successfully prevent and stop the development of severe reperfusion injuries after BPA even when the risk index for reperfusion pulmonary edema is exceeded.

Our observation demonstrates a case of a 40-year-old female with idiopathic pulmonary arterial hypertension World Health Organization functional class III, who was admitted to NMRC of Cardiology repeatedly due to disease progression including dyspnea worsening and exercise tolerance decrease after previous COVID-19 infection on riociguat (7.5 mg daily), macitentan (10 mg daily) and selexipag (1600 mcg daily) therapy. Clinical examination demonstrated high-risk status according to the expected 1-year mortality. Due to unreleased treatment goals and high-risk status, we performed transition from selexipag to inhale iloprost. After therapy escalation the patient demonstrated a significant improvement in clinical condition, dyspnea reduction and exercise tolerance increase. The current treatment strategy for pulmonary arterial hypertension is based on regular multiparametric risk stratification approach in PAH patients. The impact of COVID-19 may become an important cause of clinical worsening in PAH patients during COVID-19 pandemic. Directed on vasodilatation and antiproliferation mechanisms of action of PAH-specific drugs are supposed to be protective in COVID-19 patients. However, the probability of clinical worsening in PAH patients despite PAH-specific therapy intake needs to be closely monitored to perform timely treatment correction in order to achieve low-risk status and to improve the prognosis of PAH patients.

A method is proposed for noninvasive assessment of the pulmonary artery pressure based on separation of the aortic and pulmonary components of the second heart sound S2 and determining the delay time between them. The algorithm for separating the aortic and pulmonary components is based on the Wigner–Ville transform. Analysis of the phonocardiograms of 96 patients with pulmonary hypertension (PH) recorded by doctors of the Scientific Research Center of Cardiology using an original set of equipment was carried out. In 53 patients with PH, it was possible to separate S2 signals into components and determine the delays between them. We construct the dependence of delays between the components on pulmonary artery pressure (PAP), measured by right heart catheterization (RHC). For seven patients with PH, the obtained dependences were used to predict the PAP value from the measured time delay. For five patients, the determination coefficient for the predicted PAP values was 0.83, which indicates a fairly good correlation with measurements by the RHC method.

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.Артериит Такаясу (АТ) заболевание из группы системных васкулитов с преимущественным поражением аорты и ее крупных ветвей. В ряде случаев поражаются легочные артерии (ЛА), что приводит к формированию легочной гипертензии и ухудшает прогноз. Своевременная диагностика поражений ЛА с развитием легочной гипертензии у больных АТ, назначение адекватной патогенетической терапии и хирургическое лечение способны предотвратить необратимое повреждение сосудов, улучшить прогноз. Периоперационное назначение ингибитора интерлейкина-6 (тоцилизумаб) у больных АТ, имеющих показания к оперативному вмешательству на сосудах, следует рассматривать как перспективный подход, позволяющий контролировать воспалительную активность АТ, редуцировать дозу глюкокортикоидов и снизить риск постоперационных осложнений. Представлен клинический опыт успешного лечения больной АТ с преимущественным поражением ЛА с проведением двухэтапной баллонной ангиопластики ЛА на фоне контроля активности заболевания с использованием ингибитора интерлейкина-6 тоцилизумаба и применения специфической терапии риоцигуатом и илопростом.

Objective. To study the efficacy and safety of radiofrequency renal denervation with mono-electrode and multi-electrode devices in patients with uncontrolled arterial hypertension during follow-up period. Materials and methods. The study included 42 patients with uncontrolled arterial hypertension (mean age 51±12 years), while receiving multicomponent antihypertensive therapy, including diuretic. All patients underwent radiofrequency denervation of the renal arteries with a mono-electrode (n=27; group A) and multi-electrode devices (n=15; group B). The safety of the procedure was assessed using creatinine and glomerular filtration rate (MDRD equation), as well as according to ultrasound of the kidneys and renal arteries. The effectiveness of the procedure was study according to office blood pressure (BP) and ambulatory BP monitoring (ABPM). Results. In the general group, according to office BP after 6 months, there decreased in systolic (SBP)/diastolic BP (DBP) by 28/13 mm Hg (p=0.000001). According to ABPM, there was a decrease in the average daily SBP by 9 mm Hg (p=0.007) and DBP by 6 mm Hg (p=0.03). No significant changes in creatinine and glomerular filtration rate were detected in the general group. According to ABPM, after 6 months in group B, there was a decrease in the average daily SBP/DBP by 13 and 6 mm Hg (p=0.1). In group A, according to the ABPM, after 6 months, there was a decrease in the average daily SBP and DBP by 7 mm Hg (p=0.001) and 4 mm Hg (p=0.03). After 1 year, according to the office BP, there was a decrease in SBP/DBP by 14/11 mm Hg (p=0.002), and after 3 years at 15/17 mm Hg (p=0.3). Conclusion. The results confirm the safety and efficacy of radiofrequency renal denervation. Renal denervation in combination with drug therapy leads to decreasing of BP after 6 months and in the long-term

Russian Society of Cardiology (RSC)With the participation: Association of Cardiovascular Surgeons of Russia, Russian Respiratory Society, Federation of Anesthesiologists and Resuscitators, Association of Rheumatologists of Russia, National Congress of Radiation Diagnosticians.

Clinical and hemodynamic profile and neurohormonal indicators in patients with idiopathic pulmonary hypertension, depending on vasoreactivity reserve of pulmonary arteries. Objective: the main objective of our research work was an evaluation of clinical and hemodynamic profile, neurohormonal indicators in patients with IPH, depending on the results of vasoreactivity testing. Methods: the study included 131 patients with IPH (mean age 32,4±3,2 years. All the patients underwent the routine laboratory tests, 6-minuts walking test (6-MWT), transthoracic echocardiography, thorax organs radiography, right heart catheterization (RHC) with acute pharmacological test (APT) using one or two vasodilators (VD) In order to study neurohumoral status the analysis of the 6-keto-PgF1β, NO, ET-1, norepinephrine, epinephrine levels, NT-proANP, NT-proBNP (NUP), renin activity, AII, aldosterone (RAAS) activity was conducted. Results: significant differences of clinical and hemodynamic profile and neurohormonal indicators in patients with IPH depending on the availability (APT +) and absence (APT-) of the vasoreactivity reserve of pulmonary arteries (PA) were established. Among the APT- patients the number of patients belonging to FC III was significantly greater in comparison with APT+ patients. FC IV refered only to the APT- patients. Significantly more patients with APT+ belonged to FC I and II. The 6-MWT revealed that patients with IPH with intact vasodilation reserve overcame significantly longer distance. Doppler echocardiography showed that in APT-group a greater level of systolic pressure in the pulmonary artery (SPPA). When comparing the RHDC data all the hemodynamic parameters in the groups varied considerably, prognostically better data marked APT+ group. According to thorax organs radiography APT- group had significantly higher leel of KTI. Comparative evaluation of neurohumoral status, regardless of the outcome of the APT, showed a significant difference in the groups. Conclusion: the group of responders in comparison with the APT- patients group indicated more safe clinical-hemodynamic and functional status, and neurohumoral rates in the responders group were closer to the outcome in the control group.

Idiopathic pulmonary arterial hypertension (IPAH) is a rare cardiovascular disease of unknown etiology, characterized by pulmonary vascular resistance (PVR) and pulmonary artery pressure (PAP) increase due to pronounced remodeling of small pulmonary arteries and arterioles, with progressive deterioration and the rapid development of right ventricle failure. The key pathogenetic feature is the endothelial dysfunction, which leads to the development of vasoconstriction, thrombosis in situ, proliferative and inflammatory changes in the lung microvasculature. Optimization of drug therapy is associated with the clinical implementation of highly effective pathogenetic drugs which act on the main disease targets - activation of endothelin-1 (ET-1) system, the lack of endogenous prostacyclin and nitric oxide. The role of ET-1 as a powerful vasoconstrictor that induces cell proliferation and differentiation, production of cytokines, growth factors, biologically active substances. Endothelin receptor antagonists (ERA) represents the important class of PAH-specific therapy, including two non-selective ERAs-nonselective bosentan and selective ambrizentan. Presented clinical case demonstrates the long-term reliable efficacy and safety of ambrizentan for 10 years in the IPAH patient with the baseline functional class III (WHO).

The patient with the verified diagnosis of idiopathic pulmonary hypertension, negative acute vasoreactivity testing by RHC, functional class III (WHO) was treated by phosphodiesterase type 5 inhibitor sildenafil. After one year there was achieved the significant improvement of the functional and hemodynamic status.

Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare disease which occurs in 1-3% of patients [2, 11] after pulmonary embolism. The incidence of CTEPH is 5-10 cases per 1 million population per year. Similar to other diseases associated with pulmonary hypertension (PH), CTEPH is malignant and has a poor prognosis. [4, 6, 8] There are a number of objective difficulties in the diagnosis of the disease: complaints and symptoms are very non-specific, there are often no X-ray aor ECG changes, echocardiographic signs of overload of right heart chambers are observed in the final stages of the disease. [9, 3] A strong inclination of doctors to primarily seek problems in the left heart chambers also plays a part in this. As a result, a patient may be visiting various specialists for years and receive treatment from different ailments including heart failure, asthma, pulmonary fibrosis, obstructive bronchitis. We had a case where a female patient with CTEPH received prednisolone for asthma for a long time while she had normal spirometry!

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).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.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.In patients with CTEPH subtraction CTPA allows carrying out complex diagnostics of the state of vascular bed, parenchyma and perfusion of the lungs.

The patient with the verified diagnosis of idiopathic pulmonary hypertension, functional class III (WHO) was treated by anticoagulants, diuretics, calcium channel blocker. After one year of adding endothelin receptor antagonist bosentan there was noticed the negative dynamic with right heart chambers dilation, appearing of heart failure signs. By 11 months of the combined pathogenetic therapy with bosentan and phosphodiesterase type 5 inhibitor sildenafil there was achieved the significant improvement of the functional and hemodynamic status. This positive dynamics remained within the next 3 years of observation.

The currentguidelines on chronic thromboembolic pulmonary hypertension (CTEPH) treatment consider pulmonary thrombendarterectomy as the first therapy choice. In case of inoperability due to severe patient's condition, distal lesions of pulmonary vessels, extremely high pulmonary vascular resistance, medical treatment for CTEPH includes life-long anticoagulation therapy and reduction of heart failure symptoms. This clinical case of the 59-y-old patient M. with inoperable CTEPH demonstrates the efficacy of phosphodiеsterase-5 inhibitor Sildenafil usedin high doses. The diagnosis was confirmed by the results of the complete diagnostic process including right heart catheterization and pulmonary angiography. The initial functional class was assessed as III in accordance with WHO classification. Sildenafil 60 mg daily was added tothe standard therapy (anticoagulant, calcium channel blocker) for initial four weeks. To the 4wk visit Sildenafil dose was increased to 240 mgdailytaking into account good tolerance of the treatment.The significant improvement of functional class, positive dynamic ofhemodynamic and echo parameters were found to 4 month of follow-up.

To assess the clinical outcome of one year follow-up in patients with diffuse long lesions treated with extra-long sirolimus-eluting stents.85 patients underwent implantation of very long BioMime stents during the period from March 2014 to February 2016.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.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.

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.

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.

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.

To ascertain long-term prognosis after endovascular treatment of ischemic heart disease (IHD) in patients with arterial hypertension (AH).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.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%, p0.0001; 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.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.

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.