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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.

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.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.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.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.

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.

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.