Disclaimer. The ESC Guidelines represent the views of the ESC and were produced after careful consideration of the scientific and medical knowledge, and the evidence available at the time of their publication. The ESC is not responsible in the event of any contradiction, discrepancy, and/or ambiguity between the ESC 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 ESC 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 ESC 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 ESC 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.

Pulmonary arterial hypertension (PAH) remains a severe clinical condition despite the availability over the past 15 years of multiple drugs interfering with the endothelin, nitric oxide and prostacyclin pathways. The recent progress observed in medical therapy of PAH is not, therefore, related to the discovery of new pathways, but to the development of new strategies for combination therapy and on escalation of treatments based on systematic assessment of clinical response. The current treatment strategy is based on the severity of the newly diagnosed PAH patient as assessed by a multiparametric risk stratification approach. Clinical, exercise, right ventricular function and haemodynamic parameters are combined to define a low-, intermediate- or high-risk status according to the expected 1-year mortality. The current treatment algorithm provides the most appropriate initial strategy, including monotherapy, or double or triple combination therapy. Further treatment escalation is required in case low-risk status is not achieved in planned follow-up assessments. Lung transplantation may be required in most advanced cases on maximal medical therapy.

Рабочие группы esC: Сердечно-сосудистая фармакотерапия, Сердечно-сосу-дистая хирургия, Врожденные пороки сердца у взрослых, Легочное крово-обращение и правожелудочковая функция, Клапанная болезнь сердца.Содержание данных рекомендаций, подготовленных Европейским Общест-вом Кардиологов (european society of Cardiology, esC) и Европейским Общест-вом Пульмонологов (european respiratory society, ers), опубликовано исклю-чительно для использования в личных и образовательных целях. Не допуска-ется коммерческое использование содержания рекомендаций. Рекомендации esC не могут быть переведены на другие языки либо воспроизведены, полно-стью или частично, без письменного согласия esC. Для получения данного согласия письменная заявка должна быть направлена в Oxford University Press -организацию, издающую european Heart Journal и официально упол-номоченную esC, рассматривать подобные заявки.Отказ от ответственности. Рекомендации esC/ers отражают взгляды esC/ ers и основаны на тщательном анализе научных данных, доступных во время подготовки данных рекомендаций. Медицинским работникам следует придер-живаться данных рекомендаций в процессе принятия клинических решений. В то же время, рекомендации не могут заменить личную ответственность медицинских работников при принятии клинических решений с учетом инди-видуальных особенностей и предпочтений пациентов и, при необходимости, предпочтений их опекунов и попечителей. Медицинские работники также несут ответственность в отношении дополнительной проверки всех надлежа-щих требований и правил перед назначением лекарственных средств и использованием медицинского оборудования. Ключевые слова: рекомендации, легочная гипертензия, легочная артериаль-ная гипертензия, хроническая тромбоэмболическая легочная гипертензия, врожденные пороки сердца, системные заболевания соединительной ткани, сердечная недостаточность, дыхательная недостаточность, антагонисты рецепторов эндотелина, ингибиторы фосфодиэстеразы 5 типа, аналоги про-стациклина, заболевания легких, заболевания левых камер сердца.

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Guidelines for the diagnosis and treatment of circulatory diseases in the context of the COVID-19 pandemic

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Objective and design The aim of this double-blind, placebo-controlled, phase III CORONA clinical trial was to evaluate the efficacy and safety of IL-6 receptor inhibitor levilimab (LVL) in subjects with severe COVID-19. Subjects The study included 217 patients. The eligible were men and non-pregnant women aged 18 years or older, hospitalized for severe COVID-19 pneumonia. Treatment 206 subjects were randomized (1:1) to receive single subcutaneous administration of LVL 324 mg or placebo, both in combination with standard of care (SOC). 204 patients received allocated therapy. After the LVL/placebo administration in case of deterioration of symptoms, the investigator could perform a single open-label LVL 324 mg administration as the rescue therapy. Methods The primary efficacy endpoint was the proportion of patients with sustained clinical improvement on the 7-category ordinal scale on Day 14. All efficacy data obtained after rescue therapy administration were considered missing. For primary efficacy analysis, all subjects with missing data were considered non-responders. Results 63.1% and 42.7% of patients in the LVL and in the placebo groups, respectively, achieved sustained clinical improvement on Day 14 (P = .0017). The frequency of adverse drug reactions was comparable between the groups. Conclusion In patients with radiologically confirmed SARS-CoV-2 pneumonia, requiring or not oxygen therapy (but not ventilation) with no signs of other active infection administration of LVL + SOC results in an increase of sustained clinical improvement rate. Trail registration The trial is registered at the US National Institutes of Health (ClinicalTrials.gov; NCT04397562).