References

pulmo

Пульмонология

PULMONOLOGIYA

0869-01892541-9617

Scientific and Practical Journal “PULMONOLOGIYA” LLC

10.18093/0869-0189-2017-27-2-216-231

pulmo-846

Research Article

ОБЗОРЫ

REVIEW

Идиопатическая легочная артериальная гипертензия: современный подход к диагностике и лекарственной терапии

Idiopathic pulmonary arterial hypertension: current approach to diagnosis and pharmacological treatment

Царева

Н. А.

Tsareva

N. A.

Царева Наталья Анатольевна – кандидат медицинских наук, заведующая лабораторией интенсивной пульмонологии и дыхательной недостаточности

105077, Москва, ул. 11-я Парковая, 32, корп. 4

Natal'ya A.Tsareva, Candidate of Medicine, Head of Laboratory of Intensive Pulmonology and Respiratory Failure

Odinnadtsataya Parkovaya ul. 32, build. 4, Moscow, 105077

n-tsareva@mail.ru

Федеральное государственное бюджетное учреждение «Научно-исследовательский институт пульмонологии Федерального медико-биологического агентства России»РоссияFederal Pulmonology Research Institute, Federal Medical and Biological Agency of RussiaRussian Federation

2017

05072017

272216231

2017

Царева Н.А.

Tsareva N.A.

Данная работа распространяется под лицензией Creative Commons Attribution 4.0.

This work is licensed under a Creative Commons Attribution 4.0 License.

https://journal.pulmonology.ru/pulm/article/view/846

Рассматриваются современные аспекты диагностики, классификации и лечения легочной гипертензии (ЛГ). Представлена новая классификация, включающая все известные на сегодняшний день формы ЛГ. Приводится функциональная классификация данной патологии. Подробно изложена диагностическая программа, включающая как рутинные методы обследования больных, так и высокочувствительные и инновационные исследования (компьютерно-томографическая ангиография сосудов легких, перфузионная сцинтиграфия легких, катетеризация правых отделов сердца и т. д.) Отражены факторы прогноза 1-годичной выживаемости пациентов с ЛГ. Приводятся характеристики основных звеньев патогенеза. Рассматриваются эндотелиновый и простациклиновый пути патофизиологии ЛГ, а также роль оксида азота и стимуляторов растворимой гуанилатциклазы. Изложены традиционные подходы, используемые при терапии ЛГ, включающие применение антагонистов кальция, кислорода, антикоагулянтов и диуретиков. Приводится характеристика критериев проведения «острого» теста на вазореактивность. Представлены основные классы лекарственных препаратов (антагонисты рецепторов к эндотелину, простациклины, стимуляторы растворимой гуанилатциклазы, ингибиторы фосфодиэстеразы 5-го типа, селективные агонисты рецепторов простациклина). Подробно рассматриваются современные пути патогенетической терапии ЛГ, а также основные направления поиска новых эффективных и безопасных лекарственных средств, способных улучшить прогноз, замедлить прогрессирование заболевания, а также повлиять на качество жизни пациентов.

Current aspects of diagnosis, classification and therapy of pulmonary hypertension (PH) were reviewed in the article. A new classification, including all known clinical variants of PH, and functional classification were presented. A diagnostic algorithm with routine diagnostic methods and highly sensitive innovative techniques (CT-angiography of the lungs, perfusion scintigraphy, the right heart catheterization, etc.) was shown. The author discussed prognostic factors of 1-year survival, general pathogenic mechanisms including endothelin and prostacyclin pathways and a role of nitric oxide and soluble guanylate cyclase stimulators. Assessment criteria of acute vasoreactivity test results were reviewed. Typical therapeutic approaches including calcium channel blockers, oxygen, anticoagulants and diuretics were described. Main classes of specific drugs were listed such as endothelin receptor antagonists, prostacyclins, soluble guanylate cyclase stimulators, phosphodiesterase 5 inhibitors, selective prostacyclin receptor agonists. Current investigations are directed to searching new effective and safe drugs that could improve outcomes, slow down progression of the disease and improve quality of the patient’s life.

идиопатическая легочная артериальная гипертензияантагонисты эндотелиновых рецепторовпростациклиныстимуляторы растворимой гуанилатциклазыингибиторы фосфодиэстеразы 5-го типаселективные агонисты рецепторов простациклина.ajrccm/145.1.101

idiopathic pulmonary arterial hypertensionendothelin receptor antagonistsprostacyclinssoluble guanylate cyclase stimulatorsphosphodiesterase 5 inhibitorsselective prostacyclin receptor agonists

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The authors declare that there are no conflicts of interest present.