cardiovascularКардиоваскулярная терапия и профилактикаCardiovascular Therapy and Prevention1728-88002619-0125«SILICEA-POLIGRAF» LLC10.15829/1728-8800-2024-3753ENJXHRcardiovascular-3753Research ArticleОБЗОРЫ ЛИТЕРАТУРЫREVIEW ARTICLESЛегочная артериальная гипертензия, ассоциированная врожденными портокавальными шунтами — новая форма портопульмональной гипертензии у детей и взрослыхPulmonary hypertension associated with congenital portosystemic shunts as a new form of portopulmonary hypertension in children and adultshttps://orcid.org/0000-0001-8635-6216МиклашевичИ. М.MiklashevichI. M.

Миклашевич Ирина Михайловна — к.м.н., зав. детским кардиологическим отделением, врач детский кардиолог, врач ультразвуковой диагностики, Научно-исследовательский клинический институт педиатрии и детской хирургии им. акад. Ю.Е. Вельтищева.

Москва

Moscow

imiklashevich@pedklin.ruhttps://orcid.org/0000-0001-9836-6841ПотроховаЕ. А.PotrokhovaE. A.

Потрохова Елена Александровна — д.м.н., профессор, зав. педиатрическим отделением, в.н.с. отдела радиационной экопатологии детского возраста, Научно-исследовательский клинический институт педиатрии и детской хирургии им. акад. Ю.Е. Вельтищева.

Москва

Moscow

potrokhova@pedklin.ruhttps://orcid.org/0000-0002-1940-1395МорозовД. А.MorozovD. A.

Морозов Дмитрий Анатольевич — д.м.н., профессор, директор; заведующий кафедрой детской хирургии и урологии-андрологии им. Л.П. Александрова, Научно-исследовательский клинический институт педиатрии и детской хирургии им. акад. Ю.Е. Вельтищева; Главный внештатный детский специалист хирург Минздрава России, врач — детский хирург высшей категории.

Москва

Moscow

director@pedklin.ruФГАОУ ВО "Российский национальный исследовательский медицинский университет им. Н.И. Пирогова" Минздрава РоссииPirogov Russian National Research Medical UniversityФГАОУ ВО "Российский национальный исследовательский медицинский университет им. Н.И. Пирогова" Минздрава России; ФГАОУ ВО "Первый Московский государственный медицинский университет им. И.М. Сеченова" Минздрава России (Сеченовский Университет)Pirogov Russian National Research Medical University; I.M. Sechenov First Moscow State Medical University20242901202423337533753Copyright © Миклашевич И.М., Потрохова Е.А., Морозов Д.А., 20242024Миклашевич И.М., Потрохова Е.А., Морозов Д.А.Miklashevich I.M., Potrokhova E.A., Morozov D.A.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://cardiovascular.elpub.ru/jour/article/view/3753

Легочная артериальная гипертензия (ЛАГ), ассоциированная с портальной гипертензией, или портопульмональная гипертензия, является тяжелым, жизнеугрожающим осложнением портальной гипертензии и/или портокавального шунтирования. Врожденные портокавальные шунты (ВПКШ) — редкая сосудистая аномалия портальной системы, приводящая к каскаду тяжёлых патофизиологических реакций и мультисистемному поражению, включая ЛАГ, узловое поражение печени, когнитивные, метаболические, иммунные, гематологические и дисгормональные нарушения. Тяжелые сердечно-легочные осложнения выявляются более чем у трети больных с ВПКШ, что является основной причиной их гибели. В статье приводится описание патофизиологии, клинических характеристик, особенностей диагностики и возможностей современной таргетной терапии легочной артериальной гипертензии, ассоциированной с врожденными портокавальными шунтами. Пациенты с ЛАГ, ассоциированной с ВПКШ (ЛАГ-ВПКШ) нуждаются в комплексной специализированной помощи экспертного центра. Для долгосрочного успешного ведения пациентов решающее значение имеет постоянная, адекватная степени тяжести заболевания, таргетная терапия ЛАГ в сочетании с хирургическим лечением ВПКШ.

Pulmonary arterial hypertension (PAH) associated with portal hypertension, or portopulmonary hypertension, is a severe, life-threatening complication of portal hypertension and/or portocaval shunt surgery. Congenital portocaval shunts (CPSSs) are rare vascular anomalies of the portal system, leading to severe pathophysiological reactions and multisystem damage, including PAH, liver nodules, cognitive, metabolic, immune, hematological and hormonal disorders. Severe cardiopulmonary complications are detected in more than a third of patients with CPSSs, which is the main cause of their death. The article describes the pathophysiology, clinical characteristics, diagnostic features and possibilities of modern targeted therapy for CPSS-associated PAH. Patients with CPSS-associated PAH require comprehensive specialized care in an expert center. For long-term successful management of patients, continuous targeted therapy for PAH, in combination with surgical treatment of CPSSs, is crucial.

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