evrazkarЕвразийский Кардиологический ЖурналEurasian heart journal2225-16852305-0748Евразийская ассоциация кардиологов10.38109/2225-1685-2024-2-122-131evrazkar-6464Research ArticleОБЗОРREVIEWНеспецифическая терапия синдрома ЭйзенменгераNonspecific therapy for Eisenmenger syndromehttps://orcid.org/0000-0001-8937-1796ШмальцА. А.ShmaltsA. A.

Шмальц Антон Алексеевич, д.м.н., ведущий научный сотрудник; доцент кафедры сердечно-сосудистой хирургии

Рублевское шоссе, д. 135, г. Москва 121552

Ул. Баррикадная, д. 2/1, г. Москва 125993

Anton A. Shmalts, Dr. of Sc. (Med.), Leading Researche; Associate Professor at Department of Cardiovascular Surgery

Rublevskoe highway, 135, Moscow 121552

shmaltzanton@inbox.ruhttps://orcid.org/0000-0002-6619-3875ЧерногривовИ. Е.ChernogrivovI. E.

Черногривов Игорь Евгеньевич, д.м.н., заведующий отделением

Рублевское шоссе, д. 135, г. Москва 121552

Rublevskoe highway, 135, Moscow 121552

ФГБУ «Национальный медицинский исследовательский центр сердечно-сосудистой хирургии имени А.Н. Бакулева» Минздрава России; ФГБОУ «Российская медицинская академия непрерывного профессионального образования» Минздрава РоссииРоссияBakoulev National Medical Research Center for Cardiovascular Surgery; Russian State Medical Postgraduate AcademyRussian FederationФГБУ «Национальный медицинский исследовательский центр сердечно-сосудистой хирургии имени А.Н. Бакулева» Минздрава РоссииРоссияBakoulev National Medical Research Center for Cardiovascular SurgeryRussian Federation20242805202402122131Copyright © Шмальц А.А., Черногривов И.Е., 20242024Шмальц А.А., Черногривов И.Е.Shmalts A.A., Chernogrivov I.E.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.heartj.asia/jour/article/view/6464

Синдром Эйзенменгера (СЭ) – легочная гипертензия системного уровня с веноартериальным или двунаправленным сбросом крови между желудочками, предсердиями или магистральными артериями вследствие прогрессирования легочной сосудистой болезни при естественном течении «простых» и «сложных» врожденных пороков сердца. Пациенты с СЭ требуют постоянного внимания многопрофильной команды специалистов. Одна из главных задач – не дестабилизировать хрупкую патологическую физиологию, опирающуюся на баланс легочного и периферического сосудистых сопротивлений. Хирургические вмешательства с анестезией, если их нельзя избежать, целесообразно выполнять специализированных центрах. Беременность пациенткам с СЭ противопоказана. Клинические симптомы гипервязкости крови при СЭ встречаются редко. Анахроничной практики венесекций, приводящих к снижению кислородтранспортной функции крови, дефициту железа и повышению риска цереброваскулярных осложнений, следует избегать. Пероральное и внутривенное восполнение дефицита железа улучшает переносимость физической нагрузки и качество жизни. Рутинное назначение антикоагулянтов при СЭ не показано. Устранить кровохарканье в большинстве случаев позволяет эндоваскулярная эмболизация патологических коллатеральных артерий. Использование венозного доступа требует тщательной профилактики эмболий. Терапевтическая тактика при СЭ детально рассмотрена в настоящем обзоре.

Eisenmenger syndrome (ES) is systemic pulmonary hypertension with venoarterial or bidirectional shunting of blood between the ventricles, atria or great arteries due to the progression of pulmonary vascular disease in the natural course of “simple” and “complex” congenital heart defects.Patients with ES require constant attention from a multidisciplinary team of specialists. One of the main tasks is not to destabilize the fragile pathological physiology, which relies on the balance of pulmonary and peripheral vascular resistance. Surgical interventions with anesthesia, if they cannot be avoided, should be performed in specialized centers. Pregnancy is contraindicated in patients with ES. Clinical symptoms of blood hyperviscosity in ES are rare.The anachronistic practice of venesections, which leads to a decrease in the oxygen transport function of the blood, iron deficiency and an increased risk of cerebrovascular complications, should be avoided. Oral and intravenous replenishment of iron deficiency improves exercise tolerance and quality of life. Routine administration of anticoagulants for ES is not indicated. In most cases, endovascular embolization of pathological collateral arteries can eliminate hemoptysis. The use of venous access requires careful prevention of embolism. Therapeutic tactics for ES are discussed in detail in this review

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