transplantologiyaТрансплантологияTransplantologiya. The Russian Journal of Transplantation2074-05062542-0909IPO Association of Transplantologists10.23873/2074-0506-2017-9-1-23-34transplantologiya-151Research ArticleКЛИНИЧЕСКИЕ НАБЛЮДЕНИЯCASE REPORTSТрансплантация печени при гепатопульмональном синдроме: обзор литературы и клинический случайHepatopulmonary syndrome and liver transplantation: the review and a case reportМалиновскаяЮ. О.MalinovskayaYu. O.

научный сотрудник отделения абдоминальной хирургии  

Москва, Россия

Research Fellow of the Abdominal Surgery Unit

Moscow, Russia

ruslivertx@gmail.comДжинерД.DzhinerD.

Москва, Россия

Moscow, Russia

ОстроумовЕ. Н.OstroumovE. N.

Москва, Россия

Moscow, Russia

МойсюкЯ. Г.MoysyukYa. G.

Москва, Россия

Moscow, Russia

ГБУЗ МО МОНИКИ им. М.Ф. ВладимирскогоРоссияMoscow Regional Research and Clinical Institute n.a. M.F. VladimirskiyRussian FederationФГБОУ ВО Первый МГМУ им. И.М. Сеченова МЗ РФРоссияI.M. Sechenov First Moscow State Medical UniversityRussian FederationГБУЗ «НИИ скорой помощи им. Н.В. Склифосовского ДЗМ»РоссияN.V. Sklifosovsky Research Institute for Emergency MedicineRussian Federation201720032017912334Copyright © Малиновская Ю.О., Джинер Д., Остроумов Е.Н., Мойсюк Я.Г., 20172017Малиновская Ю.О., Джинер Д., Остроумов Е.Н., Мойсюк Я.Г.Malinovskaya Y.O., Dzhiner D., Ostroumov E.N., Moysyuk Y.G.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.jtransplantologiya.ru/jour/article/view/151

Гепатопульмональный синдром – тяжелое осложнение заболевания печени, характеризующееся триадой симптомов: болезнь печени, легочная вазодилатация и артериальная гипоксемия (РаО2 менее 70 мм рт.ст. в покое). Прекапиллярная вазодилатация, ангиогенез в легких и гипердинамический тип кровообращения приводят к артериовенозному шунтированию в легких и ограничению диффузии газов. При выявлении макроскопических артериовенозных шунтов возможна эмболизация ветвей легочной артерии, однако единственным радикальным методом лечения гепатопульмонального синдрома на данный момент является трансплантация печени. Пятилетняя выживаемость пациентов, страдающих гепатопульмональным синдромом после трансплантации, составляет около 70%. Уровень периоперационной летальности сохраняется высоким (8,5–29%), особенно у пациентов с PaO2 менее 50 мм рт.ст. В послеоперационном периоде зачастую требуется пролонгирование искусственной вентиляции легких, возможно применение неинвазивной вентиляции, а в тяжелых случаях показана экстракорпоральная мембранная оксигенация. В данной статье приводятся обзор исследований, посвященных этой проблеме, и описание клинического случая успешной трансплантации печени пациенту, страдающему циррозом печени и гепатопульмональным синдромом с тяжелой гипоксемией (PaO2 менее 50 мм рт.ст.).

Hepatopulmonary syndrome is a liver disease complication characterized by the clinical triad of an advanced chronic liver disease, a pulmonary vascular dilatation, and a reduced arterial oxygenation (hypoxemia: PaO2 less than 70 mm Hg at rest) in the absence of intrinsic cardiopulmonary disease. Precapillary vasodilatation, intrapulmonary angiogenesis and hyperdynamic circulation lead to arteriovenous shunting and reduced gas diffusion. In case of detecting macroscopic shunts, the pulmonary artery branch embolization could be performed. However, the hepatopulmonary syndrome can be cured nowadays by means of liver transplantation only. A 5-year survival in these patients is about 70%. The observed mortality is the highest during the surgery or early after, especially in patients with PaO2 lower than 50 mm Hg. It ranges from 8.5 to 29%. A prolonged oxygenation support is often required after liver transplantation using invasive or non-invasive ventilation, or even extracorporeal membrane oxygenation. In this review, we have focused on the recent advances in this field as described in available literature and have presented a case report of successful liver transplantation in a patient with a severe hepatopulmonary syndrome.

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