Журнал «Медицина неотложных состояний» Том 19, №8, 2023

Гепатопульмональний синдром (ГПС) є одним із захворювань легень, пов’язаних із цирозом печінки й портальною гіпертензією. Діагноз ґрунтується на тріаді: захворювання печінки й портальна гіпертензія, ознаки внутрішньолегеневої судинної дилатації і порушення газообміну. ГПС погіршує прогноз (23 % виживання через 5 років) і якість життя пацієнтів, тому велике значення має рання діагностика і своєчасне лікування. Трансплантація печінки дозволяє досягти регресу внутрішньолегеневої судинної дилатації майже в 100 % випадків, нормалізувати газообмін і поліпшити 5-річну виживаність після трансплантації від 76 до 87 %. Це єдиний лікувальний метод, показаний пацієнтам з тяжким ГПС, який визначається артеріальним парціальним тиском кисню (PaO2) нижче за 60 мм рт.ст. Проте в умовах глобального дефіциту трансплантатів необхідно розробити медичну терапію, щоб відстрочити або навіть відкласти трансплантацію печінки. Досягнення цієї мети видається можливим завдяки зростаючому розумінню патофізіології ГПС і розробці терапії, спрямованої на ключові механізми, головним чином запальні й ангіогенні. У цій статті наведено огляд клінічних проявів, діагностики й лікування ГПС на підставі літературних джерел з бази даних MedLine на платформі PubMed.

Hepatopulmonary syndrome (HPS) is one of the lung diseases associated with liver cirrhosis and portal hypertension. The diagnosis is based on the triad: liver disease and portal hypertension, evidence of intrapulmonary vascular dilatation and impaired gas exchange. HPS impairs prognosis (23 % survival after 5 years) and patients’ quality of life, so early diagnosis and timely treatment are of great importance. Liver transplantation allows for regression of intrapulmonary vascular dilatation in almost 100 % of cases, normalization of gas exchange and improves a 5-year survival after transplantation from 76 to 87 %. This is the only treatment method indicated for patients with severe HPS, defined by an arterial partial pressure of oxygen (PaO2) below 60 mm Hg. However, in the face of a global shortage of transplants, it is necessary to develop medical therapies to delay or even defer liver transplantation. This goal seems possible due to the growing understanding of the HPS pathophysiology and the development of therapies targeting key mechanisms, mainly inflammatory and angiogenic. This article provides an overview of the clinical manifestations, diagnosis and treatment of HPS based on literature sources from the MEDLINE database on the PubMed platform.

гепатопульмональний синдром; цироз печінки; портальна гіпертензія; діагностика; трансплантація печінки; огляд

hepatopulmonary syndrome; liver cirrhosis; portal hypertension; diagnosis; liver transplantation; review

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