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Журнал "Медицина невідкладних станів" Том 20, №3, 2024

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Портопульмональна гіпертензія: особливості діагностики та лікування

Автори: Чуклін С.М. (1), Чуклін С.С. (1), Посівнич М.М. (2), Кристопчук С.А. (2)
(1) - Медичний центр Святої Параскеви, м. Львів, Україна
(2) - Львівський національний медичний університет імені Данила Галицького, м. Львів, Україна

Рубрики: Медицина невідкладних станів

Розділи: Довідник фахівця

Версія для друку


Резюме

Портопульмональна гіпертензія визначається як розвиток легеневої артеріальної гіпертензії на тлі портальної. Точну її поширеність важко визначити через відсутність рутинного скринінгу у пацієнтів з портальною гіпертензією. Гемодинамічні зміни, пов’язані з портальною гіпертензією, включно з гіпердинамічним станом, портосистемними шунтами та спланхнічною вазодилатацією, викликають значні порушення в легеневому судинному руслі та відіграють ключову роль у патогенезі захворювання. Без лікування портопульмональна гіпертензія призводить до прогресуючої правосерцевої недостатності з поганим прогнозом. Незважаючи на те, що допплерівська ехокардіографія є найкращим інструментом початкового скринінгу для симптомних пацієнтів і кандидатів на трансплантацію печінки, катетеризація правих відділів серця залишається золотим стандартом для діагностики захворювання. Лікування пацієнтів з портопульмональною гіпертензією спрямоване на поліпшення серцевої функції, зменшення легеневого судинного опору, оптимізацію функціональної здатності. ЛАГ-специфічна терапія, яка включає агоністи простацикліну і його рецепторів, антагоністи рецепторів ендотеліну, інгібітори фосфодіестерази та стимулятори гуанілатциклази, відіграє ключову роль у лікуванні пацієнтів з портопульмональною гіпертензією. Невеликі неконтрольовані та нещодавні поодинокі рандомізовані контрольовані дослідження повідомили про багатообіцяючі результати вазодилататорної терапії щодо клінічного та гемодинамічного поліпшення хворих, що дозволило певній частині пацієнтів провести трансплантацію печінки. У цьому огляді обговорюються епідеміологія, підхід до діагностики та лікування хворих із портопульмональною гіпертензією. Для пошуку літературних джерел використовували базу даних MedLine на платформі PubMed і бібліотеку Cochrane за ключовими словами: portopulmonary hypertension, portal hypertension, lung arterial hypertension, liver cirrhosis, pulmonary complications.

Portopulmonary hypertension is defined as the development of pulmonary hypertension secondary to portal one. Its exact prevalence is difficult to determine due to the lack of routine screening in patients with portal hypertension. Hemodynamic changes associated with portal hypertension, including the hyperdynamic state, portosystemic shunts, and splanchnic vasodilation, cause significant disturbances in the pulmonary vasculature and play a key role in the pathogenesis of the disease. Without treatment, portopulmonary hypertension leads to progressive right ventricular failure with a poor prognosis. Although Doppler echocardiography is the best initial screening tool for symptomatic patients and candidates for liver transplant, right heart catheterization remains the gold standard for disease diagnosis. Treatment of patients with portopulmonary hypertension is aimed at improving cardiac function, reducing pulmonary vascular resistance, and optimizing functional capacity. Pulmonary hypertension-specific therapy, which includes prostacyclin and its receptor agonists, endothelin receptor antagonists, phosphodiesterase inhibitors, and guanylate cyclase stimulators, plays a key role in the treatment of patients with portopulmonary hypertension. Small uncontrolled and recent single randomized controlled trials have reported promising results of vasodilator therapy in terms of clinical and hemodynamic improvement in patients, allowing certain patients to undergo liver transplantation. This review discusses the epidemiology, approach to diagnosis and treatment of patients with portopulmonary hypertension. We used MEDLINE database on the PubMed platform and the Cochrane library to search for literature sources using the keywords: portopulmonary hypertension, portal hypertension, pulmonary hypertension, liver cirrhosis, pulmonary complications.


Ключові слова

портопульмональна гіпертензія; легенева артеріальна гіпертензія; гемодинаміка; транс­торакальна ехокардіографія; катетеризація серця; лікування; трансплантація печінки

portopulmonary hypertension; pulmonary hypertension; hemodynamics; transthoracic echocardiography; cardiac catheterization; treatment; liver transplantation


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