[Clinical guidelines for the diagnosis and treatment of chronic thromboembolic pulmonary hypertension (Part 1)].

Abstract:

:Chronic thromboembolic pulmonary hypertension (CTEPH) is precapillary pulmonary hypertension, in which chronic obstruction of large and middle branches of pulmonary arteries (PAs) and secondary changes in the lung microcirculatory bed result in a progressive increase in pulmonary vascular resistance and PA pressure with the development of severe right cardiac dysfunction and heart failure. CTEPH is a unique form of pulmonary hypertension since it is potentially curable by surgical treatment. The diagnostic criteria for CTEPH are a mean PA pressure of ≥25 mm Hg, as evidenced by right heart catheterization; a PA wedge pressure of ≤15 mm Hg; a pulmonary vascular resistance of >2 Wood units; the presence of chronic/organized thrombi/emboli in the elastic PAs (pulmonary trunk, lobular, segmental, subsegmental PAs); effective anticoagulant therapy at therapeutic dosages over at least 3 months. Up to now, our country has had no guidelines for the diagnosis and treatment of this rare severe disease that, when appropriately untreated, has an extremely poor prognosis. The main task in the preparation of this document was to generalize and analyze the data of current registries, multicenter randomized clinical trials, national and international guidelines, and consensus documents recently published on this problem in order to optimize a diagnostic process and treatment in this category of patients. Part 1 gives a definition of CTEPH, its place in the clinical classification, epidemiology and prognosis, risk factors, pathogenesis and morphology, diagnostic approaches and determination of operability in patients, and specific features of differential diagnosis. :Хроническая тромбоэмболическая легочная гипертензия (ХТЭЛГ) - прекапиллярная форма легочной гипертензии, при которой хроническая обструкция крупных и средних ветвей легочных артерий (ЛА), а также вторичные изменения микроциркуляторного русла легких приводят к прогрессирующему повышению легочного сосудистого сопротивления и давления в ЛА с развитием тяжелой дисфункции правых отделов сердца и сердечной недостаточности. ХТЭЛГ является уникальной формой легочной гипертензии, поскольку она потенциально излечима с помощью хирургического лечения. Диагностическими критериями ХТЭЛГ служат среднее давление в ЛА ≥25 мм рт.ст. по данным катетеризации правых отделов сердца; давление заклинивания в ЛА ≤15 мм рт.ст.; легочное сосудистое сопротивление >2 ед. Вуда; наличие хронических/организованных тромбов/эмболов в ЛА эластического типа (легочный ствол, долевые, сегментарные, субсегментарные легочные артерии); эффективная антикоагулянтная терапия на протяжении не менее 3 мес в лечебных дозировках. До настоящего времени в нашей стране отсутствовали рекомендации по диагностике и лечению этого редкого тяжелого заболевания, которое без необходимого лечения имеет крайне неблагоприятный прогноз. Основной задачей при подготовке данного документа явились обобщение и анализ данных современных регистров, многоцентровых рандомизированных клинических исследований, национальных и международных руководств и согласительных документов, опубликованных за последние годы по этой проблеме с целью оптимизации диагностического процесса и лечения данной категории больных. В I части представлены определение ХТЭЛГ, место в клинической классификации, эпидемиология и прогноз, факторы риска, патогенез и морфология, подходы к диагностике и определение операбельности больных, особенности дифференциального диагноза. :Аннотация Хроническая тромбоэмболическая легочная гипертензия (ХТЭЛГ) — прекапиллярная форма легочной гипертензии, при которой хроническая обструкция крупных и средних ветвей легочных артерий (ЛА), а также вторичные изменения микроциркуляторного русла легких приводят к прогрессирующему повышению легочного сосудистого сопротивления и давления в ЛА с развитием тяжелой дисфункции правых отделов сердца и сердечной недостаточности. ХТЭЛГ является уникальной формой легочной гипертензии, поскольку она потенциально излечима с помощью хирургического лечения. Диагностическими критериями ХТЭЛГ служат среднее давление в ЛА ≥25 мм рт.ст. по данным катетеризации правых отделов сердца; давление заклинивания в ЛА ≤15 мм рт.ст.; легочное сосудистое сопротивление >2 ед. Вуда; наличие хронических/организованных тромбов/эмболов в ЛА эластического типа (легочный ствол, долевые, сегментарные, субсегментарные легочные артерии); эффективная антикоагулянтная терапия на протяжении не менее 3 мес в лечебных дозировках. До настоящего времени в нашей стране отсутствовали рекомендации по диагностике и лечению этого редкого тяжелого заболевания, которое без необходимого лечения имеет крайне неблагоприятный прогноз. Основной задачей при подготовке данного документа явились обобщение и анализ данных современных регистров, многоцентровых рандомизированных клинических исследований, национальных и международных руководств и согласительных документов, опубликованных за последние годы по этой проблеме с целью оптимизации диагностического процесса и лечения данной категории больных. В I части представлены определение ХТЭЛГ, место в клинической классификации, эпидемиология и прогноз, факторы риска, патогенез и морфология, подходы к диагностике и определение операбельности больных, особенности дифференциального диагноза.

journal_name

Ter Arkh

journal_title

Terapevticheskii arkhiv

authors

Chazova IE,Martynyuk TV

doi

10.17116/terarkh201688990-101

subject

Has Abstract

pub_date

2016-01-01 00:00:00

pages

90-101

issue

9

eissn

0040-3660

issn

2309-5342

journal_volume

88

pub_type

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