Доклад ERS по хронической тромбоэмболической легочной гипертензии

Хроническая тромбоэмболическая (ХТЭ) легочная гипертензия (ЛГ) – редкое осложнение острой тромбоэмболии легочной артерии (ТЭЛА) – как симптоматической, так и бессимптомной. При окклюзии проксимальных сегментов легочных артерий фиброзным внутрисосудистым материалом в комбинации со вторичной микроваскулопатией сосудов диаметром < 500 мкм повышаются легочное сосудистое сопротивление и прогрессирующая правожелудочковая недостаточность. Механизм трансформации кровяных сгустков в остаточный фиброзный материал до сих пор не установлен. ХТЭ ЛГ у пациентов с ЛГ подозревается в случае, когда по данным вентиляционно-перфузионной сцинтиграфии легких выявляются несоответствующие картине вентиляции дефекты перфузии, которые подтверждаются при катетеризации правых отделов сердца и визуализации сосудов. В зависимости от локализации и типов повреждений, помимо пожизненного приема антикоагулянтов, на сегодняшний день доступны хирургическое лечение, ангиопластика и медикаментозное лечение. В настоящем докладе представлен обзор литературы и описана современная практика установления диагноза и ведения больных ХТЭ ЛГ. Документ охватывает области определения, диагностики, эпидемиологии, наблюдения после острой ТЭЛА, патофизиологии, лечения с помощью легочной тромбэндартерэктомии, баллонной ангиопластики легочной артерии, лекарственных препаратов и их комбинаций, реабилитации и новых направлений исследований в области ХТЭ ЛГ. Доклад представляет собой результат первого совместного проекта по ЛГ Европейского респираторного общества, Международной ассоциации хронической тромбоэмболической легочной гипертензии и Европейской сети медицинских учреждений по редким заболеваниям легких. В докладе резюмируются текущие знания, но не даются формальные рекомендации для клинической практики.

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