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},
"code": "I27.0",
"name": "Первичная легочная гипертензия",
"icd_name": "Первичная легочная гипертензия",
"gender": 0,
"age_min": 40,
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"slug": "i27.0_pervichnaya_legochnaya_gipertenziya",
"lead": "наследственная патология, характеризующаяся повышенным давлением внутри легочной артерии и усиленным общим легочным сосудистым сопротивлением",
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"etiology": "<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Этиология заболевания изучена недостаточно. Предполагается, что ПЛГ относится к наследственным патологиям. У пациентов обнаруживаются мутации генов, определяющих производство сосудорасширяющих соединений – NO-синтазы, карбимил-фосфат синтазы, транспортеров серотонина, а также активность рецептора типа второго костного морфогенетического белка. Механизм генетической передачи аутосомно-доминантный, сопровождается неполной пенетрацией. Это означает, что дебют болезни возможен при наличии одного или двух дефектных генов в аллели, но иногда заболевание остается непроявленным. Передача мутации происходит с тенденцией к манифестации патологии в более раннем возрасте и с более тяжелым течением в следующих поколениях. Носительство мутационного гена или генов приводит к развитию симптоматики не всегда, а только при воздействии пусковых факторов, к которым относятся:</span></p>\r\n<ul>\r\n<li><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Медикаментозные, наркотические интоксикации. ПЛГ потенцируется приемом амфетаминов, L-триптофана, аминорекса, фенфлурамина. Предполагается негативное влияние мета-амфетаминов, кокаина, препаратов химиотерапии.</span></li>\r\n<li><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Физиологические состояния. Спровоцировать начало болезни могут роды и гестация. В группе высокого риска находятся беременные женщины с заболеваниями сердечно-сосудистой системы, осложнениями в родах.</span></li>\r\n<li><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Сопутствующие заболевания. Установлено, что к развитию ПЛГ предрасполагает системная или портальная гипертензия, ВИЧ-инфекция, болезни печени, врожденные шунты между легочными и системными сосудами. Вероятно, пусковыми механизмами могут стать тиреопатии, гематологические заболевания, генетические метаболические патологии.</span></li>\r\n</ul>\r\n<p> </p>\r\n<p><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\"> </span></p>",
"pathogenesis": "<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Развитие ПЛГ происходит в несколько этапов. Вначале нарастает дисфункция или появляются повреждения сосудистого эндотелия, возникает дисбаланс производства и секреции вазодилатирующих и вазоконстрикторных соединений. Повышается продукция тромбоксана и эндотелина-1 – пептида с митогенным действием на гладкомышечные клетки. Образуется недостаток вазодилатирующих соединений, основными из которых являются окись азота и простациклин. Сосуды легких сужаются.</span></p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"> </p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">На втором этапе формируются необратимые изменения структуры сосудов легких. Из эндотелиальных клеток высвобождаются хемокины, которые вызывают перемещение клеток гладких мышц во внутреннюю оболочку легочных артериол. Запускается процесс патологического разрастания стенок сосудов внутрь и усиления производства медиаторов с выраженным сосудосуживающим действием, развивается тромбоз. Углубляется поражение эндотелиальных тканей, прогрессирует сосудистая обструкция. Патологические процессы распространяются на все слои сосудистых стенок и разные типы клеток. Увеличивается сопротивление току крови, повышается давление.</span></p>\r\n<p><span id=\"docs-internal-guid-aa895f25-7fff-20dd-4e89-9d818fb2992d\"> </span></p>",
"diagnostics": "<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Постановка диагноза первичной легочной гипертензии затруднена, поскольку симптомы неспецифичны, а распространенность болезни незначительна. Обследование проводит врач-кардиолог, терапевт. Пациенты жалуются на слабость, одышку, загрудинные боли. При сборе анамнеза нередко определяется наличие провоцирующего фактора (вирусной инфекции, беременности, родов) и семейной отягощенности. С целью уточнения диагноза, исключения сердечных пороков, рецидивирующей ТЭЛА, ХОБЛ и заболеваний миокарда осуществляется:</span></p>\r\n<ul>\r\n<li><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Физикальное исследование. В ходе осмотра обнаруживается акроцианоз (синюшность кожи). Правожелудочковая сердечная недостаточность проявляется набуханием вен шеи, отеками конечностей, накапливанием жидкости в органах брюшины. Аускультация сердца выявляет акцент второго тона над легочной артерией, пансистолический шум, шум Стилла.</span></li>\r\n<li><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Лабораторные анализы. Назначается клиническое и биохимическое исследование крови, тест на тиреоидные гормоны, антикардиолипиновые антитела, D-димер, антитромбин III и протеин C. Результаты позволяют дифференцировать сердечно-сосудистые заболевания, например, исключить тромбофилию. При ПЛГ возможно определение низкого титра антикардиолипиновых антител.</span></li>\r\n<li><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Инструментальная диагностика. По результатам ЭКГ ЭОС отклоняется вправо, правый сердечный желудочек и предсердие гипертрофированы, дилатированы. При фонокардиографии диагностируется гипертензия, гиперволемия легочного круга кровообращения. При рентгенографии ОГК левая ветвь и ствол легочной артерии выбухают, корни легких расширены, правые структуры сердца увеличены. Путем катетеризации сердца измеряется давление крови внутри артерии легких, оценивается минутный объем крови, общее легочное сосудистое сопротивление. </span></li>\r\n</ul>\r\n<p> </p>\r\n<p><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\"> </span></p>",
"treatment": "<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Терапия нацелена на нормализацию давления крови в артериях и легочном стволе, замедление прогрессирования болезни, предупреждение и устранение осложнений. Пациенты госпитализируются при первичном появлении симптомов, выявлении признаков ускоренного развития болезни, формировании декомпенсации по системному кругу и тромбоэмболии легочного ствола. Лечение проводится в трех направлениях:</span></p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"> </p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">1. Медикаментозная терапия. Лекарственные средства позволяют быстро улучшить состояние больных, полностью или частично достичь компенсации нарушенных сердечно-сосудистых функций. Используются препараты следующих групп:</span></p>\r\n<ul>\r\n<li><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Антикоагулянты, дезагреганты. Назначаются оральные антикоагулянты – варфарин, гепарин, низкомолекулярные гепарины. Среди дезагрегантов наиболее распространено применение ацетилсалициловой кислоты. Регулярный прием лекарств снижает риск тромбоэмболии.</span></li>\r\n<li><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Диуретики. Мочегонные средства (петлевые диуретики) уменьшают объем циркулирующей крови, снижают системное давление. В период терапии тщательно контролируется уровень электролитов в крови, функциональность почек.</span></li>\r\n<li><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Сердечные гликозиды, кардиотоники. Предупреждают развитие и прогрессирование недостаточности функций сердца, корректируют мерцательную аритмию. Часто препараты вводятся внутривенно.</span></li>\r\n<li><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Блокаторы кальциевых каналов. Вызывают релаксацию гладких мышечных волокон в стенках сосудов, увеличивая их просвет. Лечение целесообразно для лиц с положительной реакцией на вазодилататоры, имеющих один или несколько из следующих показателей: сердечно-сосудистый индекс более 2,1 л/мин/м2, насыщение гемоглобином крови вен больше 63%, давление в правом предсердии менее 10 мм. рт. ст.</span></li>\r\n<li><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Простагландины. Оказывают вазодилатирующее, антиагрегационное и антипролиферативное действие. Распространено использование простагландина E1.</span></li>\r\n<li><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Антагонисты эндотелиновых рецепторов. По данным исследований легочной ткани больных, эндотелин-1 участвует в патогенезе ПЛГ, оказывая вазоконстрикторный эффект. Активация системы эндотелина подтверждает необходимость применения антагонистов рецепторов к этому соединению.</span></li>\r\n<li><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Оксид азота. Данное соединение является сильнодействующим эндогенным вазодилататором. Больным показаны курсы ингаляций на протяжении 2-3 недель.</span></li>\r\n<li><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">ФДЭ-5. Препараты данной группы снижают ОЛСС и нагрузку на правый желудочек. Их прием улучшает движение крови по сосудам, повышает толерантность к физической активности.</span></li>\r\n</ul>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"> </p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">2. Коррекция образа жизни. Пациентам рекомендуются физические нагрузки, которые не сопровождаются выраженной одышкой и болями в области груди. Поскольку гипоксия усугубляет течение болезни, противопоказано пребывание в высокогорных регионах. Авиаперелеты должны сопровождаться возможностью оксигенотерапии. Необходимо проведение профилактики простудных заболеваний, особенно тяжелых вирусных инфекций, способных ухудшить течение ПЛГ. Женщинам рекомендуется использовать барьерные контрацептивные средства. Беременность и роды сопряжены с высоким риском смерти матери.</span></p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"> </p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">3. Хирургическое лечение. Процедура предсердной септостомии направлена на искусственное образование перфорации межпредсердной перегородки – дополнительный сброс крови снижает давление внутри предсердий, улучшает функциональность сердца. Операция сопровождается риском артериальной гипоксемии и смерти, летальность составляет 5-15%. Другой вариант хирургического лечения – трансплантация легких или комплекса сердца и легких. Выживаемость пациентов спустя 3 года достигает 55%.</span></p>",
"prevention": "<p> </p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Система профилактических мер не установлена, генетиками и кардиологами разрабатываются способы скрининговых обследований, которые позволят выявить семьи с отягощенным анамнезом и лиц из групп риска. Последним рекомендуется совместно с врачом решать вопрос о безопасности беременности и родов, своевременно лечить артериальную гипертензию, патологии печени, эндокринные заболевания, избегать заражения ВИЧ и приема веществ, способных спровоцировать ПЛГ.</span></p>",
"clinical_picture": "<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Первым и наиболее распространенным проявлением ПЛГ является инспираторная одышка. Сначала она отмечается лишь при умеренной физической нагрузке, позже начинает сопровождать повседневную двигательную активность. При тяжелом или продолжительном течении заболевания нарушения ритма дыхания возникают внезапно в покое. Другой частый симптом – боль в груди. По характеру она бывает ноющей, жгучей, сжимающей, давящей, колющей. Продолжительность колеблется от минуты до нескольких дней. Как правило, боль нарастает постепенно, заметно обостряется при занятиях спортом, физическом труде.</span></p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"> </p>\r\n<p style=\"line-height: 1.38; margin-top: 0pt; margin-bottom: 0pt;\" dir=\"ltr\"><span style=\"font-size: 6.999999999999999pt; font-family: Verdana; color: #000000; background-color: transparent; font-weight: 400; font-style: normal; font-variant: normal; text-decoration: none; vertical-align: baseline; white-space: pre-wrap;\">Около половины больных при нагрузке испытывают головокружения и обмороки. Внешне это проявляется резким побледнением кожных покровов, а затем появлением синюшной окраски лица, рук и ног, помутнением и иногда – потерей сознания. Длительность подобных симптомов колеблется от 1 до 20-25 минут. У 60% пациентов выявляется учащенное сердцебиение и перебои ритма сердца, у 30% – непродуктивный кашель. У каждого десятого больного развивается однократное или продолжающееся несколько дней кровохарканье. Со временем изменяется форма пальцев – колбовидно утолщаются концевые фаланги, ногти закругляются.</span></p>",
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}
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}