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"code": "Q22.5",
"name": "Аномалия Эбштейна",
"icd_name": "Аномалия Эбштейна",
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"slug": "q22.5_anomaliya_ebshteyna",
"lead": "врожденное смещение створок трехстворчатого клапана",
"description": "<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;\">Аномалия Эбштейна — это врожденная патология трикуспидального клапана, характеризующаяся смещением преимущественно септальной и задней створок клапана в полость правого желудочка, что приводит к образованию над ними так называемой атриализованной части правого желудочка.</span></p>",
"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;\">Выделяют 2 группы факторов, которые могут вызвать возникновение аномалии Эбштейна.</span></p>\r\n<ul style=\"margin-top: 0; margin-bottom: 0; padding-inline-start: 48px;\">\r\n<li><span style=\"font-size: 7pt; background-color: transparent; font-style: normal; font-variant-numeric: normal; font-variant-east-asian: normal; vertical-align: baseline; white-space: pre-wrap;\">Генетические факторы. Врожденные (возникшие внутриутробно) пороки сердца носят наследственный характер — как правило, это заболевание есть у близких родственников больного. Обычно врожденные пороки сердца вызываются точечными изменениями гена или изменениями в хромосомах – носителях генетической информации (хромосомные мутации).</span></li>\r\n<li><span style=\"font-size: 7pt; background-color: transparent; font-style: normal; font-variant-numeric: normal; font-variant-east-asian: normal; vertical-align: baseline; white-space: pre-wrap;\">Экологические факторы. Помимо генетических факторов, способствующих передаче порока сердца по наследству, возможно возникновение генных мутаций под воздействием неблагоприятных экологических факторов во время беременности матери, таких как:</span></li>\r\n<ul style=\"margin-top: 0; margin-bottom: 0; padding-inline-start: 48px;\">\r\n<li style=\"list-style-type: none; font-size: 6.5pt; 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;\" dir=\"ltr\">\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;\">- физические мутагены (факторы, вызывающие мутацию) – главным образом, ионизирующее излучение (при рентгеновских исследованиях, работе в рентгенологическом или радиологическом отделении, при взрывах атомного оружия или катастрофах);</span></p>\r\n</li>\r\n<li style=\"list-style-type: none; font-size: 6.5pt; 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;\" dir=\"ltr\">\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;\">- химические мутагены – фенолы, нитраты, бензпирен (выделяется при курении табака), алкоголь, некоторые лекарственные препараты (антибиотики, противоопухолевые препараты). Также значительную роль в развитии аномалии Эбштайна отводят поступлению в организм беременной женщины лития;</span></p>\r\n</li>\r\n<li style=\"list-style-type: none; font-size: 6.5pt; 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;\" dir=\"ltr\">\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;\">- биологические мутагены – вирус краснухи (инфекционное заболевание), сахарный диабет (нарушение обмена глюкозы (сахара крови)), фенилкетонурия (врожденное нарушение обмена аминокислот – продукта распада белков), системная красная волчанка (заболевание, связанное с действием иммунитета, который повреждает собственные ткани организма) у матери.</span></p>\r\n</li>\r\n</ul>\r\n</ul>",
"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;\">Вследствие того, что створки клапана образованы из тканей желудочка и локализуются в нем же, то происходит значительное уменьшение его полости. Соответственно уменьшению полости предсердия уменьшается и объем крови, выбрасываемый сердцем в легочной ствол.</span></p>\r\n<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;\">Также не следует забывать о том, что створки клапана имеют ненормальную форму и не способны полностью изолировать полость желудочка от полости предсердия. Происходит заброс крови в предсердие, что вызывает застой крови в последнем и его гипертрофию. А поскольку сопровождает это все часто еще и дефект перегородки, то кровь сбрасывается в левое предсердие, что ведет к смешиванию артериальной и венозной крови и снижению насыщения крови кислородом.</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;\">Пациентам с подозрением на аномалию Эбштейна проводится консультация кардиолога и кардиохирурга, ЭКГ, рентгенография грудной клетки, ЭхоКГ, фонокардиография. Перкуторно определяется увеличение размеров сердца вправо, при аускультации выслушивается характерный трех- или четырехтактный ритм, систолический и диастолический шум справа от мечевидного отростка, расщепление II тона.</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;\">ЭКГ-данные включают отклонение ЭОС вправо, признаки гипертрофии и дилатации правого предсердия, пароксизмальную желудочковую экстрасистолию и предсердную тахикардию (синдром WPW), трепетание предсердий, мерцательную аритмию, полную (неполную) блокаду правой ножки пучка Гиса. Фонокардиограмма при аномалии Эбштейна характеризуется наличием систолического шума в проекции правого желудочка; запаздыванием I тона; раздвоенным II тоном; III, IV тонами большой амплитуды.</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;\">Рентгенологические признаки, свидетельствующие в пользу аномалии Эбштейна, представлены резким увеличением правых отделов сердца, шаровидной формой тени сердца, повышенной прозрачностью легочных полей.</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;\">При проведении эхокардиографии видно смещение книзу створок трикуспидального клапана, увеличение размеров правого предсердия, замедленное смыкание трехстворчатого клапана, смещение створок, наличие атриализированного правого желудочка, шунтирующий проток крови справа налево через ДМПП (по данным допплер-эхокардиографии). Фетальная ЭхоКГ, выполненная в пренатальном периоде, позволяет диагностировать аномалию Эбштейна в 60% случаев.</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;\">Для уточнения формы и степени тяжести аномалии Эбштейна проводится МРТ, зондирование полостей сердца, вентрикулография.</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;\">Медикаментозная терапия при аномалии Эбштейна проводится с целью лечения сердечной недостаточности и устранения аритмий. Показаниями к хирургической коррекции аномалии Эбштейна служат наличие жалоб, недостаточности кровообращения и нарушений ритма сердца. Оптимальным для операции является возраст 15-17 лет, при тяжелой форме порока вмешательство проводится в более ранние сроки.</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;\">Радикальная корригирующая операция при аномалии Эбштейна включает пластику или протезирование трикуспидального клапана, пластику ДМПП, ликвидацию атриализованного правого желудочка. В некоторых случаях целесообразным является выполнение операции Фонтена. Иногда на первом этапе для увеличения легочного кровотока и уменьшения гипоксемии прибегают к наложению анастомоза по Блэлоку-Тауссигу, наложению двунаправленного кава-пульмонального анастомоза.</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;\">Добавочные пути проведения импульса при WPW синдроме подвергаются радиочастотной абляции. Для лечения аритмий применяется имплантация кардиостимуляторов или кардиовертер-дефибрилляторов.</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;\">Чтобы защитить будущего ребенка и минимизировать риск развития ВПС, беременным женщинам настоятельно рекомендуется проводить своевременное лечение имеющихся инфекционно-воспалительных и соматических заболеваний. В период вынашивания плода необходимо выполнять все врачебные рекомендации, исключить прием препаратов на основе лития и любые токсичные тератогенные воздействия.</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;\">В зависимости от тяжести нарушений гемодинамики выделяют 3 стадии течения аномалии Эбштейна: I — бессимптомную (встречается редко); II — стадию выраженных гемодинамических расстройств (IIa — без нарушений сердечного ритма; IIб — с нарушениями сердечного ритма), III — стадию стойкой декомпенсации.</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;\">Самые тяжелые формы аномалии Эбштейна могут вызывать внутриутробную гибель плода. При благоприятном варианте порока его течение долго время остается бессимптомным; физическое развитие детей соответствует возрасту. В типичных случаях аномалия Эбштейна проявляется в раннем детстве, иногда в первые месяцы жизни ребенка.</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;\">Клинические симптомы включают диффузный цианоз, плохую переносимость физической нагрузки, боли в сердце, приступы сердцебиения. У 25-50% пациентов с аномалией Эбштейна отмечается пароксизмальная наджелудочковая тахикардия, у 14% из них - синдром WPW. При внешнем осмотре обращает внимание изменения концевых фаланг пальцев по типу «барабанных палочек» и ногтей в виде «часовых стекол», «сердечный горб».</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;\">При аномалии Эбштейна может рано развиваться правожелудочковая недостаточность - одышка, увеличение печени, набухание и пульсация шейных вен. Часто отмечается артериальная гипотония. Течение аномалии Эбштейна неуклонно прогрессирующее. Причиной смерти больных старшего возраста чаще всего служат сердечная недостаточность и тяжелые нарушения ритма.</span></p>",
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