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"slug": "i37.1_nedostatochnost_klapana_legochnoy_arterii",
"lead": "порок сердца, при котором происходит обратное движение крови из легочной артерии (сосуда, несущего кровь к легким) в правый желудочек во время расслабления желудочков сердца вследствие неполного смыкания его створок",
"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;\">Недостаточность клапана легочной артерии — один из дефектов порока сердца, при котором происходит неполное закрытие клапаном во время диастолы правого желудочка отверстия легочной артерии, что приводит к возникновению оттока крови из артерии обратно в желудочек.</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;\">Врожденная форма порока формируется вследствие дилатации легочного ствола или нарушения развития створок клапана. В детской кардиологии встречается гипоплазия и полное отсутствие клапана легочной артерии (агенезия, врожденная бес­клапанная легочная артерия). Иногда в легочном клапане отсутствует одна створка, а две другие могут быть нормальными или рудиментарными. Врожденная недостаточность клапана легочной артерии обычно встречается при тетраде Фалло, сочетается с открытым артериальным протоком, дефектом межпредсердной перегородки, дефектом межжелудочковой перегородки, двойным отхождением магистральных сосудов от правого желудочка, синдромом Марфана.</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;\">Наиболее частой причиной приобретенной недостаточности клапана легочной артерии служит септический эндокардит. К расширению клапанных комиссур может приводить поражение легочной артерии при сифилисе, атеросклерозе.</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;\">Недостаточность клапана легочной артерии может возникать после баллонной вальвулопластики и вальвулотомии. Относительная недостаточность клапана легочной артерии встречается при легочной гипертензии, хронических заболеваниях легких и митральном стенозе, тромбоэмболии легочной артерии. Иногда недостаточность клапана развивается вследствие давления аневризмы аорты на легочную артерию.</span></p>",
"pathogenesis": "",
"diagnostics": "<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;\">Диагностика тяжелых врожденных пороков осуществляется непосредственно после рождения ребенка. Легкие формы клапанной недостаточности, а также приобретенные патологии диагностируются в более зрелом возрасте при проявлении симптомов или во время диспансерного наблюдения. Характерным объективным признаком порока является шум, определяемый врачом при аускультации сердца. </span></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 окончательной диагностики врожденных и приобретенных пороков клапана легочной артерии кардиологами клиники применяются высокоточные методы исследования, среди которых: </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;\">Эхокардиография – визуализация сердца при помощи ультразвукового сканера;</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;\">Рентгенография органов грудной клетки с целью оценки размера сердца и легочных изменений; </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;\">Контрастная рентгенография по протоколу исследования сосудов (ангиография), в том числе коронарная ангиография; </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;\">Измерение уровня кислорода и давления в камерах сердца при помощи малоинвазивной катетеризации; </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;\">Пульсоксиметрия – неинвазивный мониторинг насыщения крови кислородом; </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;\">Электрокардиографические исследования (запись ЭКГ в реальном времени и продолжительный мониторинг сердечного ритма). Запись электрических потенциалов сердца помогает кардиологам диагностировать сопровождающие клапанный порок ишемические нарушения, аритмии и гипертрофию миокарда; </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;\">Томографические методы исследования сердца (компьютерная и магнитно-резонансная томография); </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;\">Изотопное сканирование легочной перфузии.</span></li>\r\n</ul>",
"treatment": "<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;\">Медикаментозное лечение пороков легочной артерии направлено на предотвращение развития и прогрессирования сердечной недостаточности. </span></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;\">Хирургическое лечение, в большинстве случаев, способно нормализовать гемодинамику путем устранения сердечного порока. </span></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;\">Баллонное расширение стеноза, стентирование и применение инновационной методики режущего баллона, позволяющей не только расширить суженный участок, но и удалить избыток фиброзной ткани, приводящей к рецидиву стеноза; </span></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;\">Органосохраняющие процедуры реконструкции поврежденного клапана, предотвращающие необходимость его замены; </span></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;\">Операции на открытом сердце с целью восстановления нормальной проходимости при атрезии и стенозе легочного ствола; </span></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;\">Операции по замене клапана легочной артерии. Искусственные биологические или механические клапаны полностью справляются со своей функцией, гарантируя восстановление гемодинамики на долгие годы. </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;\">Эндоваскулярное малоинвазивное протезирование в кардиохирургии:</span></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;\">Процедура проводится под местной анестезией. С помощью катетера, который вводится в бедренную вену, искусственный клапан, упакованный в специальную гильзу-cтент, доставляется через правый желудочек в место повреждённого лёгочного клапана (чаще всего, дегенерировавшего гомографта). После контроля местоположения нового клапана с помощью Эхо-КГ и рентгена, клапан расправляется и фиксируется в правильной позиции. Процедура длится около часа и позволяет избежать повторной операции у детей и взрослых с применением аппарата искусственного кровообращения и рискa, связанного с открытием грудной клетки при наличии сращений после первой операции.</span></p>",
"prevention": "<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 ведении беременности у женщин с недостаточностью клапана легочной артерии необходимо проводить профилактику гипоксии плода и преждевременных родов. Беременным требуется УЗИ-мониторинг маточно-плацентарного кровообращения, наблюдение акушера-гинеколога, кардиолога, терапевта, кардиохирурга.</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;\">Профилактика вторичной недостаточности клапана легочной артерии сводится к предупреждению бактериального эндокардита, атеросклероза, сифилиса, хронических болезней легких, ятрогенных осложнений при операциях на сердце. Профилактика первичной недостаточности клапана легочной артерии – такая же, как при других ВПС.</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;\">Клиника врожденной недостаточности клапана легочной артерии манифестирует вскоре после рождения с признаков правожелудочковой недостаточности, респираторных нарушений, обусловленных сдавлением бронхов и трахеи расширенными ветвями легочной артерии. При изолированной вторичной недостаточности клапана легочной артерии клинических проявлений может не отмечаться длительное время.</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;\">Жалобы появляются при прогрессировании недостаточности правого желудочка и трикуспидальной недостаточности. У больных появляется сонливость, слабость, сердцебиение, приступы одышки, цианоз, боли в сердце. У взрослых больных старше 20-30 лет возможно возникновение аритмий. При осмотре обращает внимание набухание шейных вен, изменение фаланг пальцев в виде барабанных палочек. В дальнейшем возникают периферические отеки, стойкая тахикардия, гидроторакс, асцит, кардиальный цирроз печени. К наиболее опасным осложнениям недостаточности клапана легочной артерии следует отнести легочные эмболии, аневризму легочной артерии.</span></p>",
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