{"id":11049,"date":"2026-03-02T22:01:57","date_gmt":"2026-03-02T21:01:57","guid":{"rendered":"https:\/\/valintermed.com\/?p=11049"},"modified":"2026-03-02T22:01:57","modified_gmt":"2026-03-02T21:01:57","slug":"defekt-endokardialnoy-podushki","status":"publish","type":"post","link":"https:\/\/valintermed.com\/en\/medlibrary\/defect-of-the-endocardial-cushion\/","title":{"rendered":"Endocardial cushion defect"},"content":{"rendered":"<div class=\"fpm_start\"><\/div>\n<p>The defect of the endocardial cushion is a congenital heart defect in which the normal formation of structures separating the right and left parts of the heart by the interatrial and interventricular septa is disrupted. It is not just a \"hole\" in the heart, but a complex developmental anomaly affecting several key elements: the atrioventricular valves (mitral and tricuspid), the septa, and the walls of the ventricles. As a result, a mixed blood shunt occurs \u2014 part of the blood from the left atrium enters the right, and from the left ventricle \u2014 into the right, leading to overload of the pulmonary circulation, hypertension in the pulmonary artery, and ultimately heart failure. In newborns, this defect may manifest within the first days of life: shortness of breath, cyanosis, poor weight gain, rapid heartbeat. However, symptoms may also appear later \u2014 in early childhood or even in adulthood, especially if the defect is partial and functional disturbances are compensated by the body.<\/p>\n<div id=\"ez-toc-container\" class=\"ez-toc-v2_0_85 counter-flat ez-toc-counter ez-toc-light-blue ez-toc-container-direction\">\n<div class=\"ez-toc-title-container\">\n<p class=\"ez-toc-title\" style=\"cursor:inherit\">Content<\/p>\n<span class=\"ez-toc-title-toggle\"><a href=\"#\" class=\"ez-toc-pull-right ez-toc-btn ez-toc-btn-xs ez-toc-btn-default ez-toc-toggle\" aria-label=\"Toggle Table of Content\"><span class=\"ez-toc-js-icon-con\"><span class=\"\"><span class=\"eztoc-hide\" style=\"display:none;\">Toggle<\/span><span class=\"ez-toc-icon-toggle-span\"><svg style=\"fill: #999;color:#999\" xmlns=\"http:\/\/www.w3.org\/2000\/svg\" class=\"list-377408\" width=\"20px\" height=\"20px\" viewbox=\"0 0 24 24\" fill=\"none\"><path d=\"M6 6H4v2h2V6zm14 0H8v2h12V6zM4 11h2v2H4v-2zm16 0H8v2h12v-2zM4 16h2v2H4v-2zm16 0H8v2h12v-2z\" fill=\"currentColor\"><\/path><\/svg><svg style=\"fill: #999;color:#999\" class=\"arrow-unsorted-368013\" xmlns=\"http:\/\/www.w3.org\/2000\/svg\" width=\"10px\" height=\"10px\" viewbox=\"0 0 24 24\" version=\"1.2\" baseprofile=\"tiny\"><path d=\"M18.2 9.3l-6.2-6.3-6.2 6.3c-.2.2-.3.4-.3.7s.1.5.3.7c.2.2.4.3.7.3h11c.3 0 .5-.1.7-.3.2-.2.3-.5.3-.7s-.1-.5-.3-.7zM5.8 14.7l6.2 6.3 6.2-6.3c.2-.2.3-.5.3-.7s-.1-.5-.3-.7c-.2-.2-.4-.3-.7-.3h-11c-.3 0-.5.1-.7.3-.2.2-.3.5-.3.7s.1.5.3.7z\"\/><\/svg><\/span><\/span><\/span><\/a><\/span><\/div>\n<nav><ul class='ez-toc-list ez-toc-list-level-1 eztoc-toggle-hide-by-default' ><li class='ez-toc-page-1'><a class=\"ez-toc-link ez-toc-heading-1\" href=\"https:\/\/valintermed.com\/en\/medlibrary\/defect-of-the-endocardial-cushion\/#%D0%9A%D0%BB%D0%B0%D1%81%D1%81%D0%B8%D1%84%D0%B8%D0%BA%D0%B0%D1%86%D0%B8%D1%8F_%D0%B7%D0%B0%D0%B1%D0%BE%D0%BB%D0%B5%D0%B2%D0%B0%D0%BD%D0%B8%D1%8F_%D0%BF%D0%BE_%D0%9C%D0%9A%D0%91\" >Classification of the disease according to ICD<\/a><\/li><li class='ez-toc-page-1'><a class=\"ez-toc-link ez-toc-heading-2\" href=\"https:\/\/valintermed.com\/en\/medlibrary\/defect-of-the-endocardial-cushion\/#%D0%98%D1%81%D1%82%D0%BE%D1%80%D0%B8%D1%8F_%D0%B7%D0%B0%D0%B1%D0%BE%D0%BB%D0%B5%D0%B2%D0%B0%D0%BD%D0%B8%D1%8F_%D0%B8_%D0%B8%D0%BD%D1%82%D0%B5%D1%80%D0%B5%D1%81%D0%BD%D1%8B%D0%B5_%D0%B8%D1%81%D1%82%D0%BE%D1%80%D0%B8%D1%87%D0%B5%D1%81%D0%BA%D0%B8%D0%B5_%D1%84%D0%B0%D0%BA%D1%82%D1%8B\" >History of the disease and interesting historical facts<\/a><\/li><li class='ez-toc-page-1'><a class=\"ez-toc-link ez-toc-heading-3\" href=\"https:\/\/valintermed.com\/en\/medlibrary\/defect-of-the-endocardial-cushion\/#%D0%AD%D0%BF%D0%B8%D0%B4%D0%B5%D0%BC%D0%B8%D0%BE%D0%BB%D0%BE%D0%B3%D0%B8%D1%8F_%D1%81%D1%82%D0%B0%D1%82%D0%B8%D1%81%D1%82%D0%B8%D0%BA%D0%B0_%D0%B2%D0%BE%D0%B7%D0%BD%D0%B8%D0%BA%D0%BD%D0%BE%D0%B2%D0%B5%D0%BD%D0%B8%D1%8F_%D0%B7%D0%B0%D0%B1%D0%BE%D0%BB%D0%B5%D0%B2%D0%B0%D0%BD%D0%B8%D1%8F\" >Epidemiology (statistics of disease occurrence)<\/a><\/li><li class='ez-toc-page-1'><a class=\"ez-toc-link ez-toc-heading-4\" href=\"https:\/\/valintermed.com\/en\/medlibrary\/defect-of-the-endocardial-cushion\/#%D0%93%D0%B5%D0%BD%D0%B5%D1%82%D0%B8%D1%87%D0%B5%D1%81%D0%BA%D0%B0%D1%8F_%D0%BF%D1%80%D0%B5%D0%B4%D1%80%D0%B0%D1%81%D0%BF%D0%BE%D0%BB%D0%BE%D0%B6%D0%B5%D0%BD%D0%BD%D0%BE%D1%81%D1%82%D1%8C_%D0%BA_%D0%B4%D0%B0%D0%BD%D0%BD%D0%BE%D0%BC%D1%83_%D0%B7%D0%B0%D0%B1%D0%BE%D0%BB%D0%B5%D0%B2%D0%B0%D0%BD%D0%B8%D1%8E_%D0%B2%D0%BE%D0%B2%D0%BB%D0%B5%D1%87%D1%91%D0%BD%D0%BD%D1%8B%D0%B5_%D0%B3%D0%B5%D0%BD%D1%8B_%D0%B8_%D0%BC%D1%83%D1%82%D0%B0%D1%86%D0%B8%D0%B8\" >Genetic predisposition to the disease (involved genes and mutations)<\/a><\/li><li class='ez-toc-page-1'><a class=\"ez-toc-link ez-toc-heading-5\" href=\"https:\/\/valintermed.com\/en\/medlibrary\/defect-of-the-endocardial-cushion\/#%D0%A4%D0%B0%D0%BA%D1%82%D0%BE%D1%80%D1%8B_%D1%80%D0%B8%D1%81%D0%BA%D0%B0_%D0%B2%D0%BE%D0%B7%D0%BD%D0%B8%D0%BA%D0%BD%D0%BE%D0%B2%D0%B5%D0%BD%D0%B8%D1%8F_%D0%B4%D0%B0%D0%BD%D0%BD%D0%BE%D0%B3%D0%BE_%D0%B7%D0%B0%D0%B1%D0%BE%D0%BB%D0%B5%D0%B2%D0%B0%D0%BD%D0%B8%D1%8F_%D1%84%D0%B8%D0%B7%D0%B8%D1%87%D0%B5%D1%81%D0%BA%D0%B8%D0%B5_%D1%85%D0%B8%D0%BC%D0%B8%D1%87%D0%B5%D1%81%D0%BA%D0%B8%D0%B5_%D1%84%D0%B0%D0%BA%D1%82%D0%BE%D1%80%D1%8B_%D1%80%D0%B8%D1%81%D0%BA%D0%B0_%D0%B4%D1%80%D1%83%D0%B3%D0%B8%D0%B5_%D0%B2%D0%BE%D0%B7%D0%BC%D0%BE%D0%B6%D0%BD%D1%8B%D0%B5_%D1%81%D0%B2%D1%8F%D0%B7%D0%B0%D0%BD%D0%BD%D1%8B%D0%B5_%D1%81_%D0%B4%D0%B0%D0%BD%D0%BD%D1%8B%D0%BC_%D0%B7%D0%B0%D0%B1%D0%BE%D0%BB%D0%B5%D0%B2%D0%B0%D0%BD%D0%B8%D0%B5%D0%BC\" >Risk factors for the occurrence of this disease (physical and chemical risk factors, other possible associations with this disease)<\/a><\/li><li class='ez-toc-page-1'><a class=\"ez-toc-link ez-toc-heading-6\" href=\"https:\/\/valintermed.com\/en\/medlibrary\/defect-of-the-endocardial-cushion\/#%D0%94%D0%B8%D0%B0%D0%B3%D0%BD%D0%BE%D1%81%D1%82%D0%B8%D0%BA%D0%B0_%D0%B4%D0%B0%D0%BD%D0%BD%D0%BE%D0%B3%D0%BE_%D0%B7%D0%B0%D0%B1%D0%BE%D0%BB%D0%B5%D0%B2%D0%B0%D0%BD%D0%B8%D1%8F_%D0%9E%D1%81%D0%BD%D0%BE%D0%B2%D0%BD%D1%8B%D0%B5_%D1%81%D0%B8%D0%BC%D0%BF%D1%82%D0%BE%D0%BC%D1%8B_%D0%9B%D0%B0%D0%B1%D0%BE%D1%80%D0%B0%D1%82%D0%BE%D1%80%D0%BD%D1%8B%D0%B5_%D0%A0%D0%B0%D0%B4%D0%B8%D0%BE%D0%BB%D0%BE%D0%B3%D0%B8%D1%87%D0%B5%D1%81%D0%BA%D0%B8%D0%B5_%D0%BE%D0%B1%D1%81%D0%BB%D0%B5%D0%B4%D0%BE%D0%B2%D0%B0%D0%BD%D0%B8%D1%8F_%D0%94%D1%80%D1%83%D0%B3%D0%B8%D0%B5_%D0%B2%D0%B8%D0%B4%D1%8B_%D0%B4%D0%B8%D0%B0%D0%B3%D0%BD%D0%BE%D1%81%D1%82%D0%B8%D0%BA%D0%B8_%D0%B7%D0%B0%D0%B1%D0%BE%D0%BB%D0%B5%D0%B2%D0%B0%D0%BD%D0%B8%D1%8F_%D0%94%D0%B8%D1%84%D1%84%D0%B5%D1%80%D0%B5%D0%BD%D1%86%D0%B8%D0%B0%D0%BB%D1%8C%D0%BD%D1%8B%D0%B9_%D0%B4%D0%B8%D0%B0%D0%B3%D0%BD%D0%BE%D0%B7\" >Diagnosis of this disease (Main symptoms, Laboratory Radiological examinations, Other types of disease diagnosis, Differential diagnosis)<\/a><\/li><li class='ez-toc-page-1'><a class=\"ez-toc-link ez-toc-heading-7\" href=\"https:\/\/valintermed.com\/en\/medlibrary\/defect-of-the-endocardial-cushion\/#%D0%9B%D0%B5%D1%87%D0%B5%D0%BD%D0%B8%D0%B5_%D0%BE%D0%B1%D1%89%D0%B5%D0%B5_%D0%BB%D0%B5%D1%87%D0%B5%D0%BD%D0%B8%D0%B5_%D1%84%D0%B0%D1%80%D0%BC%D0%B0%D0%BA%D0%BE%D0%BB%D0%BE%D0%B3%D0%B8%D1%87%D0%B5%D1%81%D0%BA%D0%BE%D0%B5_%D0%BB%D0%B5%D1%87%D0%B5%D0%BD%D0%B8%D0%B5_%D1%85%D0%B8%D1%80%D1%83%D1%80%D0%B3%D0%B8%D1%87%D0%B5%D1%81%D0%BA%D0%BE%D0%B5_%D0%BB%D0%B5%D1%87%D0%B5%D0%BD%D0%B8%D0%B5_%D0%B4%D1%80%D1%83%D0%B3%D0%B8%D0%B5_%D0%B2%D0%B8%D0%B4%D1%8B_%D0%BB%D0%B5%D1%87%D0%B5%D0%BD%D0%B8%D1%8F_%D0%BA_%D0%B4%D0%B0%D0%BD%D0%BD%D0%BE%D0%BC%D1%83_%D0%B2%D0%B8%D0%B4%D1%83_%D0%B7%D0%B0%D0%B1%D0%BE%D0%BB%D0%B5%D0%B2%D0%B0%D0%BD%D0%B8%D1%8F\" >Treatment (general treatment, pharmacological treatment, surgical treatment, other types of treatment for this type of disease)<\/a><\/li><li class='ez-toc-page-1'><a class=\"ez-toc-link ez-toc-heading-8\" href=\"https:\/\/valintermed.com\/en\/medlibrary\/defect-of-the-endocardial-cushion\/#%D0%A1%D0%BF%D0%B8%D1%81%D0%BE%D0%BA_%D0%BB%D0%B5%D0%BA%D0%B0%D1%80%D1%81%D1%82%D0%B2_%D0%BF%D1%80%D0%B8%D0%BC%D0%B5%D0%BD%D1%8F%D0%B5%D0%BC%D1%8B%D1%85_%D0%B4%D0%BB%D1%8F_%D0%BB%D0%B5%D1%87%D0%B5%D0%BD%D0%B8%D1%8F_%D0%B4%D0%B0%D0%BD%D0%BD%D0%BE%D0%B3%D0%BE_%D0%B7%D0%B0%D0%B1%D0%BE%D0%BB%D0%B5%D0%B2%D0%B0%D0%BD%D0%B8%D1%8F\" >List of drugs used to treat this disease<\/a><\/li><li class='ez-toc-page-1'><a class=\"ez-toc-link ez-toc-heading-9\" href=\"https:\/\/valintermed.com\/en\/medlibrary\/defect-of-the-endocardial-cushion\/#%D0%9C%D0%BE%D0%BD%D0%B8%D1%82%D0%BE%D1%80%D0%B8%D0%BD%D0%B3_%D0%B7%D0%B0%D0%B1%D0%BE%D0%BB%D0%B5%D0%B2%D0%B0%D0%BD%D0%B8%D1%8F_%D0%BA%D0%BE%D0%BD%D1%82%D1%80%D0%BE%D0%BB%D1%8C%D0%BD%D1%8B%D0%B5_%D1%8D%D1%82%D0%B0%D0%BF%D1%8B_%D0%BF%D1%80%D0%BE%D0%B3%D0%BD%D0%BE%D0%B7%D1%81%D0%BB%D0%BE%D0%B6%D0%BD%D0%B5%D0%BD%D0%B8%D1%8F\" >Monitoring of the disease (control stages, prognosis, complications)<\/a><\/li><li class='ez-toc-page-1'><a class=\"ez-toc-link ez-toc-heading-10\" href=\"https:\/\/valintermed.com\/en\/medlibrary\/defect-of-the-endocardial-cushion\/#%D0%92%D0%BE%D0%B7%D1%80%D0%B0%D1%81%D1%82%D0%BD%D1%8B%D0%B5_%D0%BE%D1%81%D0%BE%D0%B1%D0%B5%D0%BD%D0%BD%D0%BE%D1%81%D1%82%D0%B8_%D0%B7%D0%B0%D0%B1%D0%BE%D0%BB%D0%B5%D0%B2%D0%B0%D0%BD%D0%B8%D1%8F_%D0%BA%D0%B0%D0%BA_%D0%BF%D1%80%D0%BE%D1%82%D0%B5%D0%BA%D0%B0%D0%B5%D1%82_%D0%B2_%D1%80%D0%B0%D0%B7%D0%BD%D1%8B%D1%85_%D0%B2%D0%BE%D0%B7%D1%80%D0%B0%D1%81%D1%82%D0%BD%D1%8B%D1%85_%D0%B3%D1%80%D1%83%D0%BF%D0%BF%D0%B0%D1%85160_%D1%83%D0%B4%D0%BC%D0%B8%D0%BD_%D0%91%D0%B5%D0%B7_%D0%BE%D0%BF%D0%B5%D1%80%D0%B0%D1%86%D0%B8%D0%B8_%D0%B2%D1%8B%D0%B6%D0%B8%D0%B2%D0%B0%D0%B5%D0%BC%D0%BE%D1%81%D1%82%D1%8C_%D0%B4%D0%BE_6_%E2%80%94_%D0%BC%D0%B5%D0%BD%D0%B5%D0%B5_30_%D0%A7%D0%B0%D1%81%D1%82%D0%B8%D1%87%D0%BD%D1%8B%D0%B9_%D0%B4%D0%B5%D1%84%D0%B5%D0%BA%D1%82_%D0%B2_%D1%8D%D1%82%D0%BE%D0%BC_%D0%B2%D0%BE%D0%B7%D1%80%D0%B0%D1%81%D1%82%D0%B5_%D1%87%D0%B0%D1%81%D1%82%D0%BE_%D0%B1%D0%B5%D1%81%D1%81%D0%B8%D0%BC%D0%BF%D1%82%D0%BE%D0%BC%D0%B5%D0%BD_%D0%B8%D0%BB%D0%B8_%D0%BC%D0%B0%D1%81%D0%BA%D0%B8%D1%80%D1%83%D0%B5%D1%82%D1%81%D1%8F_%D0%BF%D0%BE%D0%B4_%C2%AB%D1%81%D0%BB%D0%B0%D0%B1%D0%BE%D1%81%D1%82%D1%8C%C2%BB_%D0%93%D1%80%D1%83%D0%B4%D0%BD%D1%8B%D0%B5_%D0%B4%D0%B5%D1%82%D0%B81%E2%80%9312_%D0%BC%D0%B5%D1%81%D1%8F%D1%86%D0%B5%D0%B2_%D0%AD%D1%82%D0%BE_%C2%AB%D0%BA%D1%80%D0%B8%D1%82%D0%B8%D1%87%D0%B5%D1%81%D0%BA%D0%B8%D0%B9_%D0%BF%D0%B5%D1%80%D0%B8%D0%BE%D0%B4%C2%BB_%D0%B4%D0%BB%D1%8F_%D0%9F%D0%94%D0%95%D0%9F_%D0%9A_3%E2%80%934_%D0%BC%D0%B5%D1%81%D1%8F%D1%86%D0%B0%D0%BC_%D1%80%D0%B0%D0%B7%D0%B2%D0%B8%D0%B2%D0%B0%D0%B5%D1%82%D1%81%D1%8F_%D0%BB%D1%91%D0%B3%D0%BE%D1%87%D0%BD%D0%B0%D1%8F_%D0%B3%D0%B8%D0%BF%D0%B5%D1%80%D1%82%D0%B5%D0%BD%D0%B7%D0%B8%D1%8F_%D1%80%D0%BE%D1%81%D1%82_%D0%B7%D0%B0%D0%BC%D0%B5%D0%B4%D0%BB%D1%8F%D0%B5%D1%82%D1%81%D1%8F_%D0%BF%D0%BE%D1%8F%D0%B2%D0%BB%D1%8F%D0%B5%D1%82%D1%81%D1%8F_%D0%B3%D0%B5%D0%BF%D0%B0%D1%82%D0%BE%D0%BC%D0%B5%D0%B3%D0%B0%D0%BB%D0%B8%D1%8F_%D0%A3_%D0%B4%D0%B5%D1%82%D0%B5%D0%B9_%D1%81_%D0%A7%D0%94%D0%95%D0%9F_%E2%80%94_%D1%83%D0%BC%D0%B5%D1%80%D0%B5%D0%BD%D0%BD%D0%B0%D1%8F_%D0%BE%D0%B4%D1%8B%D1%88%D0%BA%D0%B0_%D0%BF%D1%80%D0%B8_%D0%BA%D0%BE%D1%80%D0%BC%D0%BB%D0%B5%D0%BD%D0%B8%D0%B8_%D0%BD%D0%BE_%D0%B2%D0%B5%D1%81_%D0%BD%D0%B0%D0%B1%D0%B8%D1%80%D0%B0%D0%B5%D1%82%D1%81%D1%8F_%D1%85%D0%BE%D1%82%D1%8F_%D0%B8_%D0%BC%D0%B5%D0%B4%D0%BB%D0%B5%D0%BD%D0%BD%D0%B5%D0%B5_%D0%BD%D0%BE%D1%80%D0%BC%D1%8B_%D0%94%D0%BE%D1%88%D0%BA%D0%BE%D0%BB%D1%8C%D0%BD%D1%8B%D0%B9_%D0%B2%D0%BE%D0%B7%D1%80%D0%B0%D1%81%D1%82_1%E2%80%935_%D0%BB%D0%B5%D1%82_%D0%95%D1%81%D0%BB%D0%B8_%D0%BE%D0%BF%D0%B5%D1%80%D0%B0%D1%86%D0%B8%D1%8F_%D0%BD%D0%B5_%D1%81%D0%B4%D0%B5%D0%BB%D0%B0%D0%BD%D0%B0_%D1%83%D1%91%D0%BD%D0%BA%D0%B0_%D1%84%D0%BE%D1%80%D0%BC%D0%B8%D1%80%D1%83%D0%B5%D1%82%D1%81%D1%8F_%C2%AB%D1%81%D0%B5%D1%80%D0%B4%D0%B5%D1%87%D0%BD%D1%8B%D0%B9_%D0%B3%D0%BE%D1%80%D0%B1%C2%BB_%D0%BF%D0%BE%D1%81%D1%82%D0%BE%D1%8F%D0%BD%D0%BD%D0%B0%D1%8F_%D1%83%D1%81%D1%82%D0%B0%D0%BB%D0%BE%D1%81%D1%82%D1%8C_%D0%BE%D0%B3%D1%80%D0%B0%D0%BD%D0%B8%D1%87%D0%B5%D0%BD%D0%B8%D0%B5_%D1%84%D0%B8%D0%B7%D0%B8%D1%87%D0%B5%D1%81%D0%BA%D0%BE%D0%B9_%D0%B0%D0%BA%D1%82%D0%B8%D0%B2%D0%BD%D0%BE%D1%81%D1%82%D0%B8_%D0%A3_%D0%BD%D0%B5%D0%BA%D0%BE%D1%82%D0%BE%D1%80%D1%8B%D1%85_%E2%80%94_%D1%80%D0%B5%D1%86%D0%B8%D0%B4%D0%B8%D0%B2%D0%B8%D1%80%D1%83%D1%8E%D1%89%D0%B8%D0%B5_%D0%B1%D1%80%D0%BE%D0%BD%D1%85%D0%B8%D1%82%D1%8B_%D0%B8%D0%B7-%D0%B7%D0%B0_%D0%B7%D0%B0%D1%81%D1%82%D0%BE%D1%8F_%D0%B2_%D0%BB%D1%91%D0%B3%D0%BA%D0%B8%D1%85_%D0%A8%D0%BA%D0%BE%D0%BB%D1%8C%D0%BD%D1%8B%D0%B9_%D0%B8_%D0%BF%D0%BE%D0%B4%D1%80%D0%BE%D1%81%D1%82%D0%BA%D0%BE%D0%B2%D1%8B%D0%B96%E2%80%9318_%D0%BB%D0%B5%D1%82_%D0%9F%D1%80%D0%B8_%D0%BD%D0%B5%D0%BE%D0%BF%D0%B5%D1%80%D0%B8%D1%80%D0%BE%D0%B2%D0%B0%D0%BD%D0%BD%D0%BE%D0%BC_%D0%A7%D0%94%D0%95%D0%9F_%E2%80%94_%D0%BF%D0%BE%D1%81%D1%82%D0%B5%D0%BF%D0%B5%D0%BD%D0%BD%D0%BE%D0%B5_%D1%80%D0%B0%D0%B7%D0%B2%D0%B8%D1%82%D0%B8%D0%B5_%D0%BB%D1%91%D0%B3%D0%BE%D1%87%D0%BD%D0%BE%D0%B9_%D0%B3%D0%B8%D0%BF%D0%B5%D1%80%D1%82%D0%B5%D0%BD%D0%B7%D0%B8%D0%B8_%D0%B0%D1%80%D0%B8%D1%82%D0%BC%D0%B8%D0%B9_%D1%81%D0%BD%D0%B8%D0%B6%D0%B5%D0%BD%D0%B8%D0%B5_%D1%82%D0%BE%D0%BB%D0%B5%D1%80%D0%B0%D0%BD%D1%82%D0%BD%D0%BE%D1%81%D1%82%D0%B8_%D0%BA_%D0%BD%D0%B0%D0%B3%D1%80%D1%83%D0%B7%D0%BA%D0%B5_%D0%A3_%D0%B2%D0%B7%D1%80%D0%BE%D1%81%D0%BB%D1%8B%D1%85_%D1%81_%D0%BD%D0%B5%D0%BB%D0%B5%D1%87%D0%B5%D0%BD%D0%BD%D1%8B%D0%BC_%D0%9F%D0%94%D0%95%D0%9F_%E2%80%94_%D0%B2%D1%8B%D1%81%D0%BE%D0%BA%D0%B8%D0%B9_%D1%80%D0%B8%D1%81%D0%BA_%D1%81%D0%B5%D1%80%D0%B4%D0%B5%D1%87%D0%BD%D0%BE%D0%B9_%D0%BD%D0%B5%D0%B4%D0%BE%D1%81%D1%82%D0%B0%D1%82%D0%BE%D1%87%D0%BD%D0%BE%D1%81%D1%82%D0%B8_%D0%B8_%D0%B2%D0%BD%D0%B5%D0%B7%D0%B0%D0%BF%D0%BD%D0%BE%D0%B9_%D1%81%D0%BC%D0%B5%D1%80%D1%82%D0%B8_%D0%BA_20%E2%80%9330_%D0%B3%D0%BE%D0%B4%D0%B0%D0%BC_%D0%92%D0%B7%D1%80%D0%BE%D1%81%D0%BB%D1%8B%D0%B5_18_%D0%BB%D0%B5%D1%82_%D0%92%D1%81%D1%82%D1%80%D0%B5%D1%87%D0%B0%D1%8E%D1%82%D1%81%D1%8F_%D1%80%D0%B5%D0%B4%D0%BA%D0%B8%D0%B5_%D1%81%D0%BB%D1%83%D1%87%D0%B0%D0%B8_%D0%BA%D0%BE%D0%B3%D0%B4%D0%B0_%D0%B4%D0%B5%D1%84%D0%B5%D0%BA%D1%82_%D0%BE%D1%81%D1%82%D0%B0%D0%B2%D0%B0%D0%BB%D1%81%D1%8F_%D0%BD%D0%B5%D0%B7%D0%B0%D0%BC%D0%B5%D1%87%D0%B5%D0%BD%D0%BD%D1%8B%D0%BC_%D0%B4%D0%BE_%D0%B2%D0%B7%D1%80%D0%BE%D1%81%D0%BB%D0%BE%D0%B3%D0%BE_%D0%B2%D0%BE%D0%B7%D1%80%D0%B0%D1%81%D1%82%D0%B0_%D0%A7%D0%B0%D1%89%D0%B5_%E2%80%94_%D1%8D%D1%82%D0%BE_%D1%87%D0%B0%D1%81%D1%82%D0%B8%D1%87%D0%BD%D0%B0%D1%8F_%D1%84%D0%BE%D1%80%D0%BC%D0%B0_%D1%81_%D0%BC%D0%B8%D0%BD%D0%B8%D0%BC%D0%B0%D0%BB%D1%8C%D0%BD%D1%8B%D0%BC%D0%B8_%D1%81%D0%B8%D0%BC%D0%BF%D1%82%D0%BE%D0%BC%D0%B0%D0%BC%D0%B8_%D0%9D%D0%BE_%D0%B4%D0%B0%D0%B6%D0%B5_%D0%BF%D1%80%D0%B8_%D1%85%D0%BE%D1%80%D0%BE%D1%88%D0%B5%D0%BC_%D1%81%D0%B0%D0%BC%D0%BE%D1%87%D1%83%D0%B2%D1%81%D1%82%D0%B2%D0%B8%D0%B8_%D1%83_%D1%82%D0%B0%D0%BA%D0%B8%D1%85_%D0%BF%D0%B0%D1%86%D0%B8%D0%B5%D0%BD%D1%82%D0%BE%D0%B2_%D0%BF%D0%BE%D0%B2%D1%8B%D1%88%D0%B5%D0%BD%D0%B0_%D0%BE%D0%BF%D0%B0%D1%81%D0%BD%D0%BE%D1%81%D1%82%D1%8C\" >Age-related features of the disease (how it progresses in different age groups) The endocardial cushion defect manifests differently depending on age \u2014 this is related to physiological changes in the vascular system and the adaptive capabilities of the body.<\/a><\/li><li class='ez-toc-page-1'><a class=\"ez-toc-link ez-toc-heading-11\" href=\"https:\/\/valintermed.com\/en\/medlibrary\/defect-of-the-endocardial-cushion\/#%D0%92%D0%BE%D0%BF%D1%80%D0%BE%D1%81%D1%8B_%D0%B8_%D0%BE%D1%82%D0%B2%D0%B5%D1%82%D1%8B\" >Questions and Answers<\/a><\/li><li class='ez-toc-page-1'><a class=\"ez-toc-link ez-toc-heading-12\" href=\"https:\/\/valintermed.com\/en\/medlibrary\/defect-of-the-endocardial-cushion\/#%D0%A2%D0%B8%D0%BF%D0%B8%D1%87%D0%BD%D1%8B%D0%B5_%D0%BE%D1%88%D0%B8%D0%B1%D0%BA%D0%B8_%D0%B8_%D0%BA%D0%B0%D0%BA_%D0%B8%D1%85_%D0%B8%D0%B7%D0%B1%D0%B5%D0%B6%D0%B0%D1%82%D1%8C\" >Typical mistakes and how to avoid them<\/a><\/li><li class='ez-toc-page-1'><a class=\"ez-toc-link ez-toc-heading-13\" href=\"https:\/\/valintermed.com\/en\/medlibrary\/defect-of-the-endocardial-cushion\/#%D0%97%D0%B0%D0%BA%D0%BB%D1%8E%D1%87%D0%B5%D0%BD%D0%B8%D0%B5\" >Conclusion<\/a><\/li><\/ul><\/nav><\/div>\n<h2><span class=\"ez-toc-section\" id=\"%D0%9A%D0%BB%D0%B0%D1%81%D1%81%D0%B8%D1%84%D0%B8%D0%BA%D0%B0%D1%86%D0%B8%D1%8F_%D0%B7%D0%B0%D0%B1%D0%BE%D0%BB%D0%B5%D0%B2%D0%B0%D0%BD%D0%B8%D1%8F_%D0%BF%D0%BE_%D0%9C%D0%9A%D0%91\"><\/span>Classification of the disease according to ICD<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>(0\u20131 month):** A complete defect manifests acutely: shortness of breath, cyanosis, refusal to breastfeed, tachycardia &gt;160 beats\/min. Without surgery, survival up to 6 months is less than 30%. <strong>A partial defect at this age is often asymptomatic or masked as \"weakness.\"<\/strong> Infants (1\u201312 months):** This is a \"critical period\" for the endocardial cushion defect. By 3\u20134 months, pulmonary hypertension develops, growth slows down, and hepatomegaly appears. In children with partial endocardial cushion defect \u2014 moderate shortness of breath during feeding, but weight is gained, albeit slower than normal.<br \/>\nPreschool age (1\u20135 years):** If surgery is not performed, the child develops a \"cardiac hump,\" constant fatigue, and limited physical activity. Some experience recurrent bronchitis due to congestion in the lungs. <\/p>\n<ul>\n<li>defect of the interatrial septum in the area of the oval window;<\/li>\n<li>defect of the interventricular septum in the area of the membranous septum;<\/li>\n<li>common atrioventricular valve instead of separate mitral and tricuspid valves.<\/li>\n<\/ul>\n<p>This combination makes AVSD one of the most severe congenital heart defects in newborns. Code Q21.2 is used for both diagnosis and health statistics, but when completing medical documentation, it is mandatory to specify the form of the defect \u2014 partial or complete \u2014 in the conclusion text.<\/p>\n<h2><span class=\"ez-toc-section\" id=\"%D0%98%D1%81%D1%82%D0%BE%D1%80%D0%B8%D1%8F_%D0%B7%D0%B0%D0%B1%D0%BE%D0%BB%D0%B5%D0%B2%D0%B0%D0%BD%D0%B8%D1%8F_%D0%B8_%D0%B8%D0%BD%D1%82%D0%B5%D1%80%D0%B5%D1%81%D0%BD%D1%8B%D0%B5_%D0%B8%D1%81%D1%82%D0%BE%D1%80%D0%B8%D1%87%D0%B5%D1%81%D0%BA%D0%B8%D0%B5_%D1%84%D0%B0%D0%BA%D1%82%D1%8B\"><\/span>History of the disease and interesting historical facts<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>The first description of the endocardial cushion defect belongs to the French anatomist Jean Senac back in 1749. He noted the anomaly of the valves and septa in a child with severe heart failure but did not give it a systematic name. Only in the 1930s did the German pathologist Hans Bergman first distinguish this anomaly as an independent group, calling it \"endocardial cushion defect\" \u2014 by analogy with the embryonic structure from which these parts of the heart are formed.<br \/>\nThere is an interesting fact: until the 1950s, it was believed that such a defect was incompatible with life beyond the first months. However, with the advent of cardiac surgery and the introduction of correction methods in the 1960s, the situation changed dramatically. The first successful operation to correct a partial defect was performed in 1962 by American surgeon Alfred Bailey. And in 1968 \u2014 a complete correction of AVSD in a 2-year-old child. This became a turning point: today, survival after surgery reaches 95% with timely intervention.<br \/>\nIt is particularly noteworthy that the endocardial cushion defect has long been associated with Down syndrome \u2014 this defect occurs in almost 40% of children with trisomy 21. This allowed doctors to use it as one of the markers in screening for chromosomal anomalies even before birth.<\/p>\n<h2><span class=\"ez-toc-section\" id=\"%D0%AD%D0%BF%D0%B8%D0%B4%D0%B5%D0%BC%D0%B8%D0%BE%D0%BB%D0%BE%D0%B3%D0%B8%D1%8F_%D1%81%D1%82%D0%B0%D1%82%D0%B8%D1%81%D1%82%D0%B8%D0%BA%D0%B0_%D0%B2%D0%BE%D0%B7%D0%BD%D0%B8%D0%BA%D0%BD%D0%BE%D0%B2%D0%B5%D0%BD%D0%B8%D1%8F_%D0%B7%D0%B0%D0%B1%D0%BE%D0%BB%D0%B5%D0%B2%D0%B0%D0%BD%D0%B8%D1%8F\"><\/span>Epidemiology (statistics of disease occurrence)<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>The endocardial cushion defect occurs in approximately <strong>2\u20133 cases per 10,000 live births.<\/strong>At the same time, the partial form predominates \u2014 about 60\u201365% of all cases, complete \u2014 35\u201340%. Interestingly, in a population with a high proportion of people with Down syndrome, the frequency of AVSD rises to 15\u201320% among all congenital heart defects in such children.<br \/>\nAccording to the National Registry of Congenital Heart Defects (Russia, 2020\u20132024), the endocardial cushion defect accounts for about 4.71% of all congenital heart anomalies in newborns. In regions with developed prenatal diagnostics, the detection rate increases: up to 80% of cases are identified before birth via ultrasound at 18\u201322 weeks; statistics show that:<\/p>\n<ul>\n<li>boys and girls are affected approximately equally (ratio 1:1);<\/li>\n<li>in 10\u201315% of cases, the defect is associated with other anomalies \u2014 for example, coarctation of the aorta or Ebstein's anomaly;<\/li>\n<li>in 5% of cases, there is a family history \u2014 a repeated birth of a child with the same defect in parents without chromosomal anomalies.<\/li>\n<\/ul>\n<p>Important: accurate statistics depend on the availability of prenatal ultrasound and the quality of neonatal screening. In countries with low levels of medical care, the defect often remains undetected until severe decompensation occurs.<\/p>\n<h2><span class=\"ez-toc-section\" id=\"%D0%93%D0%B5%D0%BD%D0%B5%D1%82%D0%B8%D1%87%D0%B5%D1%81%D0%BA%D0%B0%D1%8F_%D0%BF%D1%80%D0%B5%D0%B4%D1%80%D0%B0%D1%81%D0%BF%D0%BE%D0%BB%D0%BE%D0%B6%D0%B5%D0%BD%D0%BD%D0%BE%D1%81%D1%82%D1%8C_%D0%BA_%D0%B4%D0%B0%D0%BD%D0%BD%D0%BE%D0%BC%D1%83_%D0%B7%D0%B0%D0%B1%D0%BE%D0%BB%D0%B5%D0%B2%D0%B0%D0%BD%D0%B8%D1%8E_%D0%B2%D0%BE%D0%B2%D0%BB%D0%B5%D1%87%D1%91%D0%BD%D0%BD%D1%8B%D0%B5_%D0%B3%D0%B5%D0%BD%D1%8B_%D0%B8_%D0%BC%D1%83%D1%82%D0%B0%D1%86%D0%B8%D0%B8\"><\/span>Genetic predisposition to the disease (involved genes and mutations)<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>The genetic basis of the endocardial cushion defect is multifaceted. The most studied association is with trisomy 21 (Down syndrome). In such patients, the risk of congenital heart defects increases by 2000 times compared to the general population. This is related to the disruption of gene expression on chromosome 21, in particular <strong>COL6A1<\/strong>, <strong>DSCAM<\/strong> and <strong>ETS2<\/strong>, which are involved in the formation of the endocardial cushion during the embryogenesis stage (weeks 4\u20137 of pregnancy).<br \/>\nBut there are also other genetic reasons:<\/p>\n<ul>\n<li><strong>CRELD1<\/strong> \u2014 mutations of this gene are associated with isolated endocardial cushion defect without chromosomal abnormalities. CRELD1 encodes a protein that regulates cell adhesion in the endocardial cushion;<\/li>\n<li><strong>TFAP2B<\/strong> \u2014 mutations lead to impaired differentiation of valve structures and may be associated with defects of the interventricular septum;<\/li>\n<li><strong>GATA4<\/strong> and <strong>NKX2-5<\/strong> \u2014 transcription factors that are critically important for the formation of the atrioventricular connection. Their mutations are rare but cause severe forms of endocardial cushion defect.<\/li>\n<\/ul>\n<p>If a child is found to have an endocardial cushion defect without signs of Down syndrome, genetic testing is recommended: FISH or massive parallel sequencing (MPS) on a panel of genes associated with congenital heart defects. This is especially important in the presence of a family history \u2014 as in 2\u20133% cases the defect is inherited in an autosomal dominant manner.<\/p>\n<h2><span class=\"ez-toc-section\" id=\"%D0%A4%D0%B0%D0%BA%D1%82%D0%BE%D1%80%D1%8B_%D1%80%D0%B8%D1%81%D0%BA%D0%B0_%D0%B2%D0%BE%D0%B7%D0%BD%D0%B8%D0%BA%D0%BD%D0%BE%D0%B2%D0%B5%D0%BD%D0%B8%D1%8F_%D0%B4%D0%B0%D0%BD%D0%BD%D0%BE%D0%B3%D0%BE_%D0%B7%D0%B0%D0%B1%D0%BE%D0%BB%D0%B5%D0%B2%D0%B0%D0%BD%D0%B8%D1%8F_%D1%84%D0%B8%D0%B7%D0%B8%D1%87%D0%B5%D1%81%D0%BA%D0%B8%D0%B5_%D1%85%D0%B8%D0%BC%D0%B8%D1%87%D0%B5%D1%81%D0%BA%D0%B8%D0%B5_%D1%84%D0%B0%D0%BA%D1%82%D0%BE%D1%80%D1%8B_%D1%80%D0%B8%D1%81%D0%BA%D0%B0_%D0%B4%D1%80%D1%83%D0%B3%D0%B8%D0%B5_%D0%B2%D0%BE%D0%B7%D0%BC%D0%BE%D0%B6%D0%BD%D1%8B%D0%B5_%D1%81%D0%B2%D1%8F%D0%B7%D0%B0%D0%BD%D0%BD%D1%8B%D0%B5_%D1%81_%D0%B4%D0%B0%D0%BD%D0%BD%D1%8B%D0%BC_%D0%B7%D0%B0%D0%B1%D0%BE%D0%BB%D0%B5%D0%B2%D0%B0%D0%BD%D0%B8%D0%B5%D0%BC\"><\/span>Risk factors for the occurrence of this disease (physical and chemical risk factors, other possible associations with this disease)<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>Although most cases of endocardial cushion defect have a genetic nature, there are also external factors that increase the risk of its formation. They act during the organogenesis period \u2014 from the 3rd to the 8th week of pregnancy, when the structures of the heart are laid down.<br \/>\nPhysical factors include:<\/p><script data-noptimize=\"\" data-wpfc-render=\"false\">\nfpm_start( \"true\" );\n<\/script>\n\n<ul>\n<li><strong>Ionizing radiation<\/strong> \u2014 X-ray examination of the mother in the first trimester (especially at a dose &gt;100 mGy) increases the risk by 1.5\u20132 times <\/li>\n<li><strong>High body temperature<\/strong> \u2014 fever above 38.5\u00b0C in the first 6 weeks of pregnancy is associated with an increased frequency of congenital heart defects, including CHD.<\/li>\n<\/ul>\n<p>Chemical factors:<\/p>\n<ul>\n<li><strong>Lithium<\/strong> \u2014 a drug used for bipolar disorder increases the risk of CHD by 5\u20137 times when taken in the first trimester;<\/li>\n<p> Alcohol \u2014 regular consumption of more than 30 g of pure ethanol per day in early pregnancy increases the likelihood of CHD by 30%;<\/p>\n<li><strong>Retinoids<\/&gt;><br \/>\n<\/strong><\/li>\n<\/ul>\n<p>It is also worth noting:<\/p>\n<ul>\n<li>maternal diabetes (especially uncontrolled) \u2014 the risk is 3\u20134 times higher;<\/li>\n<li>epilepsy in the mother + taking valproate \u2014 the risk of CHD increases 6 times;<\/li>\n<li>infections in the first trimester (rubella, cytomegalovirus) \u2014 an indirect link through inflammatory reactions in embryonic tissue.<\/li>\n<\/ul>\n<p>If you are faced with the task of prevention \u2014 the main advice: start preparing for pregnancy at least 3 months in advance. Give up alcohol, check your blood glucose level, consult a geneticist if there is a family history.<\/p>\n<h2><span class=\"ez-toc-section\" id=\"%D0%94%D0%B8%D0%B0%D0%B3%D0%BD%D0%BE%D1%81%D1%82%D0%B8%D0%BA%D0%B0_%D0%B4%D0%B0%D0%BD%D0%BD%D0%BE%D0%B3%D0%BE_%D0%B7%D0%B0%D0%B1%D0%BE%D0%BB%D0%B5%D0%B2%D0%B0%D0%BD%D0%B8%D1%8F_%D0%9E%D1%81%D0%BD%D0%BE%D0%B2%D0%BD%D1%8B%D0%B5_%D1%81%D0%B8%D0%BC%D0%BF%D1%82%D0%BE%D0%BC%D1%8B_%D0%9B%D0%B0%D0%B1%D0%BE%D1%80%D0%B0%D1%82%D0%BE%D1%80%D0%BD%D1%8B%D0%B5_%D0%A0%D0%B0%D0%B4%D0%B8%D0%BE%D0%BB%D0%BE%D0%B3%D0%B8%D1%87%D0%B5%D1%81%D0%BA%D0%B8%D0%B5_%D0%BE%D0%B1%D1%81%D0%BB%D0%B5%D0%B4%D0%BE%D0%B2%D0%B0%D0%BD%D0%B8%D1%8F_%D0%94%D1%80%D1%83%D0%B3%D0%B8%D0%B5_%D0%B2%D0%B8%D0%B4%D1%8B_%D0%B4%D0%B8%D0%B0%D0%B3%D0%BD%D0%BE%D1%81%D1%82%D0%B8%D0%BA%D0%B8_%D0%B7%D0%B0%D0%B1%D0%BE%D0%BB%D0%B5%D0%B2%D0%B0%D0%BD%D0%B8%D1%8F_%D0%94%D0%B8%D1%84%D1%84%D0%B5%D1%80%D0%B5%D0%BD%D1%86%D0%B8%D0%B0%D0%BB%D1%8C%D0%BD%D1%8B%D0%B9_%D0%B4%D0%B8%D0%B0%D0%B3%D0%BD%D0%BE%D0%B7\"><\/span>Diagnosis of this disease (Main symptoms, Laboratory Radiological examinations, Other types of disease diagnosis, Differential diagnosis)<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>Diagnosis begins even before birth. During the ultrasound at 18\u201322 weeks, the doctor pays attention to:<\/p>\n<ul>\n<li>enlarged sizes of the right heart chambers;<\/li>\n<li>unusual shape of the atrioventricular connection \u2014 \"cross-shaped\" appearance on a four-chamber view;<\/li>\n<li>absence of a clear boundary between the mitral and tricuspid valves;<\/li>\n<li>wide defect of the interatrial septum in the area of the oval window.<\/li>\n<\/ul>\n<p>After birth, the clinical picture depends on the form of the defect:<\/p>\n<ul>\n<li><strong>Partial defect<\/strong>: moderate shortness of breath during feeding, growth retardation, heart murmur (systolic, localized in 3\u20134 MCB on the left), blood oxygen saturation (SpO\u2082 92\u201395% at rest).<\/li>\n<li><strong>Complete defect<\/strong>: severe heart failure in the first 2\u20133 weeks of life \u2014 tachypnea (&gt;60 per minute), sweating during feeding, cyanosis, hepatomegaly, arterial hypotension.<\/li>\n<\/ul>\n<p>Mandatory diagnostic methods:<\/p>\n<table border=\"1\" cellpadding=\"5\" cellspacing=\"0\" style=\"width:100%;border-collapse:collapse\">\n<tr>\n<th>Method<\/th>\n<th>What it reveals<\/th>\n<th>Features of interpretation<\/th>\n<\/tr>\n<tr>\n<td><strong>Echocardiography (TTE\/TPE)<\/strong><\/td>\n<td>Size and location of defects, structure of valves, degree of shunting, pressure in the pulmonary artery<\/td>\n<td>Gold standard. In PDEP \u2014 \"single valve\" with a common fibrous ring, three leaflets (anterior, posterior, septal), but without clear separation into mitral and tricuspid.<\/td>\n<\/tr>\n<tr>\n<td><strong>ECG<\/strong><\/td>\n<td>Right axis deviation, signs of right ventricular hypertrophy, right bundle branch block<\/td>\n<td>In CDEP \u2014 left axis, signs of left ventricular hypertrophy (due to volume overload).<\/td>\n<\/tr>\n<tr>\n<td><strong>Chest X-ray<\/strong><\/td>\n<td>Heart enlargement, increased pulmonary pattern, \"sheep head heart\" in PDEP<\/td>\n<td>In pronounced pulmonary hypertension \u2014 reduction of the pulmonary pattern, \"notch\" on the contour of the pulmonary artery.<\/td>\n<\/tr>\n<tr>\n<td><strong>Cardiac catheterization<\/strong><\/td>\n<td>Accurate measurement of pressure in the chambers, shunt index, determination of the degree of pulmonary hypertension<\/td>\n<td>It is performed before surgery if echocardiography does not provide complete information or if there is suspicion of accompanying anomalies.<\/td>\n<\/tr>\n<\/table>\n<p>Differential diagnosis includes:<\/p>\n<ul>\n<li>Acute interatrial septal defect (for example, in atrial septal defect type ostium secundum) \u2014 here the valves are normal\n <\/li>\n<li>Atrioventricular canal with additional anomalies (for example, with coarctation of the aorta) \u2014 requires extended examination;<\/li>\n<li>Transposition of the great arteries with ventricular septal defect \u2014 differs in vessel positioning on ultrasound.<\/li>\n<\/ul>\n<p>If you see a heart murmur in a child + growth delay \u2014 do not postpone echocardiography. The earlier the diagnosis is made, the higher the chances for successful correction.<\/p>\n<h2><span class=\"ez-toc-section\" id=\"%D0%9B%D0%B5%D1%87%D0%B5%D0%BD%D0%B8%D0%B5_%D0%BE%D0%B1%D1%89%D0%B5%D0%B5_%D0%BB%D0%B5%D1%87%D0%B5%D0%BD%D0%B8%D0%B5_%D1%84%D0%B0%D1%80%D0%BC%D0%B0%D0%BA%D0%BE%D0%BB%D0%BE%D0%B3%D0%B8%D1%87%D0%B5%D1%81%D0%BA%D0%BE%D0%B5_%D0%BB%D0%B5%D1%87%D0%B5%D0%BD%D0%B8%D0%B5_%D1%85%D0%B8%D1%80%D1%83%D1%80%D0%B3%D0%B8%D1%87%D0%B5%D1%81%D0%BA%D0%BE%D0%B5_%D0%BB%D0%B5%D1%87%D0%B5%D0%BD%D0%B8%D0%B5_%D0%B4%D1%80%D1%83%D0%B3%D0%B8%D0%B5_%D0%B2%D0%B8%D0%B4%D1%8B_%D0%BB%D0%B5%D1%87%D0%B5%D0%BD%D0%B8%D1%8F_%D0%BA_%D0%B4%D0%B0%D0%BD%D0%BD%D0%BE%D0%BC%D1%83_%D0%B2%D0%B8%D0%B4%D1%83_%D0%B7%D0%B0%D0%B1%D0%BE%D0%BB%D0%B5%D0%B2%D0%B0%D0%BD%D0%B8%D1%8F\"><\/span>Treatment (general treatment, pharmacological treatment, surgical treatment, other types of treatment for this type of disease)<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>Treatment of endocardial cushion defect is a complex process, where the main goal is not to \"cure,\" but to restore the anatomical integrity of the heart and ensure normal hemodynamics. Pharmacotherapy here is only auxiliary and aimed at stabilizing the condition before surgery.<br \/>\n<strong>General treatment:<\/strong><br \/>\n\u2014 Feeding in small portions, but more frequently (up to 8\u201310 times a day) to avoid fatigue;<br \/>\n\u2014 Weight control \u2014 daily weighings at the same time;<br \/>\n\u2014 Limitation of physical activity in older children<br \/>\n\u2014 Infection prevention \u2014 vaccination according to schedule, avoiding contacts during ARVI.<br \/>\n<strong>Pharmacological treatment:<\/strong><br \/>\nThe goal is to reduce the load on the heart and prevent the progression of heart failure:<\/p>\n<ul>\n<li><strong>Digoxin<\/strong> \u2014 improves myocardial contractility, prescribed for tachycardia and reduced ejection fraction (usually 0.01\u20130.02 mg\/kg\/day in 2 doses);<\/li>\n<li><strong>Furosemide** \u2014 diuretic, reduces blood volume, dose 1\u20132 mg\/kg\/day; <\/strong><\/li>\n<li><strong>ACE inhibitors (e.g., captopril)** \u2014 reduce afterload, dose 0.3\u20130.5 mg\/kg\/day in 2\u20133 doses;<\/strong><\/li>\n<li><strong>Oxygen therapy<\/strong> \u2014 for SpO\u2082 &lt;90% at rest, but only temporarily, to avoid provoking the development of pulmonary hypertension.<\/li>\n<\/ul>\n<p><strong>Surgical treatment \u2014 the only radical method.<\/strong><br \/>\nThe operation is performed depending on the form:<\/p>\n<ul>\n<li><strong>Partial defect<\/strong>: correction at the age of 1\u20132 years. Technique \u2014 closure of the interatrial septal defect with a patch (made of pericardium or synthetic material) + plastic surgery on the mitral valve (strengthening of the leaflets, reduction of the sail leaflet).<\/li>\n<li><strong>Complete defect<\/strong>: surgery at 3\u20136 months, in case of severe decompensation \u2014 at 1\u20132 months. Stages:\n<ol>\n<li>Closure of the interventricular septum with a patch;<\/li>\n<li>Formation of two separate atrioventricular valves from a common one (separation of the anterior leaflet into mitral and tricuspid parts);<\/li>\n<li>Closure of the interatrial septal defect;<\/li>\n<li>Strengthening of the valve rings.<\/li>\n<\/ol>\n<\/li>\n<\/ul>\n<p>Today, the method of \"bivalve reconstruction\" is used \u2014 it preserves the valve's own tissue, which reduces the risk of thromboembolism and the need for anticoagulants later. In rare cases (in very severe valve dysfunction), prosthetics are used, but this is a last resort.<\/p>\n<h2><span class=\"ez-toc-section\" id=\"%D0%A1%D0%BF%D0%B8%D1%81%D0%BE%D0%BA_%D0%BB%D0%B5%D0%BA%D0%B0%D1%80%D1%81%D1%82%D0%B2_%D0%BF%D1%80%D0%B8%D0%BC%D0%B5%D0%BD%D1%8F%D0%B5%D0%BC%D1%8B%D1%85_%D0%B4%D0%BB%D1%8F_%D0%BB%D0%B5%D1%87%D0%B5%D0%BD%D0%B8%D1%8F_%D0%B4%D0%B0%D0%BD%D0%BD%D0%BE%D0%B3%D0%BE_%D0%B7%D0%B0%D0%B1%D0%BE%D0%BB%D0%B5%D0%B2%D0%B0%D0%BD%D0%B8%D1%8F\"><\/span>List of drugs used to treat this disease<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>Below is the current list of medications used in clinical practice for endocardial cushion defect. All prescriptions must be agreed upon with a cardiologist and cardiac surgeon.<\/p>\n<table border=\"1\" cellpadding=\"5\" cellspacing=\"0\" style=\"width:100%;border-collapse:collapse\">\n<tr>\n<th>Preparation<\/th>\n<th>Group&lt;\/th &lt;thPurpose of use<\/th>\n<th>Standard dose (children)<\/th>\n<th>Features<\/th>\n<\/tr>\n<tr>\n<td>Digoxin<\/td>\n<td>Glycosides<\/td>\n<td>Increased contractility, heart rate control<\/&gt;\n>0.01\u20130.02 mg\/kg\/day in 2 doses<\/td>\n<td>Blood level monitoring is mandatory (therapeutic range 0.8\u20132.0 ng\/ml) <\/td>\n<\/tr>\n<tr>\n<td>Furosemide<\/td>\n<td>Loop diuretic<\/td>\n<td>Decreased CO volume, reduced congestion<\/td>\n<td>1\u20132 mg\/kg\/day in 1\u20132 doses<\/td>\n<td>With prolonged use \u2014 monitoring of potassium and magnesium<\/td>\n<\/tr>\n<tr>\n<td>Captopril <\/td>\n<td>ACE inhibitors<\/td>\n<td>Decreased post-load, slowed remodeling<\/td>\n<p> \u20130.5 mg\/kg\/day in 2\u20133 doses<\/p>\n<td>Start with 0.1 mg\/kg, gradually increase<\/td>\n<\/tr>\n<tr>\n<td>Spironolactone<\/td>\n<td>Potassium-sparing diuretic<\/td>\n<td>Protection against hypokalemia, antifibrotic effect<\/td>\n<td>1\u20132 mg\/kg\/day<\/td>\n<td>Often combined with furosemide<\/td>\n<\/tr>\n<p> &lt;tr <\/p>\n<td>Acetylsalicylic acid<\/td>\n<td>Antiplatelet<\/td>\n<td>Prevention of thrombosis after surgery (in valve reconstruction)<\/td>\n<td>3\u20135 mg\/kg\/day<\/td>\n<td>Appointed for 3\u20136 months after surgery<\/td>\n<\/table>\n<p>Important: anticoagulants (arin, rivaroxaban) are used only for valve prosthetics. They are not needed for reconstructive valve surgery \u2014 this is a big plus for the patient's quality of life.<\/p>\n<h2><span class=\"ez-toc-section\" id=\"%D0%9C%D0%BE%D0%BD%D0%B8%D1%82%D0%BE%D1%80%D0%B8%D0%BD%D0%B3_%D0%B7%D0%B0%D0%B1%D0%BE%D0%BB%D0%B5%D0%B2%D0%B0%D0%BD%D0%B8%D1%8F_%D0%BA%D0%BE%D0%BD%D1%82%D1%80%D0%BE%D0%BB%D1%8C%D0%BD%D1%8B%D0%B5_%D1%8D%D1%82%D0%B0%D0%BF%D1%8B_%D0%BF%D1%80%D0%BE%D0%B3%D0%BD%D0%BE%D0%B7%D1%81%D0%BB%D0%BE%D0%B6%D0%BD%D0%B5%D0%BD%D0%B8%D1%8F\"><\/span>Monitoring of the disease (control stages, prognosis, complications)<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>After surgery, monitoring continues for life \u2014 but the intensity decreases over time. Here are the key stages:<br \/>\n<strong>The first month after surgery:<\/strong><br \/>\n\u2014 Daily examination, ECG, echocardiography on days 3\u20135 and 10\u201314;<br \/>\n\u2014 BNP level control (biomarker of heart failure);<br \/>\n\u2014 Ultrasound of the kidneys and liver \u2014 to assess organ perfusion.<br \/>\n<strong>The first year:<\/strong><br \/>\n\u2014 Echocardiography every 3 months;<br \/>\n\u2014 ECG once every 6 months;<br \/>\n\u2014 Oa \u2014 once every 2\u20133 months.<br \/>\n<strong>From 1 year and older:<\/strong><br \/>\n\u2014 EchoCG once a year;<br \/>\n\u2014 ECG once a year;<br \/>\n\u2014 In the absence of complaints \u2014 examination once every 1\u20132 years.<br \/>\nThe prognosis is favorable with timely surgery:<\/p>\n<ul>\n<li>Survival after PDE correction \u2014 92\u201395% over 10 years;<\/li>\n<li>In 85% patients, heart function remains normal without medications;<\/li>\n<li>The risk of reoperation \u2014 5\u20137% (more often due to insufficiency of the reconstructed mitral valve).<\/li>\n<\/ul>\n<p>Complications that may arise:<\/p>\n<ul>\n<li><strong>Pulmonary hypertension<\/strong> \u2014 if the surgery is performed too late (after 2 years), it may become irreversible;<\/li>\n<li><strong>Mitral valve insufficiency<\/strong> \u2014 the most common complication after reconstruction (10\u201315% cases); <strong>Arrhythmias<\/strong> \u2014 nodal tachycardia, atrial fibrillation (especially in adults after CHD);<\/li>\n<li><strong>Infective endocarditis<\/strong> \u2014 the risk is increased in the first 6 months after surgery and with valve anomalies.<\/li>\n<\/ul>\n<p>If you are a parent of a child with this diagnosis: do not be afraid of the surgery. Modern methods allow for almost normal life. The main thing is not to delay the visit to a specialist.<\/p>\n<h2><span class=\"ez-toc-section\" id=\"%D0%92%D0%BE%D0%B7%D1%80%D0%B0%D1%81%D1%82%D0%BD%D1%8B%D0%B5_%D0%BE%D1%81%D0%BE%D0%B1%D0%B5%D0%BD%D0%BD%D0%BE%D1%81%D1%82%D0%B8_%D0%B7%D0%B0%D0%B1%D0%BE%D0%BB%D0%B5%D0%B2%D0%B0%D0%BD%D0%B8%D1%8F_%D0%BA%D0%B0%D0%BA_%D0%BF%D1%80%D0%BE%D1%82%D0%B5%D0%BA%D0%B0%D0%B5%D1%82_%D0%B2_%D1%80%D0%B0%D0%B7%D0%BD%D1%8B%D1%85_%D0%B2%D0%BE%D0%B7%D1%80%D0%B0%D1%81%D1%82%D0%BD%D1%8B%D1%85_%D0%B3%D1%80%D1%83%D0%BF%D0%BF%D0%B0%D1%85160_%D1%83%D0%B4%D0%BC%D0%B8%D0%BD_%D0%91%D0%B5%D0%B7_%D0%BE%D0%BF%D0%B5%D1%80%D0%B0%D1%86%D0%B8%D0%B8_%D0%B2%D1%8B%D0%B6%D0%B8%D0%B2%D0%B0%D0%B5%D0%BC%D0%BE%D1%81%D1%82%D1%8C_%D0%B4%D0%BE_6_%E2%80%94_%D0%BC%D0%B5%D0%BD%D0%B5%D0%B5_30_%D0%A7%D0%B0%D1%81%D1%82%D0%B8%D1%87%D0%BD%D1%8B%D0%B9_%D0%B4%D0%B5%D1%84%D0%B5%D0%BA%D1%82_%D0%B2_%D1%8D%D1%82%D0%BE%D0%BC_%D0%B2%D0%BE%D0%B7%D1%80%D0%B0%D1%81%D1%82%D0%B5_%D1%87%D0%B0%D1%81%D1%82%D0%BE_%D0%B1%D0%B5%D1%81%D1%81%D0%B8%D0%BC%D0%BF%D1%82%D0%BE%D0%BC%D0%B5%D0%BD_%D0%B8%D0%BB%D0%B8_%D0%BC%D0%B0%D1%81%D0%BA%D0%B8%D1%80%D1%83%D0%B5%D1%82%D1%81%D1%8F_%D0%BF%D0%BE%D0%B4_%C2%AB%D1%81%D0%BB%D0%B0%D0%B1%D0%BE%D1%81%D1%82%D1%8C%C2%BB_%D0%93%D1%80%D1%83%D0%B4%D0%BD%D1%8B%D0%B5_%D0%B4%D0%B5%D1%82%D0%B81%E2%80%9312_%D0%BC%D0%B5%D1%81%D1%8F%D1%86%D0%B5%D0%B2_%D0%AD%D1%82%D0%BE_%C2%AB%D0%BA%D1%80%D0%B8%D1%82%D0%B8%D1%87%D0%B5%D1%81%D0%BA%D0%B8%D0%B9_%D0%BF%D0%B5%D1%80%D0%B8%D0%BE%D0%B4%C2%BB_%D0%B4%D0%BB%D1%8F_%D0%9F%D0%94%D0%95%D0%9F_%D0%9A_3%E2%80%934_%D0%BC%D0%B5%D1%81%D1%8F%D1%86%D0%B0%D0%BC_%D1%80%D0%B0%D0%B7%D0%B2%D0%B8%D0%B2%D0%B0%D0%B5%D1%82%D1%81%D1%8F_%D0%BB%D1%91%D0%B3%D0%BE%D1%87%D0%BD%D0%B0%D1%8F_%D0%B3%D0%B8%D0%BF%D0%B5%D1%80%D1%82%D0%B5%D0%BD%D0%B7%D0%B8%D1%8F_%D1%80%D0%BE%D1%81%D1%82_%D0%B7%D0%B0%D0%BC%D0%B5%D0%B4%D0%BB%D1%8F%D0%B5%D1%82%D1%81%D1%8F_%D0%BF%D0%BE%D1%8F%D0%B2%D0%BB%D1%8F%D0%B5%D1%82%D1%81%D1%8F_%D0%B3%D0%B5%D0%BF%D0%B0%D1%82%D0%BE%D0%BC%D0%B5%D0%B3%D0%B0%D0%BB%D0%B8%D1%8F_%D0%A3_%D0%B4%D0%B5%D1%82%D0%B5%D0%B9_%D1%81_%D0%A7%D0%94%D0%95%D0%9F_%E2%80%94_%D1%83%D0%BC%D0%B5%D1%80%D0%B5%D0%BD%D0%BD%D0%B0%D1%8F_%D0%BE%D0%B4%D1%8B%D1%88%D0%BA%D0%B0_%D0%BF%D1%80%D0%B8_%D0%BA%D0%BE%D1%80%D0%BC%D0%BB%D0%B5%D0%BD%D0%B8%D0%B8_%D0%BD%D0%BE_%D0%B2%D0%B5%D1%81_%D0%BD%D0%B0%D0%B1%D0%B8%D1%80%D0%B0%D0%B5%D1%82%D1%81%D1%8F_%D1%85%D0%BE%D1%82%D1%8F_%D0%B8_%D0%BC%D0%B5%D0%B4%D0%BB%D0%B5%D0%BD%D0%BD%D0%B5%D0%B5_%D0%BD%D0%BE%D1%80%D0%BC%D1%8B_%D0%94%D0%BE%D1%88%D0%BA%D0%BE%D0%BB%D1%8C%D0%BD%D1%8B%D0%B9_%D0%B2%D0%BE%D0%B7%D1%80%D0%B0%D1%81%D1%82_1%E2%80%935_%D0%BB%D0%B5%D1%82_%D0%95%D1%81%D0%BB%D0%B8_%D0%BE%D0%BF%D0%B5%D1%80%D0%B0%D1%86%D0%B8%D1%8F_%D0%BD%D0%B5_%D1%81%D0%B4%D0%B5%D0%BB%D0%B0%D0%BD%D0%B0_%D1%83%D1%91%D0%BD%D0%BA%D0%B0_%D1%84%D0%BE%D1%80%D0%BC%D0%B8%D1%80%D1%83%D0%B5%D1%82%D1%81%D1%8F_%C2%AB%D1%81%D0%B5%D1%80%D0%B4%D0%B5%D1%87%D0%BD%D1%8B%D0%B9_%D0%B3%D0%BE%D1%80%D0%B1%C2%BB_%D0%BF%D0%BE%D1%81%D1%82%D0%BE%D1%8F%D0%BD%D0%BD%D0%B0%D1%8F_%D1%83%D1%81%D1%82%D0%B0%D0%BB%D0%BE%D1%81%D1%82%D1%8C_%D0%BE%D0%B3%D1%80%D0%B0%D0%BD%D0%B8%D1%87%D0%B5%D0%BD%D0%B8%D0%B5_%D1%84%D0%B8%D0%B7%D0%B8%D1%87%D0%B5%D1%81%D0%BA%D0%BE%D0%B9_%D0%B0%D0%BA%D1%82%D0%B8%D0%B2%D0%BD%D0%BE%D1%81%D1%82%D0%B8_%D0%A3_%D0%BD%D0%B5%D0%BA%D0%BE%D1%82%D0%BE%D1%80%D1%8B%D1%85_%E2%80%94_%D1%80%D0%B5%D1%86%D0%B8%D0%B4%D0%B8%D0%B2%D0%B8%D1%80%D1%83%D1%8E%D1%89%D0%B8%D0%B5_%D0%B1%D1%80%D0%BE%D0%BD%D1%85%D0%B8%D1%82%D1%8B_%D0%B8%D0%B7-%D0%B7%D0%B0_%D0%B7%D0%B0%D1%81%D1%82%D0%BE%D1%8F_%D0%B2_%D0%BB%D1%91%D0%B3%D0%BA%D0%B8%D1%85_%D0%A8%D0%BA%D0%BE%D0%BB%D1%8C%D0%BD%D1%8B%D0%B9_%D0%B8_%D0%BF%D0%BE%D0%B4%D1%80%D0%BE%D1%81%D1%82%D0%BA%D0%BE%D0%B2%D1%8B%D0%B96%E2%80%9318_%D0%BB%D0%B5%D1%82_%D0%9F%D1%80%D0%B8_%D0%BD%D0%B5%D0%BE%D0%BF%D0%B5%D1%80%D0%B8%D1%80%D0%BE%D0%B2%D0%B0%D0%BD%D0%BD%D0%BE%D0%BC_%D0%A7%D0%94%D0%95%D0%9F_%E2%80%94_%D0%BF%D0%BE%D1%81%D1%82%D0%B5%D0%BF%D0%B5%D0%BD%D0%BD%D0%BE%D0%B5_%D1%80%D0%B0%D0%B7%D0%B2%D0%B8%D1%82%D0%B8%D0%B5_%D0%BB%D1%91%D0%B3%D0%BE%D1%87%D0%BD%D0%BE%D0%B9_%D0%B3%D0%B8%D0%BF%D0%B5%D1%80%D1%82%D0%B5%D0%BD%D0%B7%D0%B8%D0%B8_%D0%B0%D1%80%D0%B8%D1%82%D0%BC%D0%B8%D0%B9_%D1%81%D0%BD%D0%B8%D0%B6%D0%B5%D0%BD%D0%B8%D0%B5_%D1%82%D0%BE%D0%BB%D0%B5%D1%80%D0%B0%D0%BD%D1%82%D0%BD%D0%BE%D1%81%D1%82%D0%B8_%D0%BA_%D0%BD%D0%B0%D0%B3%D1%80%D1%83%D0%B7%D0%BA%D0%B5_%D0%A3_%D0%B2%D0%B7%D1%80%D0%BE%D1%81%D0%BB%D1%8B%D1%85_%D1%81_%D0%BD%D0%B5%D0%BB%D0%B5%D1%87%D0%B5%D0%BD%D0%BD%D1%8B%D0%BC_%D0%9F%D0%94%D0%95%D0%9F_%E2%80%94_%D0%B2%D1%8B%D1%81%D0%BE%D0%BA%D0%B8%D0%B9_%D1%80%D0%B8%D1%81%D0%BA_%D1%81%D0%B5%D1%80%D0%B4%D0%B5%D1%87%D0%BD%D0%BE%D0%B9_%D0%BD%D0%B5%D0%B4%D0%BE%D1%81%D1%82%D0%B0%D1%82%D0%BE%D1%87%D0%BD%D0%BE%D1%81%D1%82%D0%B8_%D0%B8_%D0%B2%D0%BD%D0%B5%D0%B7%D0%B0%D0%BF%D0%BD%D0%BE%D0%B9_%D1%81%D0%BC%D0%B5%D1%80%D1%82%D0%B8_%D0%BA_20%E2%80%9330_%D0%B3%D0%BE%D0%B4%D0%B0%D0%BC_%D0%92%D0%B7%D1%80%D0%BE%D1%81%D0%BB%D1%8B%D0%B5_18_%D0%BB%D0%B5%D1%82_%D0%92%D1%81%D1%82%D1%80%D0%B5%D1%87%D0%B0%D1%8E%D1%82%D1%81%D1%8F_%D1%80%D0%B5%D0%B4%D0%BA%D0%B8%D0%B5_%D1%81%D0%BB%D1%83%D1%87%D0%B0%D0%B8_%D0%BA%D0%BE%D0%B3%D0%B4%D0%B0_%D0%B4%D0%B5%D1%84%D0%B5%D0%BA%D1%82_%D0%BE%D1%81%D1%82%D0%B0%D0%B2%D0%B0%D0%BB%D1%81%D1%8F_%D0%BD%D0%B5%D0%B7%D0%B0%D0%BC%D0%B5%D1%87%D0%B5%D0%BD%D0%BD%D1%8B%D0%BC_%D0%B4%D0%BE_%D0%B2%D0%B7%D1%80%D0%BE%D1%81%D0%BB%D0%BE%D0%B3%D0%BE_%D0%B2%D0%BE%D0%B7%D1%80%D0%B0%D1%81%D1%82%D0%B0_%D0%A7%D0%B0%D1%89%D0%B5_%E2%80%94_%D1%8D%D1%82%D0%BE_%D1%87%D0%B0%D1%81%D1%82%D0%B8%D1%87%D0%BD%D0%B0%D1%8F_%D1%84%D0%BE%D1%80%D0%BC%D0%B0_%D1%81_%D0%BC%D0%B8%D0%BD%D0%B8%D0%BC%D0%B0%D0%BB%D1%8C%D0%BD%D1%8B%D0%BC%D0%B8_%D1%81%D0%B8%D0%BC%D0%BF%D1%82%D0%BE%D0%BC%D0%B0%D0%BC%D0%B8_%D0%9D%D0%BE_%D0%B4%D0%B0%D0%B6%D0%B5_%D0%BF%D1%80%D0%B8_%D1%85%D0%BE%D1%80%D0%BE%D1%88%D0%B5%D0%BC_%D1%81%D0%B0%D0%BC%D0%BE%D1%87%D1%83%D0%B2%D1%81%D1%82%D0%B2%D0%B8%D0%B8_%D1%83_%D1%82%D0%B0%D0%BA%D0%B8%D1%85_%D0%BF%D0%B0%D1%86%D0%B8%D0%B5%D0%BD%D1%82%D0%BE%D0%B2_%D0%BF%D0%BE%D0%B2%D1%8B%D1%88%D0%B5%D0%BD%D0%B0_%D0%BE%D0%BF%D0%B0%D1%81%D0%BD%D0%BE%D1%81%D1%82%D1%8C\"><\/span>Age-related features of the disease (how it progresses in different age groups)&lt;\/h2\u0444\u0435\u043a\u0442 \u044d\u043d\u0434\u043e\u043a\u0430\u0440\u0434\u0438\u0430\u043b\u044c\u043d\u043e\u0439 \u043f\u043e\u0434\u0443\u0448\u043a\u0438 \u043f\u0440\u043e\u044f\u0432\u043b\u044f\u0435\u0442\u0441\u044f \u043f\u043e-\u0440\u0430\u0437\u043d\u043e\u043c\u0443 \u0432 \u0437\u0430\u0432\u0438\u0441\u0438\u043c\u043e\u0441\u0442\u0438 \u043e\u0442 \u0432\u043e\u0437\u0440\u0430\u0441\u0442\u0430 \u2014 \u044d\u0442\u043e \u0441\u0432\u044f\u0437\u0430\u043d\u043e \u0441 \u0444\u0438\u0437\u0438\u043e\u043b\u043e\u0433\u0438\u0447\u0435\u0441\u043a\u0438\u043c\u0438 \u0438\u0437\u043c\u0435\u043d\u0435\u043d\u0438\u044f\u043c\u0438 \u0432 \u0441\u043e\u0441\u0443\u0434\u0438\u0441\u0442\u043e\u043c \u0440\u0443\u0441\u043b\u0435 \u0438 \u0430\u0434\u0430\u043f\u0442\u0430\u0446\u0438\u043e\u043d\u043d\u044b\u043c\u0438 \u0432\u043e\u0437\u043c\u043e\u0436\u043d\u043e\u0441\u0442\u044f\u043c\u0438 \u043e\u0440\u0433\u0430\u043d\u0438\u0437\u043c\u0430.<br \/>\n<strong>N (0\u20131 month):**<br \/>\nThe complete defect manifests acutely: shortness of breath, cyanosis, refusal to breastfeed, tachycardia &gt;160 beats\/min. Without surgery, survival up to 6 months is less than 30%. A partial defect at this age is often asymptomatic or masked as \"weakness.\"<br \/>\n<\/strong><strong>Infants (1\u201312 months):**<br \/>\nThis is a \"critical period\" for PDA. By 3\u20134 months, pulmonary hypertension develops, growth slows down, and hepatomegaly appears. In children with CHD, there is moderate shortness of breath during feeding, but weight is gained, albeit slower than normal.<br \/>\n<\/strong><strong>Preschool age (1\u20135 years):**<br \/>\nIf surgery is not performed, a \"cardiac hump\" forms, constant fatigue, and limited physical activity. Some experience recurrent bronchitis due to congestion in the lungs.<br \/>\n<\/strong><strong>School and adolescent age (6\u201318 years):**<br \/>\nIn untreated CHD, there is a gradual development of pulmonary hypertension, arrhythmias, and decreased exercise tolerance. In adults with untreated PDA, there is a high risk of heart failure and sudden death by the age of 20\u201330 years.<br \/>\n<\/strong><strong>Adults (18+ years):**<br \/>\nThere are rare cases where the defect remained unnoticed until adulthood. More often, it is a partial form with minimal symptoms. But even with good health, such patients are at increased risk:<br \/>\n<\/strong><span class=\"ez-toc-section-end\"><\/span><\/h2>\n<ul>\n<li>myocardial infarction (due to left ventricular hypertrophy);<\/li>\n<li>thromboembolism (especially in atrial fibrillation);<\/li>\n<li>pregnancy \u2014 high risk of decompensation, so a consultation with a cardiologist is needed before conception.<\/li>\n<\/ul>\n<p>Important even after a successful childhood surgery, adult patients require lifelong monitoring. The heart is not \"healed,\" but \"restructured,\" and its reserves are limited.<\/p>\n<h2><span class=\"ez-toc-section\" id=\"%D0%92%D0%BE%D0%BF%D1%80%D0%BE%D1%81%D1%8B_%D0%B8_%D0%BE%D1%82%D0%B2%D0%B5%D1%82%D1%8B\"><\/span>Questions and Answers<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p><strong>Question 1: Can the endocardial cushion defect be treated without surgery?**<br \/>\nNo. No medication, diet, or physiotherapy will eliminate the anatomical anomaly. Pharmacotherapy helps stabilize the condition but does not solve the problem. Only surgical correction can restore normal hemodynamics. There are experimental catheter occlusion methods for ECD, but they are not yet standardized and are only used in clinical trials.<br \/>\n<\/strong><strong>Question 2: What is the likelihood of having a second child with the same defect?**<br \/>\nIf the first child has an endocardial cushion defect without chromosomal anomalies and without a detected mutation in the CRELD1\/GATA4 genes \u2014 the recurrence risk is 2\u20133%. If the child has Down syndrome \u2014 the risk for the next child with ECD is no higher than the population risk (2\u20133 in 10,000), but the overall risk of congenital heart defects increases to 10%. A consultation with a geneticist is mandatory before planning the next pregnancy.<br \/>\n<\/strong><strong>Question 3: Can a child with a repaired defect participate in sports?**<br \/>\nYes, but with reservations. After successful correction in childhood, most children lead an active lifestyle. Swimming, athletics, and cycling are allowed. Contact sports (football, hockey) and extreme loads (weightlifting, skydiving) are contraindicated. Before starting activities \u2014 a mandatory conclusion from a cardiologist with an EchoCG and a stress test.<br \/>\n<\/strong><strong>Question 4: Why do children with Down syndrome more often have this defect?**<br \/>\nBecause chromosome 21 contains genes that regulate the migration and differentiation of cells in the endocardial cushion during the 4th to 7th week of embryogenesis. An excess of copies of these genes disrupts the balance of signaling pathways (Wnt, Notch, BMP), leading to incomplete separation of the atrioventricular canal. This is not a \"coincidence,\" but a consequence of the genetic program.<br \/>\n<\/strong><strong>Question 5: What to do if the defect is discovered only at 15 years old?**<br \/>\nDo not panic. First, conduct a complete examination: EchoCG, CT\/MRI of the heart, catheterization (if there are signs of pulmonary hypertension), genetic analysis. Then \u2014 a joint decision by the cardiologist, cardiac surgeon, and patient. Surgery in adolescence is technically more complex, but possible. In some cases, valve reconstruction without septal replacement is sufficient. The main thing is not to wait until irreversible pulmonary hypertension develops.<\/p>\n<h2><span class=\"ez-toc-section\" id=\"%D0%A2%D0%B8%D0%BF%D0%B8%D1%87%D0%BD%D1%8B%D0%B5_%D0%BE%D1%88%D0%B8%D0%B1%D0%BA%D0%B8_%D0%B8_%D0%BA%D0%B0%D0%BA_%D0%B8%D1%85_%D0%B8%D0%B7%D0%B1%D0%B5%D0%B6%D0%B0%D1%82%D1%8C\"><\/span>Typical mistakes and how to avoid them<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>1. <\/strong><strong>\"Let's wait until the child grows up.\"<\/strong><br \/>\nMistake: delaying surgery for PDA leads to irreversible pulmonary hypertension. By the age of 2, the chances of successful correction decrease by 20\u201330%.<br \/>\n<strong>How to avoid:<\/strong> If the diagnosis is made before 3 months, surgery should be planned before 6 months.<br \/>\n2. <strong>To think that \"a heart murmur is normal in children\"**<br \/>\nMistake: not all murmurs are harmless. The murmur in PDA is systolic, localized in the 3rd-4th left intercostal space, and increases with physical exertion.<br \/>\n<\/strong><strong>How to avoid:<\/strong> In case of any murmur \u2014 echocardiography within 1 week.<br \/>\n3. <strong>Prescribing antibiotics \"just in case\" for ARVI**<br \/>\nMistake: prophylaxis of endocarditis with antibiotics is not required for reconstructed valves without prostheses. The excessive use of antibiotics leads to dysbiosis and resistance.<br \/>\n<\/strong><strong>How to avoid:<\/strong> Antibiotic prophylaxis only for invasive procedures (tooth extraction, cystoscopy) and only on the recommendation of a cardiologist.<br \/>\n4. <strong>Ignoring genetic counseling after the birth of a child with PDEP**<br \/>\nMistake: you may miss the opportunity to identify a hereditary form, which affects planning the next pregnancy.<br \/>\n<\/strong><strong>How to avoid:<\/strong> Genetic testing \u2014 within 1 month after diagnosis.<\/p>\n<h2><span class=\"ez-toc-section\" id=\"%D0%97%D0%B0%D0%BA%D0%BB%D1%8E%D1%87%D0%B5%D0%BD%D0%B8%D0%B5\"><\/span>Conclusion<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>The defect of the endocardial cushion is not a sentence, but a task that modern cardiac surgery solves with high efficiency. The key to success is early diagnosis, a clear understanding of the form of the defect, and timely surgical intervention. The partial form can be corrected at 1\u20132 years, the complete form at 3\u20136 months. After surgery, most children lead a full life: they study, engage in sports, and give birth to healthy children.<br \/>\nThe main thing to remember:<\/p>\n<ul>\n<li>Do not ignore heart murmurs in a child \u2014 this may be the first signal;<\/li>\n<li>Prenatal ultrasound at 18\u201322 weeks \u2014 your best ally;<\/li>\n<li>Genetic counseling is not a luxury, but a necessity;<\/li>\n<li>Surgery is not the end of the road, but the beginning of a new life.<\/li>\n<\/ul>\n<p>If you are faced with this diagnosis \u2014 you are not alone. In Russia, there are more than 20 high-qualification centers that correct hundreds of such cases annually. Trust the specialists, ask questions, and remember: the heart can be complex, but it is capable of amazing things \u2014 especially when handled by professionals.<\/p>\n<div class=\"fpm_end\"><\/div>","protected":false},"excerpt":{"rendered":"<p>Endocardial cushion defect is a congenital heart defect in which the normal formation of structures separating the right and left chambers of the heart is disrupted.<\/p>","protected":false},"author":1,"featured_media":17434,"comment_status":"open","ping_status":"","sticky":false,"template":"","format":"standard","meta":{"footnotes":""},"categories":[298],"tags":[],"class_list":["post-11049","post","type-post","status-publish","format-standard","has-post-thumbnail","hentry","category-medlibrary"],"_links":{"self":[{"href":"https:\/\/valintermed.com\/en\/wp-json\/wp\/v2\/posts\/11049","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/valintermed.com\/en\/wp-json\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/valintermed.com\/en\/wp-json\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/valintermed.com\/en\/wp-json\/wp\/v2\/users\/1"}],"replies":[{"embeddable":true,"href":"https:\/\/valintermed.com\/en\/wp-json\/wp\/v2\/comments?post=11049"}],"version-history":[{"count":2,"href":"https:\/\/valintermed.com\/en\/wp-json\/wp\/v2\/posts\/11049\/revisions"}],"predecessor-version":[{"id":17412,"href":"https:\/\/valintermed.com\/en\/wp-json\/wp\/v2\/posts\/11049\/revisions\/17412"}],"wp:featuredmedia":[{"embeddable":true,"href":"https:\/\/valintermed.com\/en\/wp-json\/wp\/v2\/media\/17434"}],"wp:attachment":[{"href":"https:\/\/valintermed.com\/en\/wp-json\/wp\/v2\/media?parent=11049"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/valintermed.com\/en\/wp-json\/wp\/v2\/categories?post=11049"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/valintermed.com\/en\/wp-json\/wp\/v2\/tags?post=11049"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}