{"id":12465,"date":"2024-10-11T21:11:51","date_gmt":"2024-10-11T19:11:51","guid":{"rendered":"https:\/\/valintermed.com\/?p=12465"},"modified":"2024-10-11T21:11:51","modified_gmt":"2024-10-11T19:11:51","slug":"legochnaya-arteriovenoznaya-malformatsiya-lavm","status":"publish","type":"post","link":"https:\/\/valintermed.com\/en\/medlibrary\/pulmonary-arteriovenous-malformation-of-the-lavm\/","title":{"rendered":"Pulmonary arteriovenous malformation (PAVM)"},"content":{"rendered":"<div class=\"fpm_start\"><\/div>\n<p>Pulmonary arteriovenous malformation (PAVM) is a congenital vascular disorder in which there is an abnormal connection between the pulmonary artery and veins. This results in blood flowing directly from the arterial bed to the venous bed instead of passing through the capillary bed and being enriched with oxygen. As a result, the normal gas exchange function of the lungs is disrupted, which can lead to serious clinical manifestations, including a decrease in the level of oxygen in the blood, the development of pulmonary hypertension and other cardiopulmonary complications. PAVM can be asymptomatic or manifest itself with a variety of symptoms, including dyspnea, torpidity and cyanosis. The pathology can develop at different age periods, but the most pronounced symptoms are often observed in childhood or young age.<\/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\" 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href=\"https:\/\/valintermed.com\/en\/medlibrary\/pulmonary-arteriovenous-malformation-of-the-lavm\/#%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-2\" href=\"https:\/\/valintermed.com\/en\/medlibrary\/pulmonary-arteriovenous-malformation-of-the-lavm\/#%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\" >Epidemiology<\/a><\/li><li class='ez-toc-page-1'><a class=\"ez-toc-link ez-toc-heading-3\" href=\"https:\/\/valintermed.com\/en\/medlibrary\/pulmonary-arteriovenous-malformation-of-the-lavm\/#%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\" >Genetic predisposition to this disease<\/a><\/li><li class='ez-toc-page-1'><a class=\"ez-toc-link ez-toc-heading-4\" href=\"https:\/\/valintermed.com\/en\/medlibrary\/pulmonary-arteriovenous-malformation-of-the-lavm\/#%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\" >Risk factors for the development of this disease<\/a><\/li><li class='ez-toc-page-1'><a class=\"ez-toc-link ez-toc-heading-5\" href=\"https:\/\/valintermed.com\/en\/medlibrary\/pulmonary-arteriovenous-malformation-of-the-lavm\/#%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\" >Diagnosis of 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\/pulmonary-arteriovenous-malformation-of-the-lavm\/#%D0%9B%D0%B5%D1%87%D0%B5%D0%BD%D0%B8%D0%B5\" >Treatment<\/a><\/li><li class='ez-toc-page-1'><a class=\"ez-toc-link ez-toc-heading-7\" href=\"https:\/\/valintermed.com\/en\/medlibrary\/pulmonary-arteriovenous-malformation-of-the-lavm\/#%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 medications used to treat this disease<\/a><\/li><li class='ez-toc-page-1'><a class=\"ez-toc-link ez-toc-heading-8\" href=\"https:\/\/valintermed.com\/en\/medlibrary\/pulmonary-arteriovenous-malformation-of-the-lavm\/#%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\" >Disease monitoring<\/a><\/li><li class='ez-toc-page-1'><a class=\"ez-toc-link ez-toc-heading-9\" href=\"https:\/\/valintermed.com\/en\/medlibrary\/pulmonary-arteriovenous-malformation-of-the-lavm\/#%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\" >Age-related features of the disease<\/a><\/li><li class='ez-toc-page-1'><a class=\"ez-toc-link ez-toc-heading-10\" href=\"https:\/\/valintermed.com\/en\/medlibrary\/pulmonary-arteriovenous-malformation-of-the-lavm\/#%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><\/ul><\/nav><\/div>\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 history of studying pulmonary arteriovenous malformation goes back more than a hundred years. The first mentions of this pathology appeared in the early 20th century, when doctors began to describe clinical cases with similar vascular anomalies. In 1897, British pathologist Alexander Mills first detailed the morphological features of pulmonary arteriovenous malformation, which gave impetus to further research into this condition. In the 1920s and 1930s, a number of researchers studied the clinical manifestations of PAVM, but it was only in the 1960s that active work began to develop diagnostic and treatment methods for this pathology. For a long time, PAVM remained little known, but in recent decades, with the development of visualization technologies such as computed tomography and magnetic resonance imaging, it has become possible to obtain more accurate information about this anomaly.<\/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\"><\/span>Epidemiology<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>Epidemiological studies indicate that pulmonary arteriovenous malformation occurs with a frequency of 1 in 200,000 live births. The total number of cases may vary, as some patients have asymptomatic forms that remain undetected throughout life. PAVM may be isolated or part of syndromes such as Osler-Weber-Rendu syndrome, a constitutional disorder characterized by multiple vascular anomalies. Recent data indicate that the incidence of PAVM among individuals with Osler syndrome is approximately 30-60%. Depending on the region of occurrence, genetic predisposition, and other factors, the incidence may fluctuate, but a clear predisposition is observed in certain racial and ethnic groups.<\/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\"><\/span>Genetic predisposition to this disease<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>Genetic predisposition to pulmonary arteriovenous malformation is becoming an increasingly important topic of research. The main genes associated with the development of this anomaly are ENG (endoglin) and ACVRL1 (activin A receptor type II-like kinase 1), which are involved in vascular remodeling and angiogenesis. Mutations in these genes can lead to abnormal vessel formation, which ultimately leads to the formation of arteriovenous fistulas in the lungs. Genetic studies show that about 15% cases of PAVM have a family history, which suggests a hereditary predisposition. An association has also been found between PAVM and other disorders associated with vascular system abnormalities, such as Ehlers-Danlos syndrome.<\/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\"><\/span>Risk factors for the development of this disease<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>There are several known risk factors associated with pulmonary arteriovenous malformation:<\/p>\n<ul>\n<li><strong>Genetic factors:<\/strong> Family history and the presence of genetic syndromes such as Osler-Weber-Rendu syndrome significantly increase the risk of developing LAVM.<\/li>\n<li><strong>Environmental factors:<\/strong> Prenatal exposure to toxic substances and chemicals may be a potential trigger for the development of vascular anomalies.<\/li>\n<li><strong>Injuries:<\/strong> Trauma to the chest can lead to the formation of an acquired arteriovenous malformation, but this occurs much less frequently.<\/li>\n<li><strong>Pathological conditions:<\/strong> Chronic lung disease and cardiovascular disease may increase the predisposition to the development of arteriovenous malformations.<\/li>\n<\/ul>\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\"><\/span>Diagnosis of this disease<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>Diagnosis of LAVM can be carried out at different levels and includes not only an assessment of symptoms, but also a range of imaging methods and laboratory tests. The main symptoms of the disease can vary, but often include:<\/p>\n<ul>\n<li>Dyspnea<\/li>\n<li>Cyanosis<\/li>\n<li>Fainting and loss of consciousness<\/li>\n<li>Chronic fatigue<\/li>\n<\/ul>\n<p>Laboratory tests may include a complete blood count, blood gas analysis to detect hypoxia, and specific tests for genetic mutations if hereditary forms are suspected. Radiologic tests such as chest x-rays, computed tomography (CT) scans, and magnetic resonance imaging (MRI) can visualize vascular structures and detect abnormalities. Additional imaging techniques such as angiography may be used to more accurately locate arteriovenous fistulas. The differential diagnosis should include other vascular abnormalities and lung diseases such as pulmonary hypertension, tuberculosis, or tumors.<\/p>\n<h2><span class=\"ez-toc-section\" id=\"%D0%9B%D0%B5%D1%87%D0%B5%D0%BD%D0%B8%D0%B5\"><\/span>Treatment<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>Treatment of LAVM can be multifaceted and depends on the clinical presentation, severity of symptoms, and presence of complications. General treatment may include respiratory support, oxygen therapy, and infection prevention. Pharmacological treatment is often aimed at controlling pulmonary hypertension and may include drugs such as selective phosphodiesterase-5 inhibitors. Surgery is the mainstay of therapy, including embolization of vascular anomalies, which reduces arterial blood flow to the malformations and improves oxygenation. In some cases, resection of the affected area of the lung may be necessary. Other treatments may include rehabilitation programs aimed at improving the quality of life and functional capacity of patients.<\/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 medications used to treat this disease<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<ul>\n<li><strong>Selective phosphodiesterase-5 inhibitors:<\/strong> Sildenafil, Tadalafil<\/li>\n<li><strong>Anticoagulants:<\/strong> Warfarin, Rivaroxaban<\/li>\n<li><strong>Medicines to support cardiovascular function:<\/strong> Beta blockers, ACE inhibitors<\/li>\n<\/ul>\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\"><\/span>Disease monitoring<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>Monitoring the condition of a patient with pulmonary arteriovenous malformation involves regular visits to the doctor and monitoring of clinical manifestations. Control stages may include assessment of the level of oxygen in the blood, functional tests of the lungs and regular visual examination of the lungs to track the dynamics of the disease. The prognosis for patients with PAVM largely depends on timely diagnosis and treatment. Without treatment, serious complications are possible, including the development of pulmonary hypertension, heart failure and the risk of thrombosis. Careful monitoring allows for timely detection of changes in the patient&#039;s status and adjustment of treatment measures.<\/p><script data-noptimize=\"\" data-wpfc-render=\"false\">\nfpm_start( \"true\" );\n<\/script>\n\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\"><\/span>Age-related features of the disease<span class=\"ez-toc-section-end\"><\/span><\/h2>\n<p>Pulmonary arteriovenous malformation can present at any age, but symptoms most often begin in childhood or early adulthood. In newborns and infants, PAVM may present with severe symptoms such as respiratory distress and cyanosis. In adolescents and adults, symptoms may be less severe, and many patients may not seek medical attention until serious complications develop. It is important to note that the disease may be more aggressive in younger people, and early diagnosis and treatment are critical to improving prognosis.<\/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<ul>\n<li><strong>What is pulmonary arteriovenous malformation?<\/strong> Pulmonary arteriovenous malformation is a congenital vascular disorder in which there is an abnormal connection between the pulmonary artery and veins, which disrupts the normal gas exchange function of the lungs.<\/li>\n<li><strong>What are the symptoms of pulmonary arteriovenous malformation?<\/strong> Major symptoms may include shortness of breath, cyanosis, chronic fatigue, fainting and loss of consciousness.<\/li>\n<li><strong>What are the diagnostic methods for LAVM?<\/strong> Diagnosis may include x-rays, CT scans, angiography, and laboratory tests such as blood gas analysis.<\/li>\n<li><strong>How is pulmonary arteriovenous malformation treated?<\/strong> Treatment may include pharmacological therapy, surgical correction, and vascular embolization techniques.<\/li>\n<li><strong>What is the prognosis for patients with LAVM?<\/strong> The prognosis depends on the degree and severity of the disease, but timely diagnosis and treatment can significantly improve the quality of life of patients.<\/li>\n<\/ul>\n<div class=\"fpm_end\"><\/div>","protected":false},"excerpt":{"rendered":"<p>Pulmonary arteriovenous malformation (PAVM) is a congenital vascular disorder in which there is an abnormal connection between the pulmonary artery and veins. This results in<\/p>","protected":false},"author":1,"featured_media":21545,"comment_status":"open","ping_status":"","sticky":false,"template":"","format":"standard","meta":{"footnotes":""},"categories":[298],"tags":[],"class_list":["post-12465","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\/12465","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=12465"}],"version-history":[{"count":1,"href":"https:\/\/valintermed.com\/en\/wp-json\/wp\/v2\/posts\/12465\/revisions"}],"predecessor-version":[{"id":14614,"href":"https:\/\/valintermed.com\/en\/wp-json\/wp\/v2\/posts\/12465\/revisions\/14614"}],"wp:featuredmedia":[{"embeddable":true,"href":"https:\/\/valintermed.com\/en\/wp-json\/wp\/v2\/media\/21545"}],"wp:attachment":[{"href":"https:\/\/valintermed.com\/en\/wp-json\/wp\/v2\/media?parent=12465"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/valintermed.com\/en\/wp-json\/wp\/v2\/categories?post=12465"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/valintermed.com\/en\/wp-json\/wp\/v2\/tags?post=12465"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}