{"id":2198,"date":"2025-12-23T16:02:40","date_gmt":"2025-12-23T13:02:40","guid":{"rendered":"https:\/\/wiki.dev.voka.io\/?post_type=diseases_post&#038;p=2198"},"modified":"2025-12-23T16:02:42","modified_gmt":"2025-12-23T13:02:42","slug":"cardiomyopathie-restrictive","status":"publish","type":"diseases_post","link":"https:\/\/wiki.dev.voka.io\/fr\/maladies\/cardiologie\/cardiomyopathie-restrictive\/","title":{"rendered":"Cardiomyopathie restrictive : \u00e9tiologie, pathog\u00e9nie, sympt\u00f4mes, diagnostic, m\u00e9thodes de traitement"},"content":{"rendered":"<p><?xml encoding=\"UTF-8\" ?><\/p>\n<p>La cardiomyopathie restrictive (CMR) est une forme rare de cardiomyopathie primaire ou secondaire, caract\u00e9ris\u00e9e par un trouble du remplissage diastolique du ventricule gauche et\/ou droit, avec une fonction systolique normale ou subnormale et une \u00e9paisseur pari\u00e9tale conserv\u00e9e. Elle se traduit par une rigidit\u00e9 myocardique marqu\u00e9e, entra\u00eenant une \u00e9l\u00e9vation des pressions diastoliques et une insuffisance cardiaque congestive.<\/p>\n<p>Ce tableau n\u00e9cessite un diagnostic diff\u00e9rentiel complexe, car il peut \u00eatre secondaire \u00e0 de multiples \u00e9tiologies, allant des anomalies g\u00e9n\u00e9tiques aux maladies syst\u00e9miques.<\/p>\n<figure class=\"wp-block-image size-large\"><img decoding=\"async\" src=\"https:\/\/storage.googleapis.com\/dev_wiki_voka_io_303011\/articles\/en\/cardiology\/restrictive-cardiomyopathy\/lv-cavity-restrictive-cmp.webp\" alt=\"Cavit\u00e9 du ventricule gauche dans la cardiomyopathie restrictive secondaire \u00e0 un fibro\u00e9lastose endomyocardique\"><figcaption class=\"wp-element-caption\">Cavit\u00e9 du ventricule gauche dans la cardiomyopathie restrictive secondaire \u00e0 un fibro\u00e9lastose endomyocardique \u2013 <a href=\"https:\/\/catalog.voka.io\/en\/models\/d1df170c-51ef-4d23-93b5-0380d03866e5\/7b0d0575-5bd2-4294-a1a7-c720c52b9654\/075ceff0-6f2e-42b4-a838-5397f2edea6b\/2a1626e7-2835-44f1-bef7-d41940a2b834\" target=\"_blank\" rel=\"noreferrer noopener nofollow\">mod\u00e8le 3D<\/a><\/figcaption><\/figure>\n<h2 class=\"wp-block-heading\" id=\"causes-de-la-cardiomyopathie-restrictive\"><strong>Causes de la cardiomyopathie restrictive<\/strong><\/h2>\n<p>La maladie peut \u00eatre idiopathique (primitive) ou secondaire \u00e0 des processus syst\u00e9miques ou infiltratifs.  Les principales causes se r\u00e9partissent en plusieurs groupes :<\/p>\n<ol class=\"wp-block-list\">\n<li><strong>Maladies infiltratives :<\/strong>\n<ul class=\"wp-block-list\">\n<li>Amylose (formes AL et ATTR) \u2013 cause la plus fr\u00e9quente chez l\u2019adulte.<\/li>\n<li>Sarco\u00efdose \u2013 atteinte granulomateuse du myocarde entra\u00eenant rigidit\u00e9 et troubles de conduction.<\/li>\n<\/ul>\n<\/li>\n<li><strong>Maladies de surcharge :<\/strong>\n<ul class=\"wp-block-list\">\n<li>Maladie de Fabry (d\u00e9ficit en \u03b1-galactosidase A).<\/li>\n<li>H\u00e9mochromatose \u2013 d\u00e9p\u00f4ts de fer dans le myocarde aboutissant \u00e0 une fibrose.<\/li>\n<\/ul>\n<\/li>\n<li><strong>L\u00e9sions radio- ou chimio-induites :<\/strong>\n<ul class=\"wp-block-list\">\n<li>Toxicit\u00e9 des anthracyclines (ex. : doxorubicine).<\/li>\n<li>S\u00e9quelles d\u2019irradiation m\u00e9diastinale.<\/li>\n<\/ul>\n<\/li>\n<li><strong>Atteintes endomyocardiques :<\/strong>\n<ul class=\"wp-block-list\">\n<li>Fibrose endomyocardique.<\/li>\n<li><strong>Cardiomyopathie restrictive de L\u00f6ffler<\/strong> (endocardite hyper\u00e9osinophilique) \u2014 l\u00e9sion toxique cardiaque par les \u00e9osinophiles.<\/li>\n<\/ul>\n<\/li>\n<li><strong>Maladies syst\u00e9miques\u00a0:<\/strong>\n<ul class=\"wp-block-list\">\n<li>Scl\u00e9rodermie syst\u00e9mique, lupus \u00e9ryth\u00e9mateux diss\u00e9min\u00e9.<\/li>\n<\/ul>\n<\/li>\n<\/ol>\n<p>Dans certains cas, l\u2019\u00e9tiologie reste inconnue : forme idiopathique.<\/p>\n<h2 class=\"wp-block-heading\" id=\"pathogenie-et-classification\"><strong>Pathog\u00e9nie et classification<\/strong><\/h2>\n<p>Le m\u00e9canisme principal est la diminution de la compliance (distensibilit\u00e9) myocardique avec conservation ou l\u00e9g\u00e8re alt\u00e9ration de la fonction systolique.  Les parois ventriculaires deviennent rigides et ne peuvent plus se rel\u00e2cher correctement en diastole.<\/p>\n<p>Cela d\u00e9clenche une cascade pathologique :<\/p>\n<ol class=\"wp-block-list\">\n<li><strong>\u00c9l\u00e9vation des pressions de remplissage :<\/strong> \u00a0les parois rigides n\u00e9cessitent une pression \u00e9lev\u00e9e pour le remplissage \u2192 congestion veineuse pulmonaire et syst\u00e9mique.<\/li>\n<li><strong>R\u00e9duction du d\u00e9bit cardiaque : <\/strong> volume d\u2019\u00e9jection systolique et d\u00e9bit-minute diminu\u00e9s malgr\u00e9 une fraction d\u2019\u00e9jection souvent conserv\u00e9e. <\/li>\n<li><strong>R\u00f4le des oreillettes : <\/strong> dilatation compensatrice des oreillettes, perte de la contribution auriculaire au remplissage ventriculaire. Cela entra\u00eene leur dilatation marqu\u00e9e dilatation, une r\u00e9duction de la contribution auriculaire au remplissage ventriculaire et une aggravation des sympt\u00f4mes d\u2019insuffisance cardiaque. <\/li>\n<li><strong>Fibrose et remodelage : <\/strong> cons\u00e9quence de la surcharge chronique en pression, favorisant la progression du ph\u00e9notype restrictif.<\/li>\n<li><strong>Arythmies <\/strong> : fibrillation auriculaire fr\u00e9quente, blocs auriculo-ventriculaires, bradycardies (surtout amylose et sarco\u00efdose). Et augmente \u00e9galement le risque de thrombogen\u00e8se.<\/li>\n<\/ol>\n<p>\u00c0 l\u2019issue de la cha\u00eene pathog\u00e9nique se d\u00e9veloppe une insuffisance cardiaque diastolique r\u00e9fractaire \u00e0 bas d\u00e9bit cardiaque, peu r\u00e9pondante au traitement standard et associ\u00e9e \u00e0 un risque \u00e9lev\u00e9 d\u2019\u00e9v\u00e9nements thromboemboliques.<\/p>\n<h3 class=\"wp-block-heading\" id=\"particularites-pathogeniques-selon-l-etiologie\"><strong>Particularit\u00e9s pathog\u00e9niques selon l\u2019\u00e9tiologie<\/strong><\/h3>\n<p>Les m\u00e9canismes l\u00e9sionnels varient selon l\u2019\u00e9tiologie :<\/p>\n<ul class=\"wp-block-list\">\n<li><strong>Amylose :<\/strong>   d\u00e9p\u00f4ts extracellulaires d\u2019amylo\u00efde \u2192 fibrose et troubles de conduction.<\/li>\n<li><strong>Maladie de Fabry :<\/strong> accumulation intracellulaire de glycosphingolipides \u2192 dysfonction myocytaire directe.<\/li>\n<li><strong>Sarco\u00efdose :<\/strong> remplacement du granulome par fibrose \u2192 arythmies.<\/li>\n<\/ul>\n<p id=\"h-\u043a\u043b\u0430\u0441\u0441\u0438\u0444\u0438\u043a\u0430\u0446\u0438\u044f-\u0440\u0435\u0441\u0442\u0440\u0438\u043a\u0442\u0438\u0432\u043d\u043e\u0439-\u043a\u0430\u0440\u0434\u0438\u043e\u043c\u0438\u043e\u043f\u0430\u0442\u0438\u0438\"><strong>Classification de la cardiomyopathie restrictive<\/strong><\/p>\n<figure class=\"wp-block-table table-to-cards\">\n<table class=\"has-fixed-layout\">\n<thead>\n<tr>\n<th class=\"has-text-align-center\" data-align=\"center\"><strong>Type<\/strong><\/th>\n<th class=\"has-text-align-center\" data-align=\"center\"><strong>Description<\/strong><\/th>\n<\/tr>\n<\/thead>\n<tbody>\n<tr>\n<td class=\"has-text-align-center\" data-align=\"center\"><strong>Primitive (idiopathique)<\/strong><\/td>\n<td class=\"has-text-align-center\" data-align=\"center\">Atteinte limit\u00e9e au myocarde, cause inconnue<\/td>\n<\/tr>\n<tr>\n<td class=\"has-text-align-center\" data-align=\"center\"><strong>Infiltrative<\/strong><\/td>\n<td class=\"has-text-align-center\" data-align=\"center\">Accumulation interstitielle de substances pathologiques (ex. : amylo\u00efde)<\/td>\n<\/tr>\n<tr>\n<td class=\"has-text-align-center\" data-align=\"center\"><strong>Maladies de surcharge<\/strong><\/td>\n<td class=\"has-text-align-center\" data-align=\"center\">Accumulation intracellulaire de m\u00e9tabolites (fer, glycog\u00e8ne\u2026)<\/td>\n<\/tr>\n<tr>\n<td class=\"has-text-align-center\" data-align=\"center\"><strong>Endomyocardique<\/strong><\/td>\n<td class=\"has-text-align-center\" data-align=\"center\">Fibrose de l\u2019endocarde (ex. : maladie de L\u00f6ffler)<\/td>\n<\/tr>\n<tr>\n<td class=\"has-text-align-center\" data-align=\"center\"><strong>Radio- et chimio-induite<\/strong><\/td>\n<td class=\"has-text-align-center\" data-align=\"center\">L\u00e9sions secondaires \u00e0 irradiation et\/ou agents cardiotoxiques<\/td>\n<\/tr>\n<\/tbody>\n<\/table>\n<\/figure>\n<h2 class=\"wp-block-heading\" id=\"manifestations-cliniques\"><strong>Manifestations cliniques<\/strong><\/h2>\n<p>Les sympt\u00f4mes sont principalement li\u00e9s \u00e0 la dysfonction diastolique et \u00e0 la congestion veineuse :<\/p>\n<ul class=\"wp-block-list\">\n<li><strong>Dyspn\u00e9e :<\/strong>  d\u2019effort puis de repos.<\/li>\n<li><strong>Orthopn\u00e9e,<\/strong> \u0153d\u00e8me aigu du poumon (congestion pulmonaire).<\/li>\n<li><strong>Syndrome \u0153d\u00e9mateux :<\/strong>turgescence jugulaire, \u0153d\u00e8mes des membres inf\u00e9rieurs, ascite.<\/li>\n<li><strong>Signes de bas d\u00e9bit : <\/strong> fatigue extr\u00eame, faiblesse, hypotension orthostatique, syncopes.<\/li>\n<li><strong>Troubles du rythme : <\/strong>fibrillation auriculaire fr\u00e9quente, aggravant l\u2019h\u00e9modynamique.<\/li>\n<li><strong>Thromboembolies :<\/strong> \u00a0risque \u00e9lev\u00e9 d\u2019AVC par stase dans les oreillettes dilat\u00e9es.<\/li>\n<\/ul>\n<h2 class=\"wp-block-heading\" id=\"diagnostic\"><strong>Diagnostic<\/strong><\/h2>\n<p>Le diagnostic exige une approche multimodale pour exclure le p\u00e9ricardite constrictive et identifier la cause.<\/p>\n<h3 class=\"wp-block-heading\" id=\"examens-biologiques\"><strong>Examens biologiques<\/strong><\/h3>\n<ul class=\"wp-block-list\">\n<li><strong>NT-proBNP \/ BNP : <\/strong> \u00a0\u00e9lev\u00e9s (marqueurs de gravit\u00e9).<\/li>\n<li><strong>Troponines : <\/strong> discr\u00e8tement \u00e9lev\u00e9es dans les formes infiltratives sans isch\u00e9mie.<\/li>\n<li><strong>Bilan \u00e9tiologique :<\/strong> prot\u00e9ine amylo\u00efde A s\u00e9rique, \u00e9lectrophor\u00e8se des prot\u00e9ines immunoglobulines (amylose AL), ferritin\u00e9mie et saturation de la transferrine (h\u00e9mochromatose), activit\u00e9 \u03b1-galactosidase A (Fabry).<\/li>\n<\/ul>\n<h3 class=\"wp-block-heading\" id=\"methodes-instrumentales\"><strong>M\u00e9thodes instrumentales<\/strong><\/h3>\n<p><strong>1. \u00c9chocardiographie<\/strong><strong><br \/><\/strong> \u00a0\u00c0 l\u2019\u00e9chocardiographie, la cardiomyopathie restrictive pr\u00e9sente des signes caract\u00e9ristiques :<\/p>\n<ul class=\"wp-block-list\">\n<li>\u00c9paississement pari\u00e9tal avec cavit\u00e9s ventriculaires de taille normale ou r\u00e9duite.<\/li>\n<li>Dilatation bi-auriculaire marqu\u00e9e.<\/li>\n<li>Remplissage diastolique de type restrictif (rapport E\/A \u00e9lev\u00e9, E\/e\u2019 \u00e9lev\u00e9).<\/li>\n<li>Fraction d\u2019\u00e9jection conserv\u00e9e jusqu\u2019aux stades tardifs.<\/li>\n<li>Signe \u00ab apical sparing \u00bb tr\u00e8s \u00e9vocateur d\u2019amylose cardiaque (strain longitudinal basal alt\u00e9r\u00e9, apex pr\u00e9serv\u00e9).<\/li>\n<\/ul>\n<p><strong>2. \u00c9lectrocardiogramme<\/strong><strong><br \/><\/strong>(ECG) Microvoltage, fibrillation auriculaire, blocs AV, extrasystoles ventriculaires fr\u00e9quents.<\/p>\n<p><strong>3. IRM cardiaque avec rehaussement tardif au gadolinium (LGE)<\/strong><strong><br \/><\/strong>Standard de r\u00e9f\u00e9rence pour la visualisation de l\u2019infiltration et de la fibrose :<\/p>\n<ul class=\"wp-block-list\">\n<li>Rehaussement sous-endocardique circonf\u00e9rentiel au gadolinium \u2014 hautement \u00e9vocateur de l\u2019amylose cardiaque.<\/li>\n<li>Rehaussement focal ou en plaques \u2014 typique de la sarco\u00efdose.<\/li>\n<li>Absence de rehaussement sp\u00e9cifique \u2014 dans la forme idiopathique.<\/li>\n<\/ul>\n<p><strong>4. Cath\u00e9t\u00e9risme cardiaque (h\u00e9modynamique invasive) <\/strong><\/p>\n<p>Permet la mesure directe des pressions intracardiaques.  Il est essentiel pour diff\u00e9rencier la cardiomyopathie restrictive du p\u00e9ricardite constrictive (par l\u2019absence de variation respiratoire significative des pressions intraventriculaires et l\u2019absence d\u2019\u00e9galisation des pressions diastoliques), ce qui est d\u00e9terminant pour le choix de la strat\u00e9gie th\u00e9rapeutique.<\/p>\n<p><strong>5. M\u00e9thodes compl\u00e9mentaires<\/strong><\/p>\n<ul class=\"wp-block-list\">\n<li><strong>Biopsie endomyocardique : <\/strong> proc\u00e9dure associ\u00e9e \u00e0 certains risques, mais permet une v\u00e9rification histologique pr\u00e9cise du caract\u00e8re infiltratif ou inflammatoire des l\u00e9sions.<\/li>\n<li><strong>Scintigraphie osseuse Tc-99m-PYP : <\/strong>m\u00e9thode tr\u00e8s sensible pour le diagnostic de l\u2019amylose cardiaque de type ATTR ;  permet de la diff\u00e9rencier de la forme AL.<\/li>\n<li><strong>TEP au FDG : <\/strong> met en \u00e9vidence l\u2019inflammation active dans la sarco\u00efdose cardiaque.<\/li>\n<li><strong>Scanner cardiaque : <\/strong> indiqu\u00e9 pour exclure une calcification p\u00e9ricardique (p\u00e9ricardite constrictive).<\/li>\n<\/ul>\n<figure class=\"wp-block-image size-large\"><img decoding=\"async\" src=\"https:\/\/storage.googleapis.com\/dev_wiki_voka_io_303011\/articles\/en\/cardiology\/restrictive-cardiomyopathy\/emc-fibrosis-restrictive-cmp.webp\" alt=\"Fibrose endomyocardique responsable d\u2019une cardiomyopathie restrictive\"><figcaption class=\"wp-element-caption\">Fibrose endomyocardique responsable d\u2019une cardiomyopathie restrictive \u2013 <a href=\"https:\/\/catalog.voka.io\/en\/models\/d1df170c-51ef-4d23-93b5-0380d03866e5\/7b0d0575-5bd2-4294-a1a7-c720c52b9654\/075ceff0-6f2e-42b4-a838-5397f2edea6b\/2a1626e7-2835-44f1-bef7-d41940a2b834\" target=\"_blank\" rel=\"noreferrer noopener nofollow\">mod\u00e8le 3D<\/a><\/figcaption><\/figure>\n<div class=\"social-banner-block\">\n<div class=\"social-banner-content\">\n<p class=\"h5-title text-black\">Retrouvez d\u2019autres contenus scientifiquement exacts sur nos m\u00e9dias sociaux<\/p>\n<p><span class=\"social-banner-text text-grey\">Abonnez-vous et ne manquez pas les derni\u00e8res ressources<\/span><\/p>\n<div class=\"social-links-wrapper\"><a class=\"social-icon-link\" href=\"https:\/\/www.facebook.com\/VOKA3DAnatomyAndPathology\/\" target=\"_blank\" rel=\"nofollow noopener\"><img decoding=\"async\" src=\"https:\/\/storage.googleapis.com\/dev_wiki_voka_io_303011\/common\/Social%20Icons\/facebook.svg\" alt=\"social link\"><\/a><a class=\"social-icon-link\" href=\"https:\/\/www.instagram.com\/voka.io\/\" target=\"_blank\" rel=\"nofollow noopener\"><img decoding=\"async\" src=\"https:\/\/storage.googleapis.com\/dev_wiki_voka_io_303011\/common\/Social%20Icons\/insta.svg\" alt=\"social link\"><\/a><a class=\"social-icon-link\" href=\"https:\/\/www.linkedin.com\/company\/voka-io\/posts\/?feedView=all\" target=\"_blank\" rel=\"nofollow noopener\"><img decoding=\"async\" src=\"https:\/\/storage.googleapis.com\/dev_wiki_voka_io_303011\/common\/Social%20Icons\/linkedin.svg\" alt=\"social link\"><\/a><a class=\"social-icon-link\" href=\"https:\/\/www.youtube.com\/@vokaio\" target=\"_blank\" rel=\"nofollow noopener\"><img decoding=\"async\" src=\"https:\/\/storage.googleapis.com\/dev_wiki_voka_io_303011\/common\/Social%20Icons\/youtube.svg\" alt=\"social link\"><\/a><a class=\"social-icon-link\" href=\"https:\/\/www.pinterest.com\/voka3danatomyandpathology\/\" target=\"_blank\" rel=\"nofollow noopener\"><img decoding=\"async\" src=\"https:\/\/storage.googleapis.com\/dev_wiki_voka_io_303011\/common\/Social%20Icons\/pinterest.svg\" alt=\"social link\"><\/a><a class=\"social-icon-link\" href=\"https:\/\/www.tiktok.com\/@voka.io\" target=\"_blank\" rel=\"nofollow noopener\"><img decoding=\"async\" src=\"https:\/\/storage.googleapis.com\/dev_wiki_voka_io_303011\/common\/Social%20Icons\/tiktok.svg\" alt=\"social link\"><\/a><a class=\"social-icon-link\" href=\"https:\/\/discord.gg\/7ejUpq8DRR\" target=\"_blank\" rel=\"nofollow noopener\"><img decoding=\"async\" src=\"https:\/\/storage.googleapis.com\/dev_wiki_voka_io_303011\/common\/Social%20Icons\/discord.svg\" alt=\"social link\"><\/a><\/div>\n<\/div>\n<p><img decoding=\"async\" class=\"social-banner-image\" loading=\"lazy\" src=\"https:\/\/storage.googleapis.com\/dev_wiki_voka_io_303011\/common\/social-media-banner-mobile-image.webp\" alt=\"Banner background\"><\/div>\n<h2 class=\"wp-block-heading\" id=\"traitement-de-la-cardiomyopathie-restrictive\"><strong>Traitement de la cardiomyopathie restrictive<\/strong><\/h2>\n<p>La prise en charge associe un traitement symptomatique et un traitement \u00e9tiologique sp\u00e9cifique.<\/p>\n<h3 class=\"wp-block-heading\" id=\"traitement-medicamenteux\"><strong>Traitement m\u00e9dicamenteux<\/strong><\/h3>\n<ul class=\"wp-block-list\">\n<li><strong>Diur\u00e9tiques de l\u2019anse<\/strong> (furos\u00e9mide, toras\u00e9mide) : traitement symptomatique de l\u2019insuffisance cardiaque congestive R\u00e9duisent la congestion, mais \u00e0 utiliser avec prudence afin de ne pas diminuer excessivement le d\u00e9bit cardiaque.<\/li>\n<li><strong>B\u00eata-bloquants et antagonistes calciques non dihydropyridiniques :<\/strong> indiqu\u00e9s pour le contr\u00f4le de la fr\u00e9quence cardiaque en cas de tachycardie (avec grande prudence dans l\u2019amylose cardiaque).\u00a0\u00a0<\/li>\n<li><strong>Anticoagulants oraux : <\/strong> obligatoires en cas de fibrillation auriculaire pour la pr\u00e9vention des \u00e9v\u00e9nements thromboemboliques.<\/li>\n<\/ul>\n<h3 class=\"wp-block-heading\" id=\"traitement-etiologique-specifique\"><strong>Traitement \u00e9tiologique sp\u00e9cifique<\/strong><\/h3>\n<ul class=\"wp-block-list\">\n<li><strong>Amylose ATTR :<\/strong>\u00a0tafamidis.<\/li>\n<li><strong>Amylose AL : <\/strong> chimioth\u00e9rapie (bort\u00e9zomib, cyclophosphamide), transplantation de moelle osseuse.<\/li>\n<li><strong>Maladie de Fabry :<\/strong> th\u00e9rapie chaperonne (migalastat), th\u00e9rapie de remplacement enzymatique (agalsidase alfa\/b\u00eata).<\/li>\n<li><strong>Sarco\u00efdose : <\/strong> glucocortico\u00efdes (prednisolone) et immunosuppresseurs (m\u00e9thotrexate, azathioprine).<\/li>\n<li><strong>\u00a0H\u00e9mochromatose : <\/strong> ch\u00e9lateurs du fer (d\u00e9f\u00e9roxamine).<\/li>\n<\/ul>\n<h3 class=\"wp-block-heading\" id=\"therapie-chirurgicale\"><strong>Th\u00e9rapie chirurgicale<\/strong><\/h3>\n<ul class=\"wp-block-list\">\n<li><strong>Implantation d\u2019un stimulateur cardiaque :<\/strong> indiqu\u00e9e en cas de bradycardie s\u00e9v\u00e8re et de blocs auriculo-ventriculaires.<\/li>\n<li><strong>Implantation d\u2019un d\u00e9fibrillateur automatique implantable (DAI) :<\/strong> pr\u00e9vention de la mort subite cardiaque (en particulier apr\u00e8s \u00e9pisodes de tachycardie ventriculaire document\u00e9s et dans les formes cicatricielles de sarco\u00efdose).<\/li>\n<li><strong>Transplantation cardiaque : <\/strong> indiqu\u00e9e en cas d\u2019insuffisance cardiaque s\u00e9v\u00e8re r\u00e9fractaire chez les patients jeunes sans atteinte syst\u00e9mique extracardiaque majeure.<\/li>\n<\/ul>\n<div>\n<h2 class=\"faq-title h2-article\" id=\"faq\">FAQ<\/h2>\n<div class=\"faq-section\">\n<div class=\"faq-item faq-answer-hidden\">\n<div class=\"question-block\">\n<div class=\"faq-question\">\n<p class=\" text-black h5-title\">1. Qu\u2019est-ce que la cardiomyopathie restrictive et en quoi diff\u00e8re-t-elle des autres formes ?<\/p>\n<\/div>\n<div class=\"faq-answer text-main-text-color main-text-medium\">La cardiomyopathie restrictive \u00a0est une maladie dans laquelle le myocarde devient rigide et perd sa capacit\u00e9 de relaxation. Par exemple, contrairement \u00e0 la forme dilat\u00e9e, les cavit\u00e9s cardiaques ne sont g\u00e9n\u00e9ralement pas augment\u00e9es de volume et la fonction systolique reste longtemps conserv\u00e9e.<\/div>\n<\/div>\n<div class=\"expand-button-wrapper\"><button class=\"text-accent expand-button\">+<\/button><\/div>\n<\/div>\n<div class=\"faq-item faq-answer-hidden\">\n<div class=\"question-block\">\n<div class=\"faq-question\">\n<p class=\" text-black h5-title\">2. Quels sont les sympt\u00f4mes caract\u00e9ristiques de la cardiomyopathie restrictive ? <\/p>\n<\/div>\n<div class=\"faq-answer text-main-text-color main-text-medium\">Dyspn\u00e9e, fatigue rapide, \u0153d\u00e8mes, turgescence des veines jugulaires, ascite. Ils sont li\u00e9s \u00e0 la congestion veineuse dans les circulations pulmonaire et syst\u00e9mique secondaire \u00e0 la dysfonction diastolique.<\/div>\n<\/div>\n<div class=\"expand-button-wrapper\"><button class=\"text-accent expand-button\">+<\/button><\/div>\n<\/div>\n<div class=\"faq-item faq-answer-hidden\">\n<div class=\"question-block\">\n<div class=\"faq-question\">\n<p class=\" text-black h5-title\">3. La cardiomyopathie restrictive peut-elle \u00eatre h\u00e9r\u00e9ditaire ? <\/p>\n<\/div>\n<div class=\"faq-answer text-main-text-color main-text-medium\">Oui. Oui. Certaines formes, par exemple l\u2019amylose \u00e0 transthyr\u00e9tine de variante g\u00e9n\u00e9tique (ATTRv), sont d\u2019origine h\u00e9r\u00e9ditaire.<\/div>\n<\/div>\n<div class=\"expand-button-wrapper\"><button class=\"text-accent expand-button\">+<\/button><\/div>\n<\/div>\n<div class=\"faq-item faq-answer-hidden\">\n<div class=\"question-block\">\n<div class=\"faq-question\">\n<p class=\" text-black h5-title\">4. Peut-on gu\u00e9rir compl\u00e8tement la maladie ?<\/p>\n<\/div>\n<div class=\"faq-answer text-main-text-color main-text-medium\">Dans la majorit\u00e9 des cas, la maladie est progressive.  Cependant, en cas de d\u00e9tection pr\u00e9coce d\u2019une cause r\u00e9versible (sarco\u00efdose, myocardite hyper\u00e9osinophilique par exemple), une stabilisation ou une am\u00e9lioration est possible.<\/div>\n<\/div>\n<div class=\"expand-button-wrapper\"><button class=\"text-accent expand-button\">+<\/button><\/div>\n<\/div>\n<div class=\"faq-item faq-answer-hidden\">\n<div class=\"question-block\">\n<div class=\"faq-question\">\n<p class=\" text-black h5-title\">5. Comment diagnostique-t-on la cardiomyopathie restrictive ?<\/p>\n<\/div>\n<div class=\"faq-answer text-main-text-color main-text-medium\">Par \u00e9chocardiographie, TDM\/IRM cardiaque, ECG, biomarqueurs (NT-proBNP, troponines), biopsie endomyocardique, scintigraphie et bilan biologique cibl\u00e9 pour identifier l\u2019amylose et les autres \u00e9tiologies.<\/div>\n<\/div>\n<div class=\"expand-button-wrapper\"><button class=\"text-accent expand-button\">+<\/button><\/div>\n<\/div>\n<div class=\"faq-item faq-answer-hidden\">\n<div class=\"question-block\">\n<div class=\"faq-question\">\n<p class=\" text-black h5-title\">Quelles maladies sont le plus souvent \u00e0 l\u2019origine d\u2019une cardiomyopathie restrictive ?<\/p>\n<\/div>\n<div class=\"faq-answer text-main-text-color main-text-medium\">Amylose (ATTR et AL), sarco\u00efdose, h\u00e9mochromatose, maladies syst\u00e9miques du tissu conjonctif, endocardite hyper\u00e9osinophilique, s\u00e9quelles d\u2019irradiation.<\/div>\n<\/div>\n<div class=\"expand-button-wrapper\"><button class=\"text-accent expand-button\">+<\/button><\/div>\n<\/div>\n<div class=\"faq-item faq-answer-hidden\">\n<div class=\"question-block\">\n<div class=\"faq-question\">\n<p class=\" text-black h5-title\">7. Quel traitement est appliqu\u00e9 dans la cardiomyopathie restrictive ?<\/p>\n<\/div>\n<div class=\"faq-answer text-main-text-color main-text-medium\">Traitement de la cause (ex. : chimioth\u00e9rapie dans l\u2019amylose AL), contr\u00f4le symptomatique (diur\u00e9tiques, antiarythmiques), transplantation cardiaque en cas d\u2019insuffisance cardiaque s\u00e9v\u00e8re.<\/div>\n<\/div>\n<div class=\"expand-button-wrapper\"><button class=\"text-accent expand-button\">+<\/button><\/div>\n<\/div>\n<div class=\"faq-item faq-answer-hidden\">\n<div class=\"question-block\">\n<div class=\"faq-question\">\n<p class=\" text-black h5-title\">8. Quel est le pronostic de la cardiomyopathie restrictive ? <\/p>\n<\/div>\n<div class=\"faq-answer text-main-text-color main-text-medium\">\u00a0Le pronostic d\u00e9pend de l\u2019\u00e9tiologie.  L\u2019amylose AL est associ\u00e9e \u00e0 un pronostic d\u00e9favorable, tandis que l\u2019ATTR peut avoir une \u00e9volution plus stable sous traitement cibl\u00e9.<\/div>\n<\/div>\n<div class=\"expand-button-wrapper\"><button class=\"text-accent expand-button\">+<\/button><\/div>\n<\/div>\n<div class=\"faq-item faq-answer-hidden\">\n<div class=\"question-block\">\n<div class=\"faq-question\">\n<p class=\" text-black h5-title\">9. Peut-on pratiquer un sport avec un diagnostic de la cardiomyopathie restrictive ?<\/p>\n<\/div>\n<div class=\"faq-answer text-main-text-color main-text-medium\">L\u2019activit\u00e9 physique doit \u00eatre limit\u00e9e. Seules des charges l\u00e9g\u00e8res sous contr\u00f4le m\u00e9dical sont autoris\u00e9es.<\/div>\n<\/div>\n<div class=\"expand-button-wrapper\"><button class=\"text-accent expand-button\">+<\/button><\/div>\n<\/div>\n<div class=\"faq-item faq-answer-hidden\">\n<div class=\"question-block\">\n<div class=\"faq-question\">\n<p class=\" text-black h5-title\">10. Quand la transplantation cardiaque est-elle indiqu\u00e9e dans la cardiomyopathie restrictive ?<\/p>\n<\/div>\n<div class=\"faq-answer text-main-text-color main-text-medium\">En cas d\u2019insuffisance cardiaque terminale r\u00e9fractaire au traitement, sans atteinte extracardiaque syst\u00e9mique et en l\u2019absence de contre-indications. Elle est particuli\u00e8rement pertinente lorsque le traitement sp\u00e9cifique est inefficace (ex. : amylose AL).<\/div>\n<\/div>\n<div class=\"expand-button-wrapper\"><button class=\"text-accent expand-button\">+<\/button><\/div>\n<\/div>\n<\/div>\n<\/div>\n<div class=\"sources-list-block sources-list-hidden\" id=\"references\">\n<div class=\"sources-list-content\">\n<div class=\"sources-list-title\">\n<p class=\"small-text-bold text-black sources-list-title-text\">R\u00e9f\u00e9rences<\/p>\n<div class=\"sources-expand-button-wrapper-mobile\">\n<div class=\"sources-expand-button\"><svg width=\"32\" height=\"32\" viewbox=\"0 0 32 32\" fill=\"none\" xmlns=\"http:\/\/www.w3.org\/2000\/svg\"><path d=\"M8 12L16 20L24 12\" stroke=\"#8C9AAB\" stroke-width=\"2\" stroke-linecap=\"round\" stroke-linejoin=\"round\"><\/path><\/svg><\/div>\n<\/div>\n<\/div>\n<div class=\"sources-list-items\">\n<div class=\"source-item\">\n<p class=\"main-text-semibold text-black\">1.<\/p>\n<div class=\"source-item-content\">\n<p class=\"main-text-semibold text-black\"><cite>Catalogue VOKA. [Ressource \u00e9lectronique.<\/cite><\/p>\n<p><span class=\"small-text-medium text-grey\">https:\/\/catalog.voka.io\/<\/span><\/div>\n<\/div>\n<div class=\"source-item\">\n<p class=\"main-text-semibold text-black\">2.<\/p>\n<div class=\"source-item-content\">\n<p class=\"main-text-semibold text-black\"><cite>Rapezzi C, Aimo A, Barison A, et al. Restrictive cardiomyopathy: definition and diagnosis. [Cardiomyopathie restrictive : d\u00e9finition et diagnostic] Eur Heart J. 2022 Dec 1;43(45):4679-4693. doi: 10.1093\/eurheartj\/ehac543.<\/cite><\/p>\n<\/div>\n<\/div>\n<div class=\"source-item\">\n<p class=\"main-text-semibold text-black\">3.<\/p>\n<div class=\"source-item-content\">\n<p class=\"main-text-semibold text-black\"><cite>Arbelo E, Protonotarios A, Gimeno JR, et al. 2023 ESC Guidelines for the management of cardiomyopathies (Lignes directrices de l\u2019ESC pour la prise en charge des cardiomyopathies). Eur Heart J. 2023 Oct 1;44(37):3503-3626. doi\u00a0: 10.1093\/eurheartj\/ehad194.<\/cite><\/p>\n<\/div>\n<\/div>\n<div class=\"source-item\">\n<p class=\"main-text-semibold text-black\">4.<\/p>\n<div class=\"source-item-content\">\n<p class=\"main-text-semibold text-black\"><cite>Muchtar E, Blauwet LA, Gertz MA. Restrictive Cardiomyopathy: Genetics, Pathogenesis, Clinical Manifestations, Diagnosis, and Therapy. [Cardiomyopathie restrictive : g\u00e9n\u00e9tique, pathog\u00e9nie, manifestations cliniques, diagnostic et traitement] Circ Res. 2017 Sep 15;121(7):819-837. doi: 10.1161\/CIRCRESAHA.117.310982.<\/cite><\/p>\n<\/div>\n<\/div>\n<div class=\"source-item\">\n<p class=\"main-text-semibold text-black\">5.<\/p>\n<div class=\"source-item-content\">\n<p class=\"main-text-semibold text-black\"><cite>Vio R, Angelini A, Basso C, et al. Hypertrophic Cardiomyopathy and Primary Restrictive Cardiomyopathy: Similarities, Differences and Phenocopies. [Cardiomyopathie hypertrophique et cardiomyopathie restrictive primitive : similitudes, diff\u00e9rences et ph\u00e9nocopies] J Clin Med. 2021 May 1;10(9):1954. doi: 10.3390\/jcm10091954.<\/cite><\/p>\n<\/div>\n<\/div>\n<div class=\"source-item\">\n<p class=\"main-text-semibold text-black\">6.<\/p>\n<div class=\"source-item-content\">\n<p class=\"main-text-semibold text-black\"><cite>Garcia MJ. Constrictive Pericarditis Versus Restrictive Cardiomyopathy? [P\u00e9ricardite constrictive versus cardiomyopathie restrictive ?] J Am Coll Cardiol. 2016 May 3;67(17):2061-76. doi\u00a0: 10.1016\/j.jacc.2016.01.076.<\/cite><\/p>\n<\/div>\n<\/div>\n<div class=\"source-item\">\n<p class=\"main-text-semibold text-black\">7.<\/p>\n<div class=\"source-item-content\">\n<p class=\"main-text-semibold text-black\"><cite>Arbel\u00e1ez-Cort\u00e9s \u00c1, Quintero-Gonz\u00e1lez DC, Cuesta-Astroz Y, et al. Restrictive cardiomyopathy in a patient with systemic sclerosis and Fabry disease: a case-based review. [Cardiomyopathie restrictive chez un patient atteint de scl\u00e9rodermie syst\u00e9mique et de maladie de Fabry : revue bas\u00e9e sur un cas clinique avec revue de la litt\u00e9rature] Rheumatol Int. 2020 Mar;40(3):489-497. doi: 10.1007\/s00296-019-04453-y.<\/cite><\/p>\n<\/div>\n<\/div>\n<\/div>\n<\/div>\n<div class=\"sources-expand-button-wrapper\">\n<div class=\"sources-expand-button\"><svg width=\"32\" height=\"32\" viewbox=\"0 0 32 32\" fill=\"none\" xmlns=\"http:\/\/www.w3.org\/2000\/svg\"><path d=\"M8 12L16 20L24 12\" stroke=\"#8C9AAB\" stroke-width=\"2\" stroke-linecap=\"round\" stroke-linejoin=\"round\"><\/path><\/svg><\/div>\n<\/div>\n<\/div>\n","protected":false},"excerpt":{"rendered":"<p>La cardiomyopathie restrictive (CMR) est une forme rare de cardiomyopathie primaire ou secondaire, caract\u00e9ris\u00e9e par un trouble du remplissage diastolique du ventricule gauche et\/ou droit, avec une fonction systolique normale ou subnormale et une \u00e9paisseur pari\u00e9tale conserv\u00e9e. Elle se traduit par une rigidit\u00e9 myocardique marqu\u00e9e, entra\u00eenant une \u00e9l\u00e9vation des pressions diastoliques et une insuffisance cardiaque [&hellip;]<\/p>\n","protected":false},"author":2,"featured_media":0,"template":"","diseases_category":[230],"class_list":["post-2198","diseases_post","type-diseases_post","status-publish","hentry","diseases_category-cardiologie"],"acf":[],"yoast_head":"<!-- This site is optimized with the Yoast SEO Premium plugin v25.0 (Yoast SEO v26.5) - https:\/\/yoast.com\/wordpress\/plugins\/seo\/ -->\n<title>Cardiomyopathie restrictive : causes, sympt\u00f4mes, diagnostic et traitement<\/title>\n<meta name=\"description\" content=\"Analyse d\u00e9taill\u00e9e de la cardiomyopathie restrictive. \u00c9tiologie, pathog\u00e9nie, crit\u00e8res diagnostiques (ECG, \u00e9chocardiographie), strat\u00e9gie th\u00e9rapeutique m\u00e9dicamenteuse et pronostic.\" \/>\n<meta name=\"robots\" content=\"index, follow, max-snippet:-1, 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