{"id":875,"date":"2025-07-22T17:29:02","date_gmt":"2025-07-22T14:29:02","guid":{"rendered":"https:\/\/wiki.dev.voka.io\/?post_type=diseases_post&#038;p=875"},"modified":"2026-01-14T11:24:09","modified_gmt":"2026-01-14T08:24:09","slug":"miocardiopatia-hipertrofica","status":"publish","type":"diseases_post","link":"https:\/\/wiki.dev.voka.io\/pt\/doencas\/cardiologia\/miocardiopatia-hipertrofica\/","title":{"rendered":"Cardiomiopatia hipertr\u00f3fica: etiologia, patog\u00e9nese, sintomas, diagn\u00f3stico, m\u00e9todos de tratamento"},"content":{"rendered":"<p><?xml encoding=\"UTF-8\" ?><\/p>\n<p>Cardiomiopatia hipertr\u00f3fica (CMH) \u00e9 uma doen\u00e7a mioc\u00e1rdica prim\u00e1ria caracterizada por hipertrofia inexplicada da parede do ventr\u00edculo esquerdo, geralmente assim\u00e9trica, sem dilata\u00e7\u00e3o da cavidade. A doen\u00e7a \u00e9 causada por muta\u00e7\u00f5es em v\u00e1rios genes que codificam prote\u00ednas sarcom\u00e9ricas, levando a anormalidades morfol\u00f3gicas, el\u00e9tricas e hemodin\u00e2micas.<\/p>\n<figure class=\"wp-block-image size-large\"><img decoding=\"async\" src=\"https:\/\/storage.googleapis.com\/dev_wiki_voka_io_303011\/articles\/en\/acquired-heart-diseases\/hypertrophic-cardiomyopathy\/thickened-wall-of-left-ventricle-in-hcm-2.webp\" alt=\"Espessamento da parede do ventr\u00edculo esquerdo na CMH\"><figcaption class=\"wp-element-caption\">Espessamento da parede do ventr\u00edculo esquerdo na CMH \u2014 <a href=\"https:\/\/catalog.voka.io\/en\/models\/d1df170c-51ef-4d23-93b5-0380d03866e5\/7b0d0575-5bd2-4294-a1a7-c720c52b9654\/075ceff0-6f2e-42b4-a838-5397f2edea6b\/11e0b462-cb5b-4424-9c57-d0ba4e15ade6\" target=\"_blank\" rel=\"noreferrer noopener nofollow\">Modelo 3D<\/a><\/figcaption><\/figure>\n<p>A CMH \u00e9 uma das formas heredit\u00e1rias mais comuns de cardiomiopatia, afetando aproximadamente 1 em cada 500 adultos. Os homens s\u00e3o afetados com mais frequ\u00eancia do que as mulheres (aproximadamente 3:2), mas nas mulheres, a doen\u00e7a geralmente \u00e9 diagnosticada em idade mais avan\u00e7ada e \u00e9 mais grave.<\/p>\n<h2 class=\"wp-block-heading\" id=\"etiologia\">Etiologia<\/h2>\n<p>A doen\u00e7a \u00e9 causada por uma altera\u00e7\u00e3o na s\u00edntese e na fun\u00e7\u00e3o das prote\u00ednas contr\u00e1teis do sarc\u00f4mero, mas, em alguns casos, s\u00e3o detectadas formas secund\u00e1rias associadas a dist\u00farbios sist\u00eamicos e metab\u00f3licos.<\/p>\n<p>At\u00e9 60-70% dos casos de cardiomiopatia hipertr\u00f3fica t\u00eam natureza gen\u00e9tica monomutante.<\/p>\n<p><strong>Genes associados ao desenvolvimento da CMH<\/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>Gene<\/strong><\/th>\n<th class=\"has-text-align-center\" data-align=\"center\"><strong>Prote\u00edna<\/strong><\/th>\n<th class=\"has-text-align-center\" data-align=\"center\"><strong><strong>Frequ\u00eancia das muta\u00e7\u00f5es<\/strong><\/strong><\/th>\n<\/tr>\n<\/thead>\n<tbody>\n<tr>\n<td class=\"has-text-align-center\" data-align=\"center\">MYH7<\/td>\n<td class=\"has-text-align-center\" data-align=\"center\">Cadeia pesada da beta miosina<\/td>\n<td class=\"has-text-align-center\" data-align=\"center\">O MYH7 e o MYBPC3 s\u00e3o respons\u00e1veis \u200b\u200bpor cerca de 70% das muta\u00e7\u00f5es<\/td>\n<\/tr>\n<tr>\n<td class=\"has-text-align-center\" data-align=\"center\">MYBPC3<\/td>\n<td class=\"has-text-align-center\" data-align=\"center\">Prote\u00edna C ligante da miosina<\/td>\n<td class=\"has-text-align-center\" data-align=\"center\">O MYH7 e o MYBPC3 s\u00e3o respons\u00e1veis \u200b\u200bpor cerca de 70% das muta\u00e7\u00f5es<\/td>\n<\/tr>\n<tr>\n<td class=\"has-text-align-center\" data-align=\"center\">TNNT2<\/td>\n<td class=\"has-text-align-center\" data-align=\"center\">Troponina \u0422<\/td>\n<td class=\"has-text-align-center\" data-align=\"center\">Aproximadamente 5%<\/td>\n<\/tr>\n<tr>\n<td class=\"has-text-align-center\" data-align=\"center\">TNNI3<\/td>\n<td class=\"has-text-align-center\" data-align=\"center\">Troponina I<\/td>\n<td class=\"has-text-align-center\" data-align=\"center\"><5%<\/td>\n<\/tr>\n<tr>\n<td class=\"has-text-align-center\" data-align=\"center\">TPM1<\/td>\n<td class=\"has-text-align-center\" data-align=\"center\">Tropomiosina <\/td>\n<td class=\"has-text-align-center\" data-align=\"center\"><5%<\/td>\n<\/tr>\n<\/tbody>\n<\/table>\n<\/figure>\n<ul class=\"wp-block-list\">\n<li>A doen\u00e7a \u00e9 de padr\u00e3o de heran\u00e7a autoss\u00f4mica dominante, o que significa que para desenvolv\u00ea-la, basta herdar uma c\u00f3pia do gene alterado de um dos pais. Embora a probabilidade de desenvolver a doen\u00e7a seja alta, a gravidade e a forma das manifesta\u00e7\u00f5es podem variar muito, mesmo dentro da mesma fam\u00edlia.<\/li>\n<li>Em aproximadamente 30% dos casos, a muta\u00e7\u00e3o se manifesta pela primeira vez, sem hist\u00f3rico familiar.<\/li>\n<\/ul>\n<p>Algumas doen\u00e7as podem mimetizar a apresenta\u00e7\u00e3o cl\u00ednica da cardiomiopatia hipertr\u00f3fica, tendo, por\u00e9m, uma origem patogen\u00e9tica diferente. \u00c9 extremamente importante distingui-las da forma prim\u00e1ria (sarcom\u00e9rica), uma vez que o tratamento e o progn\u00f3stico s\u00e3o diferentes.<\/p>\n<p><strong>Variantes com hipertrofia secund\u00e1ria (fen\u00f3tipos da miocardiopatia hipertr\u00f3fica)<\/strong><\/p>\n<figure class=\"wp-block-table table-to-cards\">\n<table>\n<thead>\n<tr>\n<th class=\"has-text-align-center\" data-align=\"center\"><strong>Doen\u00e7a<\/strong><\/th>\n<th class=\"has-text-align-center\" data-align=\"center\"><strong>Mecanismo<\/strong><\/th>\n<th class=\"has-text-align-center\" data-align=\"center\"><strong>Caracter\u00edsticas<\/strong><\/th>\n<\/tr>\n<\/thead>\n<tbody>\n<tr>\n<td class=\"has-text-align-center\" data-align=\"center\">Doen\u00e7a de Fabry<\/td>\n<td class=\"has-text-align-center\" data-align=\"center\">Doen\u00e7a heredit\u00e1ria de dep\u00f3sito lisoss\u00f4mico (defici\u00eancia da enzima \u03b1-galactosidase A)<\/td>\n<td class=\"has-text-align-center\" data-align=\"center\">Sinais de envolvimento sist\u00eamico (angioqueratoma, neuropatia, protein\u00faria)<\/td>\n<\/tr>\n<tr>\n<td class=\"has-text-align-center\" data-align=\"center\">Amiloidose card\u00edaca<\/td>\n<td class=\"has-text-align-center\" data-align=\"center\">Deposi\u00e7\u00e3o de prote\u00ednas (AL, ATTR) no mioc\u00e1rdio<\/td>\n<td class=\"has-text-align-center\" data-align=\"center\">Espessamento da parede, diminui\u00e7\u00e3o da contratilidade, disfun\u00e7\u00e3o diast\u00f3lica<\/td>\n<\/tr>\n<tr>\n<td class=\"has-text-align-center\" data-align=\"center\">Ataxia de Friedreich<\/td>\n<td class=\"has-text-align-center\" data-align=\"center\">Doen\u00e7a autoss\u00f4mica recessiva causada por muta\u00e7\u00f5es no gene FXN (doen\u00e7a mitocondrial)<\/td>\n<td class=\"has-text-align-center\" data-align=\"center\">Neurodegenera\u00e7\u00e3o progressiva (ataxia, fraqueza e atrofia muscular, dist\u00farbios de linguagem, etc.)<\/td>\n<\/tr>\n<tr>\n<td class=\"has-text-align-center\" data-align=\"center\">Glicogenose (ex.: doen\u00e7a de Pompe)<\/td>\n<td class=\"has-text-align-center\" data-align=\"center\">Ac\u00famulo de glicog\u00eanio nos lisossomos celulares, especialmente nos m\u00fasculos e no cora\u00e7\u00e3o<\/td>\n<td class=\"has-text-align-center\" data-align=\"center\">Frequentemente com envolvimento do sistema m\u00fasculo-esquel\u00e9tico<\/td>\n<\/tr>\n<tr>\n<td class=\"has-text-align-center\" data-align=\"center\">Hipertens\u00e3o arterial sist\u00eamica<\/td>\n<td class=\"has-text-align-center\" data-align=\"center\">Hipertrofia mioc\u00e1rdica reativa<\/td>\n<td class=\"has-text-align-center\" data-align=\"center\">Geralmente sim\u00e9trica, com hist\u00f3rico de hipertens\u00e3o<\/td>\n<\/tr>\n<\/tbody>\n<\/table>\n<\/figure>\n<ul class=\"wp-block-list\">\n<li>Mesmo na presen\u00e7a de uma muta\u00e7\u00e3o da prote\u00edna sarcom\u00e9rica  no gene, a gravidade cl\u00ednica e o curso da CMH dependem de fatores adicionais:\n<ul class=\"wp-block-list\">\n<li>Reguladores epigen\u00e9ticos;<\/li>\n<li>Hipertens\u00e3o arterial concomitante;<\/li>\n<li>Atividade f\u00edsica de alta intensidade (especialmente na adolesc\u00eancia);<\/li>\n<li>Sexo e estado hormonal (mulheres s\u00e3o mais propensas a formas obstrutivas, por\u00e9m costumam apresentar uma manifesta\u00e7\u00e3o mais tardia);<\/li>\n<li>Hist\u00f3rico familiar de morte s\u00fabita card\u00edaca.<\/li>\n<\/ul>\n<\/li>\n<\/ul>\n<h2 class=\"wp-block-heading\" id=\"patogenese\">Patog\u00eanese<\/h2>\n<ul class=\"wp-block-list\">\n<li><strong>Disfun\u00e7\u00e3o sarcom\u00e9rica<\/strong><\/li>\n<\/ul>\n<p>As muta\u00e7\u00f5es gen\u00e9ticas (v. etiologia) levam a:<\/p>\n<ul class=\"wp-block-list\">\n<li>Aumento da sensibilidade ao c\u00e1lcio;<\/li>\n<li>Diminui\u00e7\u00e3o da efici\u00eancia contr\u00e1til;<\/li>\n<li>Aumento das necessidades energ\u00e9ticas.<\/li>\n<\/ul>\n<p>Consequ\u00eancias: desenvolve-se a hipertrofia compensat\u00f3ria do mioc\u00e1rdio, principalmente do septo interventricular, especialmente na regi\u00e3o da via de sa\u00edda do ventr\u00edculo esquerdo (VSVE).<\/p>\n<ul class=\"wp-block-list\">\n<li><strong>Hipertrofia e altera\u00e7\u00f5es do relaxamento ventricular (disfun\u00e7\u00e3o diast\u00f3lica)<\/strong><\/li>\n<\/ul>\n<p>O espessamento das paredes leva a:<\/p>\n<ul class=\"wp-block-list\">\n<li>Diminui\u00e7\u00e3o da complac\u00eancia ventricular esquerda;<\/li>\n<li>Deificuldade do enchimento diast\u00f3lico;<\/li>\n<li>Aumento da press\u00e3o diast\u00f3lica.<\/li>\n<\/ul>\n<p>Consequ\u00eancias: desenvolvimento de congest\u00e3o pulmonar, manifestada por falta de ar e outros sintomas de insufici\u00eancia card\u00edaca com fra\u00e7\u00e3o de eje\u00e7\u00e3o preservada.<\/p>\n<ul class=\"wp-block-list\">\n<li><strong>Estreitamento grave da via de sa\u00edda do ventr\u00edculo esquerdo (forma obstrutiva)<\/strong>\n<ul class=\"wp-block-list\">\n<li>Em 60\u201370% dos pacientes, desenvolve-se uma obstru\u00e7\u00e3o da via de sa\u00edda do ventr\u00edculo esquerdo.<\/li>\n<li>Devido ao efeito Venturi durante a s\u00edstole, ocorre: a retra\u00e7\u00e3o da c\u00faspide anterior da valva mitral em dire\u00e7\u00e3o ao septo interventricular (fen\u00f4meno SAM), o agravamento do gradiente de press\u00e3o e o desenvolvimento de regurgita\u00e7\u00e3o mitral.<\/li>\n<\/ul>\n<\/li>\n<\/ul>\n<p>Consequ\u00eancias: aumento da sobrecarga hemodin\u00e2mica, agravando os sintomas e aumentando o risco de arritmias.<\/p>\n<figure class=\"wp-block-image size-large\"><img decoding=\"async\" src=\"https:\/\/storage.googleapis.com\/dev_wiki_voka_io_303011\/articles\/en\/acquired-heart-diseases\/hypertrophic-cardiomyopathy\/obstruction-of-outflow-tract-due-to-thickening-of-interventricular-septum.webp\" alt=\"Obstru\u00e7\u00e3o da via de sa\u00edda do ventr\u00edculo esquerdo devido ao espessamento do septo interventricular\"><figcaption class=\"wp-element-caption\">Obstru\u00e7\u00e3o da via de sa\u00edda do ventr\u00edculo esquerdo devido ao espessamento do septo interventricular \u2014 <a href=\"https:\/\/catalog.voka.io\/en\/models\/d1df170c-51ef-4d23-93b5-0380d03866e5\/7b0d0575-5bd2-4294-a1a7-c720c52b9654\/075ceff0-6f2e-42b4-a838-5397f2edea6b\/11e0b462-cb5b-4424-9c57-d0ba4e15ade6\" target=\"_blank\" rel=\"noreferrer noopener nofollow\">Modelo 3D<\/a><\/figcaption><\/figure>\n<ul class=\"wp-block-list\">\n<li><strong>Isquemia microvascular<\/strong><\/li>\n<\/ul>\n<p>O mioc\u00e1rdio hipertrofiado requer mais oxig\u00eanio, mas:<\/p>\n<ul class=\"wp-block-list\">\n<li>A rede capilar \u00e9 incapaz de compensar o crescimento do tecido;<\/li>\n<li>H\u00e1 um desequil\u00edbrio entre a demanda e a oferta de oxig\u00eanio;<\/li>\n<li>Muitas vezes, observa-se a fibrose de pequenos vasos.<\/li>\n<\/ul>\n<p>Consequ\u00eancias: desenvolve-se a isquemia mioc\u00e1rdica com art\u00e9rias coron\u00e1rias normais, levando \u00e0 dor tor\u00e1cica, \u00e0 fibrose e ao aumento do risco de arritmias.<\/p>\n<ul class=\"wp-block-list\">\n<li><strong>Fibrose e instabilidade el\u00e9trica<\/strong><\/li>\n<\/ul>\n<p>Em resposta \u00e0 isquemia e \u00e0 sobrecarga mec\u00e2nica do mioc\u00e1rdio, desenvolve-se a fibrose intersticial e focal, levando a:<\/p>\n<ul class=\"wp-block-list\">\n<li>Condu\u00e7\u00e3o prejudicada dos impulsos;<\/li>\n<li>Desenvolvimento de arritmias ventriculares;<\/li>\n<li>Aumento do risco de morte s\u00fabita card\u00edaca (MSC).<\/li>\n<\/ul>\n<figure class=\"wp-block-video\" id=\"animacao-3d-cardiomiopatia-hipertrofica\"><video controls><source data-src=\"https:\/\/storage.googleapis.com\/dev_wiki_voka_io_303011\/articles\/en\/acquired-heart-diseases\/hypertrophic-cardiomyopathy\/hypertrophic-cardiomyopathy.webm\" type=\"video\/webm\"><\/source><\/video><figcaption class=\"wp-element-caption\">Anima\u00e7\u00e3o 3D \u2014 cardiomiopatia hipertr\u00f3fica<\/figcaption><\/figure>\n<h2 class=\"wp-block-heading\" id=\"manifestacoes-clinicas\">Manifesta\u00e7\u00f5es cl\u00ednicas<\/h2>\n<ul class=\"wp-block-list\">\n<li>Falta de ar aos esfor\u00e7os, fadiga;<\/li>\n<li>Dor no peito (angina) sem doen\u00e7a arterial coronariana;<\/li>\n<li>S\u00edncope ou pr\u00e9-s\u00edncope (especialmente durante esfor\u00e7o f\u00edsico);<\/li>\n<li>Arritmias ventriculares, fibrila\u00e7\u00e3o atrial;<\/li>\n<li>Morte s\u00fabita \u2014 especialmente em pacientes jovens e atletas que apresentam a forma obstrutiva;<\/li>\n<li>Insufici\u00eancia card\u00edaca: pode se desenvolver tanto com fra\u00e7\u00e3o de eje\u00e7\u00e3o preservada (ICFEP) quanto com sua diminui\u00e7\u00e3o em est\u00e1gios mais avan\u00e7ados. Manifesta-se como <span class=\"glossary-term\" data-title=\"Edema\" data-tooltip=\"O edema \u00e9 uma acumula\u00e7\u00e3o excessiva de l\u00edquido nos espa\u00e7os intercelulares (intersticiais) dos tecidos ou nas cavidades serosas do corpo. Clinicamente, manifesta-se por incha\u00e7o, aumento do volume de um \u00f3rg\u00e3o ou de uma parte do corpo. \" data-link=\"https:\/\/wiki.dev.voka.io\/pt\/glossario\/edema\/\">edema<\/span>, ortopneia, taquicardia e diminui\u00e7\u00e3o da toler\u00e2ncia ao exerc\u00edcio.<\/li>\n<li>Assintom\u00e1tica: em 25 a 30% dos pacientes, a doen\u00e7a \u00e9 detectada por meio da an\u00e1lise do hist\u00f3rico familiar. Apresenta alto risco de complica\u00e7\u00f5es.<\/li>\n<\/ul>\n<h2 class=\"wp-block-heading\" id=\"diagnostico-de-cardiomiopatia-hipertrofica\">Diagn\u00f3stico de cardiomiopatia hipertr\u00f3fica<\/h2>\n<ul class=\"wp-block-list\">\n<li><strong>A ecocardiografia (ecocardiografia transtor\u00e1cica)<\/strong> \u00e9 o m\u00e9todo fundamental do diagn\u00f3stico inicial. Permite:\n<ul class=\"wp-block-list\">\n<li>Avaliar a espessura do mioc\u00e1rdio. O diagn\u00f3stico \u00e9 prov\u00e1vel com uma espessura da parede \u226515 mm em adultos ou \u226513 mm em parentes de primeiro grau com diagn\u00f3stico de CMH confirmado;<\/li>\n<li>Determinar a distribui\u00e7\u00e3o da hipertrofia: assim\u00e9trica, conc\u00eantrica, apical;<\/li>\n<li>Detectar a obstru\u00e7\u00e3o da via de sa\u00edda do ventr\u00edculo esquerdo (gradiente \u226530 mmHg, clinicamente significativo \u226550 mmHg);<\/li>\n<li>Detectar o fen\u00f4meno SAM (retra\u00e7\u00e3o sist\u00f3lica da valva mitral) e a regurgita\u00e7\u00e3o mitral;<\/li>\n<li>Avaliar a fun\u00e7\u00e3o do ventr\u00edculo esquerdo e a presen\u00e7a de disfun\u00e7\u00e3o diast\u00f3lica;<\/li>\n<li>Aferir o tamanho dos \u00e1trios (especialmente o \u00e1trio esquerdo, que indica risco de FA).<\/li>\n<\/ul>\n<\/li>\n<li><strong><span class=\"glossary-term\" data-title=\"RM (Resson\u00e2ncia magn\u00e9tica)\" data-tooltip=\"A resson\u00e2ncia magn\u00e9tica (RM) \u00e9 uma t\u00e9cnica de imagem moderna n\u00e3o invasiva que produz imagens em camadas altamente detalhadas de \u00f3rg\u00e3os e tecidos internos. O m\u00e9todo baseia-se no fen\u00f3meno da resson\u00e2ncia magn\u00e9tica nuclear. A principal vantagem da RM \u00e9 a aus\u00eancia de radia\u00e7\u00e3o ionizante.\" data-link=\"https:\/\/wiki.dev.voka.io\/pt\/glossario\/rm\/\">Resson\u00e2ncia magn\u00e9tica<\/span> do cora\u00e7\u00e3o<\/strong> \u00e9 recomendada se:\n<ul class=\"wp-block-list\">\n<li>A ecocardiografia n\u00e3o permitir avaliar com precis\u00e3o a espessura da parede.<\/li>\n<li>Houver suspeita de cardiomiopatia hipertr\u00f3fica apical ou at\u00edpica.<\/li>\n<li>For necess\u00e1ria a avalia\u00e7\u00e3o da fibrose mioc\u00e1rdica.<\/li>\n<\/ul>\n<\/li>\n<\/ul>\n<p>Revela:<\/p>\n<ul class=\"wp-block-list\">\n<li>Distribui\u00e7\u00e3o e grau de hipertrofia;<\/li>\n<li>\u00c1reas fibrosas est\u00e3o associadas a um risco aumentado de arritmias e morte s\u00fabita card\u00edaca;<\/li>\n<li>Diferencia\u00e7\u00e3o de fenoc\u00f3pias (por exemplo, amiloidose).<\/li>\n<\/ul>\n<ul class=\"wp-block-list\">\n<li><strong>O eletrocardiograma (ECG)<\/strong> \u00e9 inespec\u00edfico, mas altera\u00e7\u00f5es patol\u00f3gicas s\u00e3o detectadas em mais de 90% dos pacientes. Sinais de hipertrofia ventricular esquerda:\n<ul class=\"wp-block-list\">\n<li>Ondas Q at\u00edpicas (nas deriva\u00e7\u00f5es V4\u2013V6, I, aVL) \u2014 podem mimetizar o infarto do mioc\u00e1rdio;<\/li>\n<li>Altera\u00e7\u00f5es do segmento ST e invers\u00e3o da onda T;<\/li>\n<li>Arritmia: fibrila\u00e7\u00e3o atrial, extrass\u00edstoles ventriculares, taquicardia ventricular;<\/li>\n<li>Bloqueios atrioventriculares ou intraventriculares.<\/li>\n<\/ul>\n<\/li>\n<li><strong> Exame Holter. <\/strong>Indica\u00e7\u00f5es:\n<ul class=\"wp-block-list\">\n<li>Suspeita de arritmia (extrass\u00edstole ventricular, taquicardia ventricular, fibrila\u00e7\u00e3o atrial);<\/li>\n<li>Desmaio ou pr\u00e9-s\u00edncope;<\/li>\n<li>Avalia\u00e7\u00e3o da gravidade das arritmias e indica\u00e7\u00f5es para implante de cardioversor desfibrilhador implant\u00e1vel (CDI).<\/li>\n<\/ul>\n<\/li>\n<li><strong>Teste de esfor\u00e7o (esteira ou cicloerg\u00f4metro). <\/strong>\u00c9 realizado para:\n<ul class=\"wp-block-list\">\n<li>Avaliar a toler\u00e2ncia aos exerc\u00edcios;<\/li>\n<li>Detectar a obstru\u00e7\u00e3o induzida da via de sa\u00edda do ventr\u00edculo esquerdo;<\/li>\n<li>Determinar o gradiente durante esfor\u00e7o;<\/li>\n<li>Identificar os sintomas isqu\u00eamicos sem estenose da art\u00e9ria coron\u00e1ria.<\/li>\n<\/ul>\n<\/li>\n<li><strong>Testagem gen\u00e9tica. <\/strong>\u00c9 recomendado:\n<ul class=\"wp-block-list\">\n<li>Para pacientes com diagn\u00f3stico de CMH confirmado (especialmente pacientes jovens ou com hist\u00f3rico familiar de morte s\u00fabita card\u00edaca);<\/li>\n<li>Para o screening de parentes de primeiro grau (filhos, irm\u00e3os, pais);<\/li>\n<li>Se houver suspeita de fenoc\u00f3pias (doen\u00e7a de Fabry, doen\u00e7as mitocondriais, etc.).<\/li>\n<\/ul>\n<\/li>\n<\/ul>\n<p>Os genes testados: MYH7, MYBPC3, TNNT2, TNNI3 e TPM1; s\u00e3o os mais comuns.<\/p>\n<ul class=\"wp-block-list\">\n<li><strong>Marcadores laboratoriais:<\/strong>\n<ul class=\"wp-block-list\">\n<li>NT-proBNP \/ BNP \u2014 valores elevados em casos de sobrecarga de press\u00e3o e disfun\u00e7\u00e3o diast\u00f3lica;<\/li>\n<li>Troponina T\/I \u2014 pode estar moderadamente elevada em caso de isquemia microvascular.<\/li>\n<\/ul>\n<\/li>\n<\/ul>\n<div class=\"social-banner-block\">\n<div class=\"social-banner-content\">\n<p class=\"h5-title text-black\">Encontra mais conte\u00fados cientificamente exactos nas nossas redes sociais<\/p>\n<p><span class=\"social-banner-text text-grey\">Subscreve e n\u00e3o percas os recursos mais recentes<\/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=\"tratamento-da-cardiomiopatia-hipertrofica\">Tratamento da cardiomiopatia hipertr\u00f3fica<\/h2>\n<ol class=\"wp-block-list\">\n<li><strong>Modifica\u00e7\u00e3o do estilo de vida:<\/strong><\/li>\n<\/ol>\n<ul class=\"wp-block-list\">\n<li>Evitar atividades f\u00edsicas intensas e esportes;<\/li>\n<li>Controlar a press\u00e3o arterial e o peso;<\/li>\n<li>Screening de parentes de primeiro grau.<\/li>\n<\/ul>\n<ol start=\"2\" class=\"wp-block-list\">\n<li><strong>Tratamento medicamentoso:<\/strong><\/li>\n<\/ol>\n<ul class=\"wp-block-list\">\n<li>Betabloqueadores (preferencialmente bisoprolol, metoprolol);<\/li>\n<li>Verapamil \u2014 se os betabloqueadores forem contraindicados.<\/li>\n<li>Disopiramida \u2014 como adjuvante na forma obstrutiva.<\/li>\n<li>Mavacamten \u2014 um novo medicamento com efeito comprovado na redu\u00e7\u00e3o do gradiente e na melhora dos sintomas (ESC 2023 e AHA 2020).<\/li>\n<\/ul>\n<ol start=\"3\" class=\"wp-block-list\">\n<li><strong>Terapia cir\u00fargica<\/strong><\/li>\n<\/ol>\n<p>Indica\u00e7\u00f5es:<\/p>\n<ul class=\"wp-block-list\">\n<li>Gradiente da via de sa\u00edda do ventr\u00edculo esquerdo (VSVE) \u226550 mmHg. p. em repouso ou induzido pelo exerc\u00edcio;<\/li>\n<li>Sintomas graves (NYHA III\u2013IV) resistentes ao tratamento com os betabloqueadores, o verapamil ou a disopiramida;<\/li>\n<li>Insufici\u00eancia mitral grave associada ao fen\u00f4meno SAM.<\/li>\n<\/ul>\n<p>Miectomia septal extensa: <\/p>\n<ul class=\"wp-block-list\">\n<li>O padr\u00e3o ouro para cirurgia na cardiomiopatia hipertr\u00f3fica obstrutiva;<\/li>\n<li>\u00c9 realizada por meio de miniesternotomia ou esternotomia completa e, em alguns casos, por meio de minitoracotomia anterior direita;<\/li>\n<li>Uma se\u00e7\u00e3o do septo interventricular hipertrofiado \u00e9 removida, eliminando o gradiente de press\u00e3o;<\/li>\n<li>Se necess\u00e1rio, \u00e9 relizado o reparo da valva mitral ou a ressec\u00e7\u00e3o das cordoalhas secund\u00e1rias;<\/li>\n<li>Poss\u00edveis complica\u00e7\u00f5es: obstru\u00e7\u00f5es, necessidade de implante de CDI, recorr\u00eancia do gradiente.<\/li>\n<\/ul>\n<ol start=\"4\" class=\"wp-block-list\">\n<li><strong>A abla\u00e7\u00e3o septal alco\u00f3lica<\/strong> (interven\u00e7\u00e3o endovascular) \u00e9 normalmente utilizada em pacientes para os quais a cirurgia aberta \u00e9 contraindicada ou apresenta riscos muito elevados.<\/li>\n<\/ol>\n<ul class=\"wp-block-list\">\n<li>Inje\u00e7\u00e3o de etanol na art\u00e9ria perfurante \u2192 infarto local \u2192 redu\u00e7\u00e3o da espessura septal;<\/li>\n<li>Risco de bloqueio atrioventricular completo (at\u00e9 10%) \u2013 \u00e9 necess\u00e1rio estar preparado para o implante de marca-passo;<\/li>\n<li>Resultados menos previs\u00edveis;<\/li>\n<li>Possibilidade de remo\u00e7\u00e3o incompleta da obstru\u00e7\u00e3o.<\/li>\n<\/ul>\n<ol start=\"5\" class=\"wp-block-list\">\n<li><strong>Implanta\u00e7\u00e3o de um cardioversor-desfibrilador (CDI)<\/strong><\/li>\n<\/ol>\n<p>Indica\u00e7\u00f5es:<\/p>\n<ul class=\"wp-block-list\">\n<li>Morte s\u00fabita card\u00edaca pr\u00e9via ou taquicardia ventricular sustentada;<\/li>\n<li>Espessura da parede do ventr\u00edculo esquerdo \u00e9 >30 mm;<\/li>\n<li>Hist\u00f3rico familiar de morte s\u00fabita card\u00edaca;<\/li>\n<li>Desmaio de etiologia desconhecida;<\/li>\n<li>FEVE <50% com doen\u00e7a progressiva.<\/li>\n<\/ul>\n<ol start=\"6\" class=\"wp-block-list\">\n<li><strong>Transplante card\u00edaco<\/strong> em pacientes com insufici\u00eancia card\u00edaca terminal refrat\u00e1ria, apesar do tratamento.<\/li>\n<\/ol>\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. O que \u00e9 a cardiomiopatia hipertr\u00f3fica?<\/p>\n<\/div>\n<div class=\"faq-answer text-main-text-color main-text-medium\">A cardiomiopatia hipertr\u00f3fica (CMH) \u00e9 uma doen\u00e7a na qual o m\u00fasculo card\u00edaco (geralmente a parede entre os ventr\u00edculos) se torna anormalmente espessado. Ao contr\u00e1rio da hipertrofia causada por hipertens\u00e3o ou defeitos valvares, na CMH, o espessamento est\u00e1 associado \u00e0s muta\u00e7\u00f5es gen\u00e9ticas em vez da sobrecarga card\u00edaca.<\/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. A CMH \u00e9 heredit\u00e1ria? \u00c9 necess\u00e1rio examinar parentes?<\/p>\n<\/div>\n<div class=\"faq-answer text-main-text-color main-text-medium\">Sim, na maioria dos casos, a CMH \u00e9 herdada de forma autoss\u00f4mica dominante. \u00c9 recomendado examinar parentes de primeiro grau.<\/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. Quais sintomas podem indicar a CMH e quando \u00e9 necess\u00e1rio procurar um m\u00e9dico?<\/p>\n<\/div>\n<div class=\"faq-answer text-main-text-color main-text-medium\">Os sintomas incluem falta de ar, dor no peito, tontura e desmaios, especialmente durante esfor\u00e7o. Se voc\u00ea apresentar esses sintomas ou possui hist\u00f3rico familiar de morte s\u00fabita card\u00edaca, deve consultar um cardiologista.<\/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. A CMH \u00e9 perigosa? \u00c9 poss\u00edvel viver muitos anos com ela?<\/p>\n<\/div>\n<div class=\"faq-answer text-main-text-color main-text-medium\">Com diagn\u00f3stico precoce e tratamento adequado, a maioria dos pacientes tem uma vida plena. No entanto, em alguns casos, a CMH aumenta o risco de arritmias e morte s\u00fabita, especialmente se n\u00e3o for tratada.<\/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. Como a CMH obstrutiva difere da CMH n\u00e3o obstrutiva?<\/p>\n<\/div>\n<div class=\"faq-answer text-main-text-color main-text-medium\">Na forma obstrutiva, um septo espessado impede o fluxo sangu\u00edneo do ventr\u00edculo esquerdo, causando sintomas mais graves. Na forma n\u00e3o obstrutiva, o fluxo sangu\u00edneo n\u00e3o \u00e9 comprometido.<\/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\">6. \u00c9 poss\u00edvel praticar atividades f\u00edsicas na CMH? <\/p>\n<\/div>\n<div class=\"faq-answer text-main-text-color main-text-medium\">Esportes intensos e competitivos n\u00e3o s\u00e3o recomendados para pessoas com CMH. O exerc\u00edcio moderado \u00e9 permitido, mediante aprova\u00e7\u00e3o pelo m\u00e9dico.<\/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. Quais exames s\u00e3o necess\u00e1rios para diagnosticar a CMH?<\/p>\n<\/div>\n<div class=\"faq-answer text-main-text-color main-text-medium\">Normalmente, s\u00e3o realizados o ECG, a ecocardiografia (ultrassom do cora\u00e7\u00e3o), a resson\u00e2ncia magn\u00e9tica card\u00edaca, a monitoriza\u00e7\u00e3o eletrocardiogr\u00e1fica de 24 horas e os testes gen\u00e9ticos (quando indicados).<\/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. Como a CMH \u00e9 tratada: \u00e9 necess\u00e1rio realizar a cirurgia ou os medicamentos s\u00e3o suficientes?<\/p>\n<\/div>\n<div class=\"faq-answer text-main-text-color main-text-medium\">Os medicamentos s\u00e3o a base do tratamento. Em casos graves, pode ser necess\u00e1ria a cirurgia ou a abla\u00e7\u00e3o septal percut\u00e2nea.<\/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. O que \u00e9 o CDI e quando ele \u00e9 implantado para tratar a CMH?<\/p>\n<\/div>\n<div class=\"faq-answer text-main-text-color main-text-medium\">O CDI \u00e9 um cardioversor-desfibrilador implant\u00e1vel, um dispositivo que previne a morte s\u00fabita causada pelas arritmias. Ele \u00e9 implantado em pacientes de alto risco por recomenda\u00e7\u00e3o m\u00e9dica.<\/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. H\u00e1 cura exata para HCM? Qual \u00e9 o progn\u00f3stico?<\/p>\n<\/div>\n<div class=\"faq-answer text-main-text-color main-text-medium\">N\u00e3o h\u00e1 cura exata para CMH, mas seus sintomas podem ser controlados de forma eficaz. Com a abordagem correta, o progn\u00f3stico \u00e9 favor\u00e1vel, especialmente na aus\u00eancia de complica\u00e7\u00f5es graves.<\/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=\"referencias\">\n<div class=\"sources-list-content\">\n<div class=\"sources-list-title\">\n<p class=\"small-text-bold text-black sources-list-title-text\">Refer\u00eancias<\/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\">Cat\u00e1logo VOKA (VOKA Catalog). <\/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\">Maron BJ, Maron MS. Hypertrophic cardiomyopathy. Lancet. 2013 Jan 19;381(9862):242-55. doi: 10.1016\/S0140-6736(12)60397-3<\/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\">Zhang Y, Adamo M, Zou C, Porcari A, Tomasoni D, Rossi M, Merlo M, Liu H, Wang J, Zhou P, Metra M, Sinagra G, Zhang J. Management of hypertrophic cardiomyopathy. J Cardiovasc Med (Hagerstown). 2024 Jun 1;25(6):399-419. doi: 10.2459\/JCM.0000000000001616.<\/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\">Teekakirikul P, Zhu W, Huang HC, Fung E. Hypertrophic Cardiomyopathy: An Overview of Genetics and Management. Biomolecules. 2019 Dec 16;9(12):878. doi: 10.3390\/biom9120878.<\/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\">Maron BJ, Desai MY, Nishimura RA, Spirito P, Rakowski H, Towbin JA, Rowin EJ, Maron MS, Sherrid MV. Diagnosis and Evaluation of Hypertrophic Cardiomyopathy: JACC State-of-the-Art Review. J Am Coll Cardiol. 2022 Feb 1;79(4):372-389. doi: 10.1016\/j.jacc.2021.12.002.<\/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\">Matthia EL, Setteducato ML, Elzeneini M, Vernace N, Salerno M, Kramer CM, Keeley EC. Circulating Biomarkers in Hypertrophic Cardiomyopathy. J Am Heart Assoc. 2022 Dec 6;11(23):e027618. doi: 10.1161\/JAHA.122.027618.<\/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\">Ommen SR, Nishimura RA, Schaff HV, Dearani JA. Hypertrophic Cardiomyopathy: State of the Art. Mayo Clin Proc. 2025 Mar;100(3):557-566. doi: 10.1016\/j.mayocp.2024.07.013.<\/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>Cardiomiopatia hipertr\u00f3fica (CMH) \u00e9 uma doen\u00e7a mioc\u00e1rdica prim\u00e1ria caracterizada por hipertrofia inexplicada da parede do ventr\u00edculo esquerdo, geralmente assim\u00e9trica, sem dilata\u00e7\u00e3o da cavidade. A doen\u00e7a \u00e9 causada por muta\u00e7\u00f5es em v\u00e1rios genes que codificam prote\u00ednas sarcom\u00e9ricas, levando a anormalidades morfol\u00f3gicas, el\u00e9tricas e hemodin\u00e2micas. A CMH \u00e9 uma das formas heredit\u00e1rias mais comuns de cardiomiopatia, afetando [&hellip;]<\/p>\n","protected":false},"author":2,"featured_media":0,"template":"","diseases_category":[293],"class_list":["post-875","diseases_post","type-diseases_post","status-publish","hentry","diseases_category-cardiologia"],"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>Cardiomiopatia hipertr\u00f3fica (CMH): causas, sintomas, diagn\u00f3stico, tratamento<\/title>\n<meta name=\"description\" content=\"Cardiomiopatia hipertr\u00f3fica \u00e9 uma doen\u00e7a mioc\u00e1rdica prim\u00e1ria caracterizada por hipertrofia inexplic\u00e1vel da parede do ventr\u00edculo esquerdo. 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