{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,3]],"date-time":"2026-06-03T09:02:29Z","timestamp":1780477349951,"version":"3.54.1"},"reference-count":36,"publisher":"Sociedade Brasileira de Cardiologia","issue":"2","license":[{"start":{"date-parts":[[2026,1,1]],"date-time":"2026-01-01T00:00:00Z","timestamp":1767225600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2026,1,1]],"date-time":"2026-01-01T00:00:00Z","timestamp":1767225600000},"content-version":"am","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2026,1,1]],"date-time":"2026-01-01T00:00:00Z","timestamp":1767225600000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Arq. Bras. Cardiol."],"abstract":"<jats:p>Resumo  Fundamento  As caracter\u00edsticas histopatol\u00f3gicas da cardiomiopatia PRKAG2 foram relatadas de forma fragmentada.  Objetivo  Nosso objetivo foi avaliar sistematicamente as caracter\u00edsticas patol\u00f3gicas card\u00edacas da cardiomiopatia PRKAG2 em uma grande coorte de pacientes e avaliar seu potencial diagn\u00f3stico em compara\u00e7\u00e3o com o sequenciamento gen\u00e9tico.  M\u00e9todos  Realizamos um estudo observacional, transversal, caso-controle, incluindo 18 pacientes com cardiomiopatia associada ao gene PRKAG2 e 11 receptores de transplante card\u00edaco como grupo controle. Todos os pacientes foram submetidos a bi\u00f3psia endomioc\u00e1rdica percut\u00e2nea do ventr\u00edculo direito. As amostras de tecido foram analisadas por meio de colora\u00e7\u00e3o com hematoxilina-eosina (H&amp;E), colora\u00e7\u00e3o com \u00e1cido peri\u00f3dico-Schiff para glicog\u00eanio, tricr\u00f4mico de Masson para fibrose e avalia\u00e7\u00e3o ultraestrutural por microscopia eletr\u00f4nica de transmiss\u00e3o. A signific\u00e2ncia estat\u00edstica foi definida em p &lt; 0,05 para todas as an\u00e1lises.  Resultados  Os cora\u00e7\u00f5es com cardiomiopatia associada ao gene PRKAG2 apresentaram aumento significativo dos cardiomi\u00f3citos, mitoc\u00f4ndrias com apar\u00eancia normal, extensa vacuoliza\u00e7\u00e3o da maioria das miofibras, fibrose intersticial m\u00ednima (apenas dois pacientes apresentaram fibrose leve) e aus\u00eancia de infiltra\u00e7\u00e3o de c\u00e9lulas inflamat\u00f3rias. A microscopia eletr\u00f4nica de transmiss\u00e3o revelou abundante glicog\u00eanio citos\u00f3lico, principalmente na regi\u00e3o perinuclear, com dep\u00f3sitos adicionais nas \u00e1reas intermiofibrilares e subsarcolem\u00e1ticas. Esse ac\u00famulo pronunciado de glicog\u00eanio, observado consistentemente em todos os pacientes com PRKAG2, estava ausente nos controles.  Conclus\u00e3o  O exame histol\u00f3gico e ultraestrutural de amostras de bi\u00f3psia endomioc\u00e1rdica do ventr\u00edculo direito revela um conjunto distinto de caracter\u00edsticas que sugerem fortemente a cardiomiopatia associada ao gene PRKAG2.<\/jats:p>","DOI":"10.36660\/abc.20240616","type":"journal-article","created":{"date-parts":[[2026,3,17]],"date-time":"2026-03-17T18:49:22Z","timestamp":1773773362000},"source":"Crossref","is-referenced-by-count":2,"title":["Cardiomiopatia PRKAG2: Um Estudo Caso-Controle sobre o Rendimento Diagn\u00f3stico da Histopatologia e da An\u00e1lise Ultraestrutural da Bi\u00f3psia Endomioc\u00e1rdica"],"prefix":"10.36660","volume":"123","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-8154-9606","authenticated-orcid":false,"given":"Kinulpe","family":"Honorato-Sampaio","sequence":"first","affiliation":[{"name":"Universidade Federal do Vale do Jequitinhonha e Mucuri,  Brasil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carla 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