{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,28]],"date-time":"2026-02-28T04:33:12Z","timestamp":1772253192064,"version":"3.50.1"},"reference-count":17,"publisher":"Sociedade Brasileira de Cardiologia","issue":"8","license":[{"start":{"date-parts":[[2024,8,1]],"date-time":"2024-08-01T00:00:00Z","timestamp":1722470400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2024,8,1]],"date-time":"2024-08-01T00:00:00Z","timestamp":1722470400000},"content-version":"am","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2024,8,1]],"date-time":"2024-08-01T00:00:00Z","timestamp":1722470400000},"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: A s\u00edndrome do PRKAG2 tipicamente se manifesta na adolesc\u00eancia e in\u00edcio da idade adulta, cursando com hipertrofia ventricular esquerda, arritmias e risco de morte s\u00fabita. O achado de marcadores ecocardiogr\u00e1ficos antes da manifesta\u00e7\u00e3o cl\u00ednica nos filhos de pais acometidos pela doen\u00e7a pode facilitar a estrat\u00e9gia de preven\u00e7\u00e3o e planejamento terap\u00eautico para esse grupo de pacientes.  Objetivo: Identificar a exist\u00eancia de achados ecocardiogr\u00e1ficos que se manifestem precocemente nos filhos de pais acometidos por s\u00edndrome do PRKAG2, enquanto ainda assintom\u00e1ticos.  M\u00e9todos: Estudo observacional transversal em que sete participantes, filhos de pais com diagn\u00f3stico estabelecido de s\u00edndrome do PRKAG2, com idades entre 9 meses e 12 anos e diagn\u00f3stico gen\u00e9tico comprovado, foram submetidos \u00e0 ecocardiografia convencional e por t\u00e9cnicas avan\u00e7adas, tendo seus achados comparados aos de grupo controle composto por sete volunt\u00e1rios pareados por sexo e idade, h\u00edgidos do ponto de vista cardiovascular. Um valor de p &lt; 0,05 foi considerado significante.  Resultados: A ecocardiografia convencional mostrou valores aumentados com signific\u00e2ncia estat\u00edstica no grupo caso para \u00e1trio esquerdo, septo interventricular, parede posterior do ventr\u00edculo esquerdo, massa ventricular indexada e espessura relativa da parede (p &lt; 0,05). O strain sist\u00f3lico longitudinal global obtido pelo ecocardiograma bidimensional n\u00e3o mostrou diferen\u00e7a estatisticamente significativa entre os grupos caso e controle. Nenhum dos par\u00e2metros ao ecocardiograma tridimensional apresentou signific\u00e2ncia estat\u00edstica entre os grupos.  Conclus\u00e3o: Crian\u00e7as diagnosticadas com PRKAG2 demonstraram achados ecocardiogr\u00e1ficos indicativos de tend\u00eancia \u00e0 hipertrofia card\u00edaca. A ecocardiografia pode ser uma ferramenta \u00fatil na avalia\u00e7\u00e3o e seguimento desse grupo de pacientes, antes do in\u00edcio de manifesta\u00e7\u00f5es cl\u00ednicas.<\/jats:p>","DOI":"10.36660\/abc.20230531","type":"journal-article","created":{"date-parts":[[2024,9,13]],"date-time":"2024-09-13T10:48:25Z","timestamp":1726224505000},"source":"Crossref","is-referenced-by-count":0,"title":["Achados Ecocardiogr\u00e1ficos em Crian\u00e7as de Pacientes com Diagn\u00f3stico de S\u00edndrome do PRKAG2"],"prefix":"10.36660","volume":"121","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3239-393X","authenticated-orcid":false,"given":"Dinamar Amador dos","family":"Santos Neto","sequence":"first","affiliation":[{"name":"Faculdade Ci\u00eancias M\u00e9dicas de Minas Gerais,  Brasil"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0009-0005-6537-5000","authenticated-orcid":false,"given":"Igor de","family":"Souza 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