{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,5,17]],"date-time":"2025-05-17T04:08:07Z","timestamp":1747454887190,"version":"3.40.5"},"reference-count":28,"publisher":"Sociedade Brasileira de Cardiologia","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2022,11,11]]},"DOI":"10.36660\/abc.20210801","type":"journal-article","created":{"date-parts":[[2022,11,11]],"date-time":"2022-11-11T16:45:29Z","timestamp":1668185129000},"source":"Crossref","is-referenced-by-count":1,"title":["Acometimento do Ventr\u00edculo Direito na Cardiomiopatia por Dep\u00f3sito de Glicog\u00eanio (PRKAG2): An\u00e1lise Ecocardiogr\u00e1fica Convencional e Avan\u00e7ada"],"prefix":"10.36660","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-8920-5173","authenticated-orcid":false,"given":"Jos\u00e9 Luiz Barros","family":"Pena","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Fabricio Junqueira de","family":"Melo","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Wander Costa","family":"Santos","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Isabel Cristina Gomes","family":"Moura","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gabriela Pansanato","family":"Nakashima","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Natalia Costa","family":"Freitas","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Eduardo Back","family":"Sternick","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"22636","reference":[{"issue":"2","key":"key20221111114521_B1","doi-asserted-by":"crossref","first-page":"258","DOI":"10.1006\/geno.2000.6376","article-title":"Molecular cloning, genomic organization, and mapping of PRKAG2, a heart abundant gamma2 subunit of 5\u2019 AMP-activated protein kinase, to human chromosome 7q36","volume":"70","author":"Lang","year":"2000","unstructured":"1. Lang T, Yu L, Tu Q, Jiang J, Chen Z, Xin Y, et al. Molecular cloning, genomic organization, and mapping of PRKAG2, a heart abundant gamma2 subunit of 5\u2019 AMP-activated protein kinase, to human chromosome 7q36. Genomics. 2000;70(2):258\u201363. doi: 10.1006\/geno.2000.6376.","journal-title":"Genomics"},{"key":"key20221111114521_B2","doi-asserted-by":"crossref","first-page":"659","DOI":"10.1042\/bj3460659","article-title":"Characterization of AMP-activated protein kinase \u03b3-subunit isoforms and their role in AMP binding","volume":"346","author":"Cheung","year":"2000","unstructured":"2. Cheung PC, Salt IP, Davies PD, Hardie DG, Carling D. Characterization of AMP-activated protein kinase \u03b3-subunit isoforms and their role in AMP binding. Biochem J. 2000;346(Pt 3):659\u201369. PMID:10698692","journal-title":"Biochem J"},{"issue":"4","key":"key20221111114521_B3","first-page":"651","article-title":"High prevalence of arrhythmic and myocardial complications in patients with cardiac glycogenosis due to PRKAG2 mutations","volume":"19","author":"Thevenon","year":"2017","unstructured":"3. Thevenon J, Laurent G, Ader F, Laf\u00f4ret P, Klug D, Duva Pentiehl H, et al. High prevalence of arrhythmic and myocardial complications in patients with cardiac glycogenosis due to PRKAG2 mutations. Europace. 2017;19(4):651-9. doi: 10.1093\/europace\/euw067.","journal-title":"Europace"},{"issue":"24","key":"key20221111114521_B4","doi-asserted-by":"crossref","first-page":"1823","DOI":"10.1056\/NEJM200106143442403","article-title":"Identification of a gene responsible for familial Wolff-Parkinson-White syndrome","volume":"344","author":"Gollob","year":"2001","unstructured":"4. Gollob MH, Green MS, Tang AS, Gollob T, Karibe A, Ali Hassan AS, et al. Identification of a gene responsible for familial Wolff-Parkinson-White syndrome. N Engl J Med. 2001;344(24):1823\u201331. doi:10.1056\/NEJM200106143442403.","journal-title":"N Engl J Med"},{"issue":"2","key":"key20221111114521_B5","doi-asserted-by":"crossref","first-page":"259","DOI":"10.1111\/j.1432-1033.1997.00259.x","article-title":"The AMP-activated protein kinase--fuel gauge of the mammalian cell?","volume":"246","author":"Hardie","year":"1997","unstructured":"5. Hardie DG, Carling D. The AMP-activated protein kinase--fuel gauge of the mammalian cell? Eur J Biochem. 1997;246(2):259\u201373. doi: 10.1111\/j.1432-1033.1997.00259.x.","journal-title":"Eur J Biochem"},{"issue":"1","key":"key20221111114521_B6","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1161\/CIRCEP.115.003121","article-title":"Clinical spectrum of PRKAG2 syndrome","volume":"9","author":"Porto","year":"2016","unstructured":"6. Porto AG, Brun F, Severini GM, Losurdo P, Fabris E, Taylor MRG, et al. Clinical spectrum of PRKAG2 syndrome. Circ Arrhythm Electrophysiol. 2016;9(1):1\u20139. doi: 10.1161\/CIRCEP.115.003121.","journal-title":"Circ Arrhythm Electrophysiol"},{"issue":"6","key":"key20221111114521_B7","doi-asserted-by":"crossref","first-page":"591","DOI":"10.1093\/ehjci\/jey042","article-title":"Standardization of Left Atrial, Right Ventricular, and Right Atrial Deformation Imaging Using Two-Dimensional Speckle Tracking. Echocardiography: A Consensus Document of the EACVI\/ASE\/Industry Task Force to Standardize Deformation Imaging","volume":"19","author":"Badano","year":"2018","unstructured":"7. Badano LP, Kolias TJ, Muraru D, Abraham TP, Aurigemma G, edvardsen T, et al. Standardization of Left Atrial, Right Ventricular, and Right Atrial Deformation Imaging Using Two-Dimensional Speckle Tracking. Echocardiography: A Consensus Document of the EACVI\/ASE\/Industry Task Force to Standardize Deformation Imaging. Eur Heart J Cardiovasc Imaging. 2018;19(6):591-600. doi: 10.1093\/ehjci\/jey042.","journal-title":"Eur Heart J Cardiovasc Imaging"},{"issue":"5","key":"key20221111114521_B8","doi-asserted-by":"crossref","first-page":"700","DOI":"10.1161\/CIRCIMAGING.113.000706","article-title":"Age-, Body Size-, and Sex-Specific Reference Values for Right Ventricular Volumes and Ejection Fraction by Three-Dimensional Echocardiography: A Multicenter Echocardiographic Study in 507 Healthy Volunteers","volume":"6","author":"Maffessanti","year":"2013","unstructured":"8. Maffessanti F, Muraru D, Esposito R, Gripari P, Ermacora D, Santoro C, et al. Age-, Body Size-, and Sex-Specific Reference Values for Right Ventricular Volumes and Ejection Fraction by Three-Dimensional Echocardiography: A Multicenter Echocardiographic Study in 507 Healthy Volunteers. Circ Cardiovasc Imaging. 2013;6(5):700-10. Doi:10.1161\/CIRCIMAGING113.000.706","journal-title":"Circ Cardiovasc Imaging"},{"issue":"7","key":"key20221111114521_B9","doi-asserted-by":"crossref","first-page":"800","DOI":"10.1093\/ehjci\/jeaa176","article-title":"Glycogen storage cardiomyopathy (PRKAG2): diagnostic findings of standard and advanced echocardiography techniques","volume":"22","author":"Pena","year":"2021","unstructured":"9. Pena JLB, Santos WC, Siqueira MHA, Sampaio IH, Moura OCG, Sternick EB, et al. Glycogen storage cardiomyopathy (PRKAG2): diagnostic findings of standard and advanced echocardiography techniques. Eur Heart J Cardiovasc Imaging. 2021;22(7):800-7. doi: 10.1093\/ehjci\/jeaa176.","journal-title":"Eur Heart J Cardiovasc Imaging"},{"issue":"11","key":"key20221111114521_B10","doi-asserted-by":"crossref","first-page":"891","DOI":"10.1016\/j.jchf.2018.05.022","article-title":"Forgotten no more: a focused update on the right ventricle in cardiovascular disease","author":"Amsallem","year":"2018","unstructured":"10. Amsallem M, Mercier O, Kobayashi Y, Moneghetti K, Haddad F. Forgotten no more: a focused update on the right ventricle in cardiovascular disease. JACC Heart Fail. 2018 (11):891-903. doi: 10.1016\/j.jchf.2018.05.022.","journal-title":"JACC Heart Fail"},{"issue":"20","key":"key20221111114521_B11","doi-asserted-by":"crossref","first-page":"e578","DOI":"10.1161\/CIR.0000000000000560","article-title":"Evaluation and management of right-sided heart failure: a scientific statement from the American Heart Association","volume":"137","author":"Konstam","year":"2018","unstructured":"11. Konstam MA, Kiernan MS, Bernstein DBozkurt B, Jacob M, Kapur NK, et al. Evaluation and management of right-sided heart failure: a scientific statement from the American Heart Association. Circulation. 2018;137(20):e578\u2013e622. doi: 10.1161\/CIR.0000000000000560.","journal-title":"Circulation"},{"issue":"1","key":"key20221111114521_B12","article-title":"The right ventricle: interaction with the pulmonary circulation","volume":"20","author":"Pinsky","year":"2016","unstructured":"12. Pinsky MR. The right ventricle: interaction with the pulmonary circulation. Crit Care. 2016;20(1):266. doi: 10.1186\/s13054-016-1440-0.","journal-title":"Crit Care"},{"issue":"10","key":"key20221111114521_B13","first-page":"653","article-title":"The right ventricle: anatomy. physiology. and clinical importance","volume":"16","author":"Dell\u2019Italia","year":"1991","unstructured":"13. Dell\u2019Italia LJ. The right ventricle: anatomy. physiology. and clinical importance. Curr Probl Cardiol. 1991;16(10): 653\u2013720. doi: 10.1016\/0146-2806(91)90009-y","journal-title":"Curr Probl Cardiol"},{"issue":"2","key":"key20221111114521_B14","doi-asserted-by":"crossref","first-page":"501","DOI":"10.1081\/JCMR-200053610","article-title":"Assessment of myocardial function: a review of quantification methods and results using tagged MRI","volume":"7","author":"Petitjean","year":"2005","unstructured":"14. Petitjean C, Rougon N, Cluzel P. Assessment of myocardial function: a review of quantification methods and results using tagged MRI. J Cardiovasc Magn Reson. 2005;7(2): 501\u201316. doi: 10.1081\/jcmr-200053610","journal-title":"J Cardiovasc Magn Reson"},{"issue":"1","key":"key20221111114521_B15","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1016\/j.echo.2014.10.003","article-title":"Recommendations for cardiac chamber quantification by echocardiography in adults: an update from the American Society of Echocardiography and European Association of Cardiovascular Imaging","volume":"28","author":"Lang","year":"2015","unstructured":"15. Lang RM, Badano LP, Mor-Avi V, Afilalo J, Armstrong A, Ernande L, et al. Recommendations for cardiac chamber quantification by echocardiography in adults: an update from the American Society of Echocardiography and European Association of Cardiovascular Imaging. J Am Soc Echocardiogr. 2015;28(1):1-39.e14. doi: 10.1016\/j.echo.2014.10.003.","journal-title":"J Am Soc Echocardiogr"},{"issue":"7","key":"key20221111114521_B16","doi-asserted-by":"crossref","first-page":"685","DOI":"10.1016\/j.echo.2010.05.010","article-title":"Guidelines for the Echocardiographic Assessment of the Right Heart in Adults: A Report from the American Society of Echocardiography","volume":"23","author":"Rudski","year":"2010","unstructured":"16. Rudski LG, Lai WW, Afilalo J, Hua L, Handschumaker MD, Chandrasekaran K, et al. Guidelines for the Echocardiographic Assessment of the Right Heart in Adults: A Report from the American Society of Echocardiography endorsed by the European Association of Echocardiography, a Registered Branch of the European Society of Cardiology, and the Canadian Society of Echocardiography. J Am Soc Echocardiogr.2010;23(7):685-713. doi: 10.1016\/j.echo.2010.05.010.","journal-title":"J Am Soc Echocardiogr"},{"key":"key20221111114521_B17","doi-asserted-by":"crossref","DOI":"10.1016\/j.ebiom.2020.102723","article-title":"Identification, Clinical Manifestation and Structural Mechanisms of Mutations in AMPK Associated Cardiac Glycogen Storage Disease","volume":"54","author":"Hu","year":"2020","unstructured":"17. Hu D, Hu D, Liu L, Barr D, Liu Y, Valderrabano-Saucedo N, et al. Identification, Clinical Manifestation and Structural Mechanisms of Mutations in AMPK Associated Cardiac Glycogen Storage Disease. EBioMedicine. 2020;54:102723. doi: 10.1016\/j.ebiom.2020.102723.","journal-title":"EBioMedicine"},{"issue":"4","key":"key20221111114521_B18","first-page":"651","article-title":"High prevalence of arrhythmic and myocardial complications in patients with cardiac glycogenosis due to PRKAG2 mutations","volume":"19","author":"Thevenon","year":"2017","unstructured":"18. Thevenon J, Laurent G, Ader F, Laforet P, Klug D, Pentiah AD, et al. High prevalence of arrhythmic and myocardial complications in patients with cardiac glycogenosis due to PRKAG2 mutations. EP Europace.2017;19(4):651-9. doi:10.1093\/europace\/euw067.","journal-title":"EP Europace"},{"issue":"1","key":"key20221111114521_B19","doi-asserted-by":"crossref","first-page":"58","DOI":"10.1016\/j.hrthm.2010.09.081","article-title":"Clinical, electrocardiographic, and electrophysiologic characteristics of patients with a fasciculoventricular pathway: the role of PRKAG2 mutation","volume":"8","author":"Sternick","year":"2011","unstructured":"19. Sternick EB, Oliva A, Gerken LM; Magalh\u00e3es L, Scarpelli R, Correia FS, et al. Clinical, electrocardiographic, and electrophysiologic characteristics of patients with a fasciculoventricular pathway: the role of PRKAG2 mutation. Heart Rhythm. 2011;8(1):58\u201364. doi: 10.1016\/j.hrthm.2010.09.081.","journal-title":"Heart Rhythm"},{"issue":"2","key":"key20221111114521_B20","doi-asserted-by":"crossref","first-page":"186","DOI":"10.1016\/j.jacc.2020.05.029","article-title":"Clinical features and natural history of PRKAG2 variant cardiac glycogenosis","volume":"76","author":"Lopez-Sainz","year":"2020","unstructured":"20. Lopez-Sainz A, Dominguez F, Lopes LR, Ochoa JP, Barriales-Villa R, Climent V, et al. Clinical features and natural history of PRKAG2 variant cardiac glycogenosis. J Am Coll Cardiol.2020;76(2):186-97. doi: 10.1016\/j.jacc.2020.05.029.","journal-title":"J Am Coll Cardiol"},{"issue":"12","key":"key20221111114521_B21","doi-asserted-by":"crossref","first-page":"1463","DOI":"10.1016\/j.jacc.2018.12.076","article-title":"Anatomy, Function, and Dysfunction of the Right Ventricle: JACC State-of-the-Art Review","volume":"73","author":"Sanz","year":"2019","unstructured":"21. Sanz J, S\u00e1nchez-Quintana D, Bossone E, Bogaard H, Meije R. Anatomy, Function, and Dysfunction of the Right Ventricle: JACC State-of-the-Art Review. J Am Coll Cardiol. 2019;73(12):1463-82. doi: 10.1016\/j.jacc.2018.12.076.","journal-title":"J Am Coll Cardiol"},{"issue":"7","key":"key20221111114521_B22","doi-asserted-by":"crossref","first-page":"855","DOI":"10.1056\/NEJMra1710575","article-title":"Clinical Course and Management of Hypertrophic Cardiomyopathy","volume":"379","author":"Maron","year":"2018","unstructured":"22. Maron BJ. Clinical Course and Management of Hypertrophic Cardiomyopathy. N Engl J Med.2018;379(7):855-68. doi: 10.1056\/NEJMra1710575.","journal-title":"N Engl J Med"},{"issue":"11","key":"key20221111114521_B23","doi-asserted-by":"crossref","first-page":"1329","DOI":"10.1016\/j.echo.2015.07.015","article-title":"Right ventricular remodeling, its correlates, and its clinical impact in hypertrophic cardiomyopathy","volume":"28","author":"Ro\u015fca","year":"2015","unstructured":"23. Ro\u015fca M, C\u0103lin A, Beladan CC, Enache R, Meetescu AD, Gurzun MN, et al. Right ventricular remodeling, its correlates, and its clinical impact in hypertrophic cardiomyopathy. J Am Soc Echocardiogr.2015;28(11):1329-38. Doi:10.1016\/j.echo.2015.07.015.","journal-title":"J Am Soc Echocardiogr"},{"issue":"7","key":"key20221111114521_B24","doi-asserted-by":"crossref","first-page":"845","DOI":"10.1016\/j.echo.2019.03.005","article-title":"Role of Right Ventricular Strain Measured by Two-Dimensional Echocardiography in the Diagnosis of Cardiac Amyloidosis","volume":"32","author":"Mo\u00f1ivas Palomero","year":"2019","unstructured":"24. Mo\u00f1ivas Palomero V, Durante-Lopez A, Sanabria MT, Cubero JS, Gonzalez-Mirelis J, Lopez-Ibor JV, et al. Role of Right Ventricular Strain Measured by Two-Dimensional Echocardiography in the Diagnosis of Cardiac Amyloidosis. J Am Soc Echocardiogr. 2019;32(7):845-53.e1. doi: 10.1016\/j.echo.2019.03.005.","journal-title":"J Am Soc Echocardiogr"},{"issue":"4","key":"key20221111114521_B25","doi-asserted-by":"crossref","first-page":"602","DOI":"10.1016\/j.hrthm.2013.12.020","article-title":"Right ventricular apical pacing-induced left ventricular dyssynchrony is associated with a subsequent decline in ejection fraction","volume":"11","author":"Ahmed","year":"2014","unstructured":"25. Ahmed M, Gorcsan J 3rd, Marek J, Ryo K, Haugaa K, R Ludwig D, et al. Right ventricular apical pacing-induced left ventricular dyssynchrony is associated with a subsequent decline in ejection fraction. Heart Rhythm. 2014;11(4):602-8. doi: 10.1016\/j.hrthm.2013.12.020.","journal-title":"Heart Rhythm"},{"issue":"2","key":"key20221111114521_B26","doi-asserted-by":"crossref","DOI":"10.1161\/CIRCIMAGING.116.005384","article-title":"Prognostic Value of Right Ventricular Ejection Fraction Assessed by Transthoracic 3D Echocardiography","volume":"10","author":"Nagata","year":"2017","unstructured":"26. Nagata Y, Wu VC, Kado Y, Otani K, Lin FC, Otsugi Y, et al. Prognostic Value of Right Ventricular Ejection Fraction Assessed by Transthoracic 3D Echocardiography. Circ Cardiovasc Imaging.2017;10(2):e005384 doi:10.1161\/CIRCIMAGING.116.005384.","journal-title":"Circ Cardiovasc Imaging"},{"issue":"2","key":"key20221111114521_B27","doi-asserted-by":"crossref","first-page":"301","DOI":"10.1007\/s10554-014-0551-4","article-title":"Global cardiac alterations detected by speckle-tracking echocardiography in Fabry disease: left ventricular, right ventricular, and left atrial dysfunction are common and linked to worse symptomatic status","volume":"31","author":"Morris","year":"2015","unstructured":"27. Morris DA, Blaschke D, Canaan-K\u00fchl S, Plockinger U, Knobloch G, Walter TC, et al. Global cardiac alterations detected by speckle-tracking echocardiography in Fabry disease: left ventricular, right ventricular, and left atrial dysfunction are common and linked to worse symptomatic status. Int J Cardiovasc Imaging. 2015 Feb;31(2):301-13. doi: 10.1007\/s10554-014-0551-4.","journal-title":"Int J Cardiovasc Imaging"},{"issue":"8","key":"key20221111114521_B28","doi-asserted-by":"crossref","DOI":"10.1161\/CIRCHEARTFAILURE.116.003367","article-title":"Glycogen storage disease because of a PRKAG2 mutation causing severe biventricular hypertrophy and high-grade atrio-ventricular block","volume":"9","author":"Yogasundaram","year":"2016","unstructured":"28. Yogasundaram H, Paterson ID, Graham M, Sergi C, Oudit GY. et al. Glycogen storage disease because of a PRKAG2 mutation causing severe biventricular hypertrophy and high-grade atrio-ventricular block. Circ Heart Fail. 2016;9(8):e003367. doi: 10.1161\/CIRCHEARTFAILURE.116.003367.","journal-title":"Circ Heart Fail"}],"container-title":["Arquivos Brasileiros de Cardiologia"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/abccardiol.org\/article\/acometimento-do-ventriculo-direito-na-cardiomiopatia-por-deposito-de-glicogenio-prkag2-analise-ecocardiografica-convencional-e-avancada\/","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,5,16]],"date-time":"2025-05-16T13:10:57Z","timestamp":1747401057000},"score":1,"resource":{"primary":{"URL":"https:\/\/abccardiol.org\/article\/acometimento-do-ventriculo-direito-na-cardiomiopatia-por-deposito-de-glicogenio-prkag2-analise-ecocardiografica-convencional-e-avancada\/"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,11,11]]},"references-count":28,"alternative-id":["10.36660\/abc.20210801"],"URL":"https:\/\/doi.org\/10.36660\/abc.20210801","relation":{},"ISSN":["0066-782X","1678-4170"],"issn-type":[{"type":"print","value":"0066-782X"},{"type":"electronic","value":"1678-4170"}],"subject":[],"published":{"date-parts":[[2022,11,11]]}}}