Reclasificar algunas canalopatías: Cambios tras una larga espera

Margarita Dorantes Sánchez, Marleny Cruz Cardentey, Jesús A. Castro Hevia, Osmín Castañeda Chirino

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Resumen

Desde hace tiempo la muerte súbita cardíaca y las arritmias ventriculares malignas, en sujetos sin cardiopatía estructural demostrable por métodos convencionales, ha sido tema sometido de controversia. Las enfermedades eléctricas primarias se hicieron equivalentes a enfermedades puramente eléctricas y el término de canalopatía ha sufrido modificaciones porque a las mutaciones genéticas se añaden alteraciones en algunas proteínas interactuantes y porque existen anomalías estructurales tales como inflamación, fibrosis, adiposis, miocarditis, microaneurismas, aumento del colágeno, disfunción mecánica y otros, evidenciadas por procedimientos diagnósticos más finos: el corazón es normal en apariencia pero los miocitos no lo son. A la base genética (disfunción iónica) y a la clínica, se integran alteraciones estructurales y se consideran los síndromes de Brugada, de QT largo y de QT corto, como enfermedades orgánico-funcionales, electroanatómicas, con la necesidad de su reclasificación, lo cual es trascendente para estratificar riesgo y adoptar la conducta terapéutica más apropiada.

Referencias

Chugh SS, Kelly KL, Titus JL. Sudden cardiac death with apparently normal heart. Circulation. 2000;102(6):649-54. DOI: https://doi.org/10.1161/01.cir.102.6.649

Martini M, Nava A, Thiene G, Buja GF, Canciani B, Scognamiglio R, et al. Ventricular fibrillation without apparent heart disease: description of six cases. Am Heart J. 1989;118(6):1203-9. DOI: https://doi.org/10.1016/0002-8703(89)90011-2

Cerrone M, Priori SG. Genetics of sudden death: focus on inherited channelopathies. Eur Heart J. 2011;32(17):2109-18. DOI: https://doi.org/10.1093/eurheartj/ehr082

Martini B, Corrado D, Nava A, Thiene G. Syndrome of right bundle branch block, ST segment elevation and sudden death: Evidence of an organic substrate. En: Nava A, Rossi L, Thiene G, Eds. Arrhythmogenic right ventricular cardiomyopathy/dysplasia. Amsterdam: Elsevier; 1997. p. 438-53.

Papadakis M, Raju H, Behr ER, De Noronha SV, Spath N, Kouloubinis A, et al. Sudden cardiac death with autopsy findings of uncertain significance: potential for erroneous interpretation. Circ Arrhythm Electrophysiol. 2013;6(3):588-96. DOI: https://doi.org/10.1161/circep.113.000111

Kim H, Cho Y, Park Y, Lee H, Kang H, Nah DY, et al. Underlying cardiomyopathy in patients with ST-segment elevation in the right precordial leads. Circ J. 2006;70(6):719-25. DOI: https://doi.org/10.1253/circj.70.719

Nador F, Beria G, De Ferrari GM, Stramba-Badiale M, Locati EH, Lotto A, et al. Unsuspected echocardiographic abnormality in the long QT syndrome. Diagnostic, prognostic, and pathogenetic implications. Circulation. 1991;84(4):1530-42. DOI: https://doi.org/10.1161/01.cir.84.4.1530

Brado J, Dechant MJ, Menza M, Komancsek A, Lang CN, Bugger H, et al. Phase-contrast magnet resonance imaging reveals regional, transmural, and base-to-apex dispersion of mechanical dysfunction in patients with long QT syndrome. Heart Rhythm. 2017;14(9):1388-97. DOI: https://doi.org/10.1016/j.hrthm.2017.04.045

Rosenbaum DS. Is long QT syndrome a disease of abnormal mechanical contraction? Circulation. 2010;122(14):1353-4. DOI: https://doi.org/10.1161/circulationaha.110.980706

Arbelo E. Another piece of the puzzle: Assessment of mechanical dispersion by magnetic resonance in long QT syndrome. Heart Rhythm. 2017;14(9):1398-9. DOI: https://doi.org/10.1016/j.hrthm.2017.06.026

De Ferrari GM, Schwartz PJ. Long QT syndrome, a purely electrical disease? Not anymore. Eur Heart J. 2009;30(3):253-5. DOI: https://doi.org/10.1093/eurheartj/ehn587

Frea S, Giustetto C, Capriolo M, Scrocco C, Fornengo C, Benedetto S, et al. New echocardiographic insights in short QT syndrome: More than a channelopathy? Heart Rhythm. 2015;12(10):2096-105. DOI: https://doi.org/10.1016/j.hrthm.2015.05.024

Haïssaguerre M, Nademanee K, Hocini M, Cheniti G, Duchateau J, Frontera A, et al. Depolarization versus repolarization abnormality underlying inferolateral J-wave syndromes: New concepts in sudden cardiac death with apparently normal hearts. Heart Rhythm. 2019;16(5):781-90. DOI: https://doi.org/10.1016/j.hrthm.2018.10.040

Martini B, Martini N, Dorantes M, Márquez M, Zhang L, Fontaine G, et al. Pistas de una enfermedad orgánica subyacente en el síndrome de Brugada. Arch Cardiol Mex. 2017;87(1):49-60. DOI: https://doi.org/10.1016/j.acmx.2016.10.001

Martini B. Brugada syndrome; an organic syndrome. Iran Cardiovasc Res J. 2007;1(2):64-70.

Frustaci A, Priori SG, Pieroni M, Chimenti C, Napolitano C, Rivolta I, et al. Cardiac histological substrate in patients with clinical phenotype of Brugada syndrome. Circulation. 2005;112(24):3680-7. DOI: https://doi.org/10.1161/circulationaha.105.520999

Pappone C, Monasky MM, Micaglio E, Ciconte G. Right ventricular electromechanical abnormalities in Brugada syndrome: is this a cardiomyopathy? Eur Heart J. 2020;22(Suppl E):E101-E104. DOI: https://doi.org/10.1093/eurheartj/suaa071

Pappone C, Ciconte G, Manguso F, Vicedomini G, Mecarocci V, Conti M, et al. Assessing the Malignant Ventricular Arrhythmic Substrate in Patients With Brugada Syndrome. J Am Coll Cardiol. 2018;71(15):1631-46. DOI: https://doi.org/10.1016/j.jacc.2018.02.022

Brugada J, Pappone C, Berruezo A, Vicedomini G, Manguso F, Ciconte G, et al. Brugada Syndrome Phenotype Elimination by Epicardial Substrate Ablation. Circ Arrhythm Electrophysiol. 2015;8(6):1373-81. DOI: https://doi.org/10.1161/circep.115.003220

Brugada P, Brugada J, Brugada R. 'Localized' right ventricular morphological abnormalities in patients with the Brugada syndrome: what is their significance? Eur Heart J. 2001;22(12):982-4. DOI: https://doi.org/10.1053/euhj.2000.2522

Haïssaguerre M, Hocini M, Cheniti G, Duchateau J, Sacher F, Puyo S, et al. Localized Structural Alterations Underlying a Subset of Unexplained Sudden Cardiac Death. Circ Arrhythm Electrophysiol [Internet]. 2018 [citado 2 Oct 2020];11(7):e006120. Disponible en: https://doi.org/10.1161/circep.117.006120

Shah AJ, Hocini M, Lamaison D, Sacher F, Derval N, Haissaguerre M. Regional substrate ablation abolishes Brugada syndrome. J Cardiovasc Electrophysiol. 2011;22(11):1290-1. DOI: https://doi.org/10.1111/j.1540-8167.2011.02054.x

Peters S. The history of Brugada syndrome Continuum with arrhythmogenic cardiomyopathy or lone disease? Int J Cardiol. 2016;211:84-5. DOI: https://doi.org/10.1016/j.ijcard.2016.02.132

Morgera T, Sinagra GF, Viel E, Ricci C, Bussani R, Camerini F. The syndrome of right bundle branch block, persistent ST segment elevation and sudden cardiac death. Which is the histological substrate? Eur Heart J. 1997;18(7):1190-1. DOI: https://doi.org/10.1093/oxfordjournals.eurheartj.a015421

Marras E, Basso C, Sciarra L, Delise P. Unexplained syncope, Brugada-like ECG and minimal structural right ventricular abnormalities: which is the right diagnosis? J Cardiovasc Med (Hagerstown). 2009;10(3):273-5. DOI: https://doi.org/10.2459/jcm.0b013e328322fc09

Morita H, Kusano KF, Miura D, Nagase S, Nakamura K, Morita ST, et al. Fragmented QRS as a marker of conduction abnormality and a predictor of prognosis of Brugada syndrome. Circulation. 2008;118(17):1697-704. DOI: https://doi.org/10.1161/circulationaha.108.770917

Naccarella F. Aritmie ventricolari maligne in pazienti con blocco di branca destra e persistente sopraslivellamento di ST in V1-V3: probabile cardiomiopatia aritmogena del ventricolo destro. G Ital Cardiol. 1993;23(12):1219-22.

Pappone C, Brugada J, Vicedomini G, Ciconte G, Manguso F, Saviano M, et al. Electrical Substrate Elimination in 135 Consecutive Patients With Brugada Syndrome. Circ Arrhythm Electrophysiol [Internet]. 2017 [citado 2 Oct 2020];10(5):e005053. Disponible en: https://doi.org/10.1161/circep.117.005053

Coronel R, Casini S, Koopmann TT, Wilms-Schopman FJ, Verkerk AO, de Groot JR, et al. Right ventricular fibrosis and conduction delay in a patient with clinical signs of Brugada syndrome: a combined electrophysiological, genetic, histopathologic, and computational study. Circulation. 2005;112(18):2769-77. DOI: https://doi.org/10.1161/circulationaha.105.532614

Pieroni M, Notarstefano P, Oliva A, Campuzano O, Santangeli P, Coll M, et al. Electroanatomic and Pathologic Right Ventricular Outflow Tract Abnormalities in Patients With Brugada Syndrome. J Am Coll Cardiol. 2018;72(22):2747-57. DOI: https://doi.org/10.1016/j.jacc.2018.09.037

Corrado D, Migliore F, Zorzi A. Brugada Syndrome: In Search of a Cause. J Am Coll Cardiol. 2018;72(22):2758-60. DOI: https://doi.org/10.1016/j.jacc.2018.08.2199

Hoogendijk MG, Opthof T, Postema PG, Wilde AA, de Bakker JM, Coronel R. The Brugada ECG pattern: a marker of channelopathy, structural heart disease, or neither? Toward a unifying mechanism of the Brugada syndrome. Circ Arrhythm Electrophysiol. 2010;3(3):283-90. DOI: https://doi.org/10.1161/circep.110.937029

Zhang J, Sacher F, Hoffmayer K, O'Hara T, Strom M, Cuculich P, et al. Cardiac electrophysiological substrate underlying the ECG phenotype and electrogram abnormalities in Brugada syndrome patients. Circulation. 2015;131(22):1950-9. DOI: https://doi.org/10.1161/circulationaha.114.013698

Takagi M, Aihara N, Kuribayashi S, Taguchi A, Shimizu W, Kurita T, et al. Localized right ventricular morphological abnormalities detected by electron-beam computed tomography represent arrhythmogenic substrates in patients with the Brugada syndrome. Eur Heart J. 2001;22(12):1032-41. DOI: https://doi.org/10.1053/euhj.2000.2424

Furukawa S, Morita S, Okunaga H, Wingenfeld L, Takaya A, Nakagawa T, et al. An autopsic examination case of diagnosed Brugada syndrome. Am J Int Med. 2014;2(4):79-82.

Bastiaenen R, Cox AT, Castelletti S, Wijeyeratne YD, Colbeck N, Pakroo N, et al. Late gadolinium enhancement in Brugada syndrome: A marker for subtle underlying cardiomyopathy? Heart Rhythm. 2017;14(4):583-9. DOI: https://doi.org/10.1016/j.hrthm.2016.12.004

Ohkubo K, Watanabe I, Okumura Y, Takagi Y, Ashino S, Kofune M, et al. Right ventricular histological substrate and conduction delay in patients with Brugada syndrome. Int Heart J. 2010;51(1):17-23. DOI: https://doi.org/10.1536/ihj.51.17

Morimoto S, Uemura A, Watanabe E, Hishida H, Watanabe I, Obata A, et al. A multicentre histological study of autopsied and biopsied specimens in Brugada syndrome. Eur Heart J. 2003;24(Supl 1):147. [Resumen]

Morimoto S, Uemura A, Hishida H. An autopsy case of Brugada syndrome with significant lesions in the sinus node. J Cardiovasc Electrophysiol. 2005;16(3):345-7. DOI: https://doi.org/10.1046/j.1540-8167.2004.40378.x

Juang JJ, Lin JL. Brugada syndrome: Merely a ion channelopathy, a structural heart disease, or mixed? Heart Rhythm. 2017;14(4):590-1. DOI: https://doi.org/10.1016/j.hrthm.2016.12.005

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