Fibrosis, connexin-43, and conduction abnormalities in the Brugada syndrome

Journal of the American College of Cardiology

Volumn 66, Issue 18, 2015, Pages 1976-1986

  Nademanee, Koonlawee ^a   Raju, Hariharan ^b   De Noronha, Sofia V ^b   Papadakis, Michael ^b   Robinson, Laurence ^b   Rothery, Stephen ^c   Makita, Naomasa ^d   Kowase, Shinya ^e   Boonmee, Nakorn ^f   Vitayakritsirikul, Vorapot ^f   Ratanarapee, Samrerng ^g   Sharma, Sanjay ^b   Van Der Wal, Allard C ^h   Christiansen, Michael ⁱ   Tan, Hanno L ^h   Wilde, Arthur A ^h,j   Nogami, Akihiko ^k   Sheppard, Mary N ^b   Veerakul, Gumpanart ^f   Behr, Elijah R ^b  

^a Pacific Rim Electrophysiology Research Institute   (United States)

^b ST GEORGE'S UNIVERSITY OF LONDON   (United Kingdom)

^c HAMMERSMITH HOSPITAL   (United Kingdom)

^d NAGASAKI UNIVERSITY   (Japan)

^e YOKOHAMA ROSAI HOSPITAL   (Japan)

^f Royal Thai Air Force   (Thailand)

^g MAHIDOL UNIVERSITY   (Thailand)

^h ACADEMIC MEDICAL CENTER   (Netherlands)

ⁱ STATENS SERUM INSTITUT   (Denmark)

^j Princess Al Jawhara Al Brahim Centre of Excellence in Research of Hereditary Disorders   (Saudi Arabia)

^k UNIVERSITY OF TSUKUBA   (Japan)

more..

Author keywords

gap junction; myocardial fibrosis; right ventricular outflow tract; sudden arrhythmic death syndrome; sudden unexpected death

Indexed keywords

COLLAGEN; CONNEXIN 43;

ADOLESCENT; ADULT; ARRHYTHMOGENESIS; ARTICLE; AUTOPSY; BRUGADA SYNDROME; COMPUTER ASSISTED TOMOGRAPHY; CONTROLLED STUDY; ENDOCARDIUM; EPICARDIUM; FIBROSING ALVEOLITIS; GENE MUTATION; HEART ARRHYTHMIA; HEART CONDUCTION; HEART MUSCLE; HEART RIGHT VENTRICLE OUTFLOW TRACT; HUMAN; HUMAN TISSUE; IMMUNOFLUORESCENCE TEST; IN VIVO STUDY; MALE; NUCLEAR MAGNETIC RESONANCE IMAGING; PATHOPHYSIOLOGY; PHENOTYPE; PRIORITY JOURNAL; SUDDEN CARDIAC DEATH; SUDDEN DEATH; THORACOTOMY; ABLATION THERAPY; CARDIAC MUSCLE; COMPLICATION; DEATH, SUDDEN, CARDIAC; ELECTROCARDIOGRAPHY; FIBROSIS; GAP JUNCTION; HEART MUSCLE CONDUCTION SYSTEM; METABOLISM; PATHOLOGY; PERICARDIUM; PROCEDURES; VENTRICULAR OUTFLOW OBSTRUCTION;

ABLATION TECHNIQUES; ADULT; BRUGADA SYNDROME; COLLAGEN; CONNEXIN 43; DEATH, SUDDEN, CARDIAC; ELECTROCARDIOGRAPHY; FIBROSIS; GAP JUNCTIONS; HEART CONDUCTION SYSTEM; HUMANS; MALE; MYOCARDIUM; PERICARDIUM; THORACOTOMY; VENTRICULAR OUTFLOW OBSTRUCTION;

EID: 84945296649     PISSN: 07351097     EISSN: 15583597     Source Type: Journal    
DOI: 10.1016/j.jacc.2015.08.862     Document Type: Article

References (34)

1. S.G. Priori, A.A. Wilde, M. Horie, and et al. Executive summary: HRS/EHRA/APHRS expert consensus statement on the diagnosis and management of patients with inherited primary arrhythmia syndromes Europace 15 2013 1389 1406
2. P. Brugada, and J. Brugada Right bundle branch block, persistent ST segment elevation and sudden cardiac death: a distinct clinical and electrocardiographic syndrome. A multicenter report J Am Coll Cardiol 20 1992 1391 1396
3. E. Behr, D.A. Wood, M. Wright, et al. for the Sudden Arrhythmic Death Syndrome (SADS) Steering Group Cardiological assessment of first-degree relatives in sudden arrhythmic death syndrome Lancet 362 2003 1457 1459
4. H. Raju, and E.R. Behr Unexplained sudden death, focussing on genetics and family phenotyping Curr Opin Cardiol 28 2013 19 25
5. P.L. Hedley, P. Jørgensen, S. Schlamowitz, and et al. The genetic basis of Brugada syndrome: a mutation update Hum Mutat 30 2009 1256 1266
6. G.X. Yan, and C. Antzelevitch Cellular basis for the Brugada syndrome and other mechanisms of arrhythmogenesis associated with ST-segment elevation Circulation 100 1999 1660 1666
7. D. Corrado, A. Nava, G. Buja, and et al. Familial cardiomyopathy underlies syndrome of right bundle branch block, ST segment elevation and sudden death J Am Coll Cardiol 27 1996 443 448
8. D. Corrado, C. Basso, G. Buja, and et al. Right bundle branch block, right precordial ST-segment elevation, and sudden death in young people Circulation 103 2001 710 717
9. K. Ohkubo, I. Watanabe, Y. Okumura, and et al. Right ventricular histological substrate and conduction delay in patients with Brugada syndrome Int Heart J 51 2010 17 23
10. K. Nademanee, G. Veerakul, P. Chandanamattha, and et al. Prevention of ventricular fibrillation episodes in Brugada syndrome by catheter ablation over the anterior right ventricular outflow tract epicardium Circulation 123 2011 1270 1279
11. H. Morita, D.P. Zipes, S.T. Morita, and et al. Epicardial ablation eliminates ventricular arrhythmias in an experimental model of Brugada syndrome Heart Rhythm 6 2009 665 671
12. M. Papadakis, H. Raju, E.R. Behr, and et al. Sudden cardiac death with autopsy findings of uncertain significance: potential for erroneous interpretation Circ Arrhythm Electrophysiol 6 2013 588 596
13. A. Frustaci, S.G. Priori, M. Pieroni, and et al. Cardiac histological substrate in patients with clinical phenotype of Brugada syndrome Circulation 112 2005 3680 3687
14. R. Coronel, S. Casini, T.T. Koopmann, and 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 112 2005 2769 2777
15. A.A.M. Wilde, P.G. Postema, J.M. Di Diego, and et al. The pathophysiological mechanism underlying Brugada syndrome: depolarization versus repolarization J Mol Cell Cardiol 49 2010 543 553
16. K.L. Waldo, C.W. Lo, and M.L. Kirby Connexin 43 expression reflects neural crest patterns during cardiovascular development Dev Biol 208 1999 307 323
17. M.V. Elizari, R. Levi, R.S. Acunzo, and et al. Abnormal expression of cardiac neural crest cells in heart development: a different hypothesis for the etiopathogenesis of Brugada syndrome Heart Rhythm 4 2007 359 365
18. H. Raju, M. Papadakis, M. Govindan, and et al. Low prevalence of risk markers in cases of sudden death due to Brugada syndrome: relevance to risk stratification in Brugada syndrome J Am Coll Cardiol 57 2011 2340 2345
19. M. Govindan, V.N. Batchvarov, H. Raju, and et al. Utility of high and standard right precordial leads during ajmaline testing for the diagnosis of Brugada syndrome Heart 96 2010 1904 1908
20. C. Basso, M. Burke, P. Fornes, et al. for the Association for European Cardiovascular Pathology Guidelines for autopsy investigation of sudden cardiac death Pathologica 102 2010 391 404
21. A. Royer, T.A.B. van Veen, S. Le Bouter, and et al. Mouse model of SCN5A-linked hereditary Lenègre's disease: age-related conduction slowing and myocardial fibrosis Circulation 111 2005 1738 1746
22. +/- hearts: the effects of ageing and sex Mech Ageing Dev 133 2012 591 599
23. Y. Zhang, L. Guzadhur, K. Jeevaratnam, and et al. Arrhythmic substrate, slowed propagation and increased dispersion in conduction direction in the right ventricular outflow tract of murine Scn5a+/- hearts Acta Physiol (Oxf) 211 2014 559 573
24. M.G. Hoogendijk, M. Potse, A.C. Linnenbank, and et al. Mechanism of right precordial ST-segment elevation in structural heart disease: excitation failure by current-to-load mismatch Heart Rhythm 7 2010 238 248
25. P.G. Meregalli, H.L. Tan, V. Probst, and et al. Type of SCN5A mutation determines clinical severity and degree of conduction slowing in loss-of-function sodium channelopathies Heart Rhythm 6 2009 341 348
26. X. Hao, Y. Zhang, X. Zhang, and et al. TGF-β1-mediated fibrosis and ion channel remodeling are key mechanisms in producing the sinus node dysfunction associated with SCN5A deficiency and aging Circ Arrhythm Electrophysiol 4 2011 397 406
27. P.D. Lambiase, A.K. Ahmed, E.J. Ciaccio, and et al. High-density substrate mapping in Brugada syndrome: combined role of conduction and repolarization heterogeneities in arrhythmogenesis Circulation 120 2009 106 117
28. F. Sacher, L. Jesel, P. Jais, and et al. Insight into the mechanism of Brugada syndrome: epicardial substrate and modification during ajmaline testing Heart Rhythm 11 2014 732 734
29. P.G. Postema, P.F.H.M. van Dessel, J.M.T. de Bakker, and et al. Slow and discontinuous conduction conspire in Brugada syndrome: a right ventricular mapping and stimulation study Circ Arrhythm Electrophysiol 1 2008 379 386
30. J. Zhang, F. Sacher, K. Hoffmayer, and et al. Cardiac electrophysiological substrate underlying the ECG phenotype and electrogram abnormalities in Brugada syndrome patients Circulation 131 2015 1950 1959
31. T. Papavassiliu, C. Wolpert, S. Flüchter, and et al. Magnetic resonance imaging findings in patients with Brugada syndrome J Cardiovasc Electrophysiol 15 2004 1133 1138
32. F. Van Hoorn, M.E. Campian, A. Spijkerboer, and et al. SCN5A mutations in Brugada syndrome are associated with increased cardiac dimensions and reduced contractility PloS One 7 2012 e42037
33. S. Zumhagen, T. Spieker, J. Rolinck, and et al. Absence of pathognomonic or inflammatory patterns in cardiac biopsies from patients with Brugada syndrome Circ Arrhythm Electrophysiol 2 2009 16 23
34. M.G. Hoogendijk, T. Opthof, P.G. Postema, and et al. The Brugada ECG pattern: a marker of channelopathy, structural heart disease, or neither? Toward a unifying mechanism of the Brugada syndrome Circ Arrhythm Electrophysiol 3 2010 283 290