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Brugada ECG pattern :a physiopathological prospective study based on clinical, electrophysiological, angiographic, and genetic findings.


Brugada syndrome (BrS) is considered a primary electrical disease. Arrhythmogenic right ventricular (RV) dysplasia/cardiomyopathy (ARVD/C) may mimic its phenotype, raising the question of the possible overlap between these two conditions. Aim of the present study is to assess prospectively the prevalence of BrS and ARVD/C on the basis of international criteria, in patients with BrS-ECG and normal echocardiography, looking for a potential overlap between the two pathologies.
Myocardial Disease

Main results

114 consecutive patients matched in age underwent prospectively cardiac catheterization and quantitative biventricular contrast angiography to rule out a structural heart disease. Studied population included: 51 patients with a BrS-ECG (BrS group, 7F, 44M, 43 ± 11 y)  who had a spontaneous or ajmaline-induced BrS coved type ECG, 49 patients with localized ARVD/C but without ST segment elevation in the right precordial leads (14F, 35M, 39±13y), and 14 control patients (7 F, 7 M, 38 ± 16 y). Among BrS group, the authors identified three main angiographic phenotypes: BrS group I = patients with normal RV (n = 15, 29%); BrS group II = patients with segmental RV wall motion abnormalities but no structural arguments for ARVD/C (n = 26, 51%); BrS group III = patients with localized abnormalities suggestive of focal ARVD/C (n = 10, 20%).
Seventy-one percent of patients with BrS-ECG had abnormal RV wall motion. In BrS group III, 8/10 patients (16% of BrS patients) finally fulfilled international ESC/WHF 2000 ARVD/C criteria and 5/10 (10% of BrS patients) fulfilled BrS diagnostic criteria. An overlap was observed in 4 patients (8% of BrS patients) who fulfilled both ARVD/C and BrS criteria. Among the 45 genotyped patients, only one presented a SCN5A mutation, whereas a TRPM4 mutation was found in another patient. Both belonged to BrS group II. MOG1 gene analysis was negative for all patients, as were PKP2, DSP, DSG2, and DSC2 analyzes performed in BrS group III.


This is the first study to show prospectively an overlap between BrS and ARVD/C.
The main finding of the study is that 71% of patients with a BrS-ECG had abnormal RV wall motion and 16% had structural alterations corresponding to localized (anteroapical and/or diaphragmatic) ARVD/C. Moreover, 8% of BrS-ECG patients fulfilled both BrS and ARVD/C criteria.
These findings support the hypothesis of an overlap between BrS and localized or concealed forms of ARVD/C, but also that Brugada ECG pattern is not specific of a pathophysiological entity, i.e., BrS, but can be due to other conditions such as myocarditis or localized RV cardiomyopathy. BrS ECG pattern may be an indicator of ARVC, particularly in its clinically concealed phase, thus requiring morphological RV evaluation, such as MRI, contrast echocardiography and/or contrast angiography.
As pointed out by the authors this overlap between BrS and ARVD/C does not mean that these two diseases are the same one, but that a substantial proportion of patients with a Brugada ECG pattern present RV structural alterations.
In this study genetic screening was poorly contributive for both diseases. One of the explanations of the low prevalence of SCN5A mutations in thus series may lie in the high proportion of structural abnormalities and of localized ARVD/C.


1: Rouzet F, Algalarrondo V, Burg S, et al. Contraction delay of the RV outflow tract in patients with Brugada syndrome is dependent on the spontaneous ST-segment elevation pattern. Heart Rhythm. 2011 Dec;8(12):1905-12.

2: Yodogawa K, Morita N, Kobayashi Y, et al. A new approach for the comparison of conduction abnormality between arrhythmogenic right ventricular cardiomyopathy/dysplasia and Brugada syndrome. Ann Noninvasive Electrocardiol. 2011 Jul;16(3):263-9.

3: Duthoit G, Fressart V, Hidden-Lucet F, et al. Brugada ECG pattern: a physiopathological prospective study based on clinical, electrophysiological, angiographic, and genetic findings. Front Physiol.2012;3:474.

4: Iacoviello M, Forleo C, Puzzovivo A, et al. Altered two-dimensional strain measures of the right ventricle in patients with Brugada syndrome and arrhythmogenic right ventricular dysplasia/cardiomyopathy. Eur J Echocardiogr. 2011 Oct;12(10):773-81.

Notes to editor

Presented by : Massimo Imazio, MD, FESC
Cardiology Dpt. Maria Vittoria Hospital, Torino, Italy.

Guillaume Duthoit(*1) Véronique Fressart(*2), Françoise Hidden-Lucet(*1), Françoise Simon(*2), Darouna Kattygnarath(*3), Philippe Charron(*4), Caroline Himbert(*1), Philip Aouate(*5), Pascale Guicheney(*3), Yves Lecarpentier(*6), Robert Frank(*1) and Jean-Louis Hébert(*6)

*1 Unité de Rythmologie, Institut de Cardiologie, GHU Pitié-Salpêtrière, Paris, France
*2 UF de Cardiogénétique et Myogénétique, GHU Pitié-Salpêtrière, Paris, France
*3 Faculté de Médecine Pierre et Marie Curie, Inserm-UPMC UMR S 956, Paris, France
*4 Département de Génétique, GHU Pitié-Salpêtrière, Paris, France
*5 Service de Cardiologie, Hôpital Laënnec, Creil, France
*6 Service de Physiologie Cardiorespiratoire, CHU de Bicêtre, Le Kremlin-Bicêtre, France
Front. Physiol., 27 December 2012 | doi: 10.3389/fphys.2012.00474
The content of this article reflects the personal opinion of the author/s and is not necessarily the official position of the European Society of Cardiology.