Journal Article

Magnetic resonance investigations in Brugada syndrome reveal unexpectedly high rate of structural abnormalities

Oronzo Catalano, Serena Antonaci, Guido Moro, Maria Mussida, Mauro Frascaroli, Maurizia Baldi, Franco Cobelli, Paola Baiardi, Janni Nastoli, Raffaella Bloise, Nicola Monteforte, Carlo Napolitano and Silvia G. Priori

in European Heart Journal

Published on behalf of European Society of Cardiology

Volume 30, issue 18, pages 2241-2248
Published in print September 2009 | ISSN: 0195-668x
Published online June 2009 | e-ISSN: 1522-9645 | DOI: https://dx.doi.org/10.1093/eurheartj/ehp252
Magnetic resonance investigations in Brugada syndrome reveal unexpectedly high rate of structural abnormalities

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Aims

Recent data suggest that sub-clinical structural abnormalities may be part of the Brugada syndrome (BrS) phenotype, a disease traditionally thought to occur in the structurally normal heart. In this study, we carried out detailed assessment of cardiac morphology and function using cardiac magnetic resonance imaging (CMRI).

Methods and results

Thirty consecutive patients with BrS were compared with 30 sex- (26/4 male/female), body surface area- (±0.2 m2), and age-matched (±5 years) normal volunteers. CMRI exam included long- and short-axis ECG-gated breath-hold morphological T1-TSE sequences for fatty infiltration and cine-SSFP sequences for kinetic assessment. Fatty infiltration was not found in any subject. Patients with BrS compared with normal subjects showed higher incidence of mild right ventricle (RV) wall-motion abnormalities [15 (50%) vs. 5 (17%) subjects (P = 0.006) with reduced radial fractional shortening in more than two segments], reduction of outflow tract ejection fraction (49 ± 11% vs. 55 ± 10%; P = 0.032), enlargement of the inflow tract diameter (46 ± 4 vs. 41 ± 5 mm, P < 0.001 in short-axis; 46 ± 4 vs. 42 ± 5 mm, P = 0.001 in four-chamber long-axis view) and area (22 ± 2 vs. 20 ± 3 cm2; P = 0.050), and of global RV end-systolic volume (34 ± 10 vs. 30 ± 6 mL/m2; P = 0.031) but comparable outflow tract dimensions, global RV end-diastolic volume, left ventricle parameters, and atria areas.

Conclusion

CMRI detects a high prevalence of mild structural changes of the RV, and suggests further pathophysiological complexity in BrS. Prospective studies to assess the long-term evolution of such abnormalities are warranted.

Keywords: Brugada syndrome; Cardiac magnetic resonance imaging; Sudden cardiac death; SCN5A

Journal Article.  4776 words.  Illustrated.

Subjects: Cardiovascular Medicine

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