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

被引:132
作者
Catalano, Oronzo [1 ]
Antonaci, Serena [1 ]
Moro, Guido [2 ]
Mussida, Maria [1 ]
Frascaroli, Mauro [2 ]
Baldi, Maurizia [2 ]
Cobelli, Franco [1 ]
Baiardi, Paola [4 ]
Nastoli, Janni
Bloise, Raffaella
Monteforte, Nicola
Napolitano, Carlo
Priori, Silvia G. [3 ]
机构
[1] Fdn Salvatore Maugeri, IRCCS, Div Cardiol, I-27100 Pavia, Italy
[2] Fdn Salvatore Maugeri, IRCCS, Div Radiol, I-27100 Pavia, Italy
[3] Univ Pavia, Dept Cardiol, I-27100 Pavia, Italy
[4] Fdn Salvatore Maugeri, IRCCS, Consorzio Valutaz Biol & Farmacol, I-27100 Pavia, Italy
关键词
Brugada syndrome; Cardiac magnetic resonance imaging; Sudden cardiac death; SCN5A; ST-SEGMENT ELEVATION; BUNDLE-BRANCH BLOCK; HEART-FAILURE; CARDIOMYOPATHY; MUTATIONS; ENHANCEMENT; DEATH;
D O I
10.1093/eurheartj/ehp252
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
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). Thirty consecutive patients with BrS were compared with 30 sex- (26/4 male/female), body surface area- (+/- 0.2 m(2)), 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 cm(2); P = 0.050), and of global RV end-systolic volume (34 +/- 10 vs. 30 +/- 6 mL/m(2); P = 0.031) but comparable outflow tract dimensions, global RV end-diastolic volume, left ventricle parameters, and atria areas. 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.
引用
收藏
页码:2241 / 2248
页数:8
相关论文
共 27 条
[1]   Brugada syndrome - Report of the second consensus conference - Endorsed by the Heart Rhythm Society and the European Heart Rhythm Association [J].
Antzelevitch, C ;
Brugada, P ;
Borggrefe, M ;
Brugada, J ;
Brugada, R ;
Corrado, D ;
Gussak, I ;
LeMarec, H ;
Nademanee, K ;
Riera, ARP ;
Shimizu, W ;
Schulze-Bahr, E ;
Tan, H ;
Wilde, A .
CIRCULATION, 2005, 111 (05) :659-670
[2]   Brugada syndrome: From cell to bedside [J].
Antzelevitch, C ;
Brugada, P ;
Brugada, J ;
Brugada, R .
CURRENT PROBLEMS IN CARDIOLOGY, 2005, 30 (01) :9-54
[3]   The Brugada syndrome: Ionic basis and arrhythmia mechanisms [J].
Antzelevitch, C .
JOURNAL OF CARDIOVASCULAR ELECTROPHYSIOLOGY, 2001, 12 (02) :268-272
[4]   Loss-of-function mutations in the cardiac calcium channel underlie a new clinical entity characterized by ST-Segment elevation, short QT intervals, and sudden cardiac death [J].
Antzelevitch, Charles ;
Pollevick, Guido D. ;
Cordeiro, Jonathan M. ;
Casis, Oscar ;
Sanguinetti, Michael C. ;
Aizawa, Yoshiyasu ;
Guerchicoff, Alejandra ;
Pfeiffer, Ryan ;
Oliva, Antonio ;
Wollnik, Bernd ;
Gelber, Philip ;
Bonaros, Elias P., Jr. ;
Burashnikov, Elena ;
Wu, Yuesheng ;
Sargent, John D. ;
Schickel, Stefan ;
Oberheiden, Ralf ;
Bhatia, Atul ;
Hsu, Li-Fern ;
Haissaguerre, Michel ;
Schimpf, Rainer ;
Borggrefe, Martin ;
Wolpert, Christian .
CIRCULATION, 2007, 115 (04) :442-449
[5]   The cardiac sodium channel: Gating function and molecular pharmacology [J].
Balser, JR .
JOURNAL OF MOLECULAR AND CELLULAR CARDIOLOGY, 2001, 33 (04) :599-613
[6]   Reduction in sample size for studies of remodeling in heart failure by the use of cardiovascular magnetic resonance [J].
Bellenger, NG ;
Davies, LC ;
Francis, JM ;
Coats, AJS ;
Pennell, DJ .
JOURNAL OF CARDIOVASCULAR MAGNETIC RESONANCE, 2000, 2 (04) :271-278
[7]   Agreement between methods of measurement with multiple observations per individual [J].
Bland, J. Martin ;
Altman, Douglas G. .
JOURNAL OF BIOPHARMACEUTICAL STATISTICS, 2007, 17 (04) :571-582
[8]   RIGHT BUNDLE-BRANCH BLOCK, PERSISTENT ST SEGMENT ELEVATION AND SUDDEN CARDIAC DEATH - A DISTINCT CLINICAL AND ELECTROCARDIOGRAPHIC SYNDROME - A MULTICENTER REPORT [J].
BRUGADA, P ;
BRUGADA, J .
JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY, 1992, 20 (06) :1391-1396
[9]   Genetic basis and molecular mechanism for idiopathic: ventricular fibrillation [J].
Chen, QY ;
Kirsch, GE ;
Zhang, DM ;
Brugada, R ;
Brugada, J ;
Brugada, P ;
Potenza, D ;
Moya, A ;
Borggrefe, M ;
Breithardt, G ;
Ortiz-Lopez, R ;
Wang, Z ;
Antzelevitch, C ;
O'Brien, RE ;
Schulze-Bahr, E ;
Keating, MT ;
Towbin, JA ;
Wang, Q .
NATURE, 1998, 392 (6673) :293-296
[10]   Familial cardiomyopathy underlies syndrome of right bundle branch block, ST segment elevation and sudden death [J].
Corrado, D ;
Nava, A ;
Buja, G ;
Martini, B ;
Fasoli, G ;
Oselladore, L ;
Turrini, P ;
Thiene, G .
JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY, 1996, 27 (02) :443-448