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

Magnetic resonance investigations in Brugada syndrome reveal unexpectedly high rate of structural abnormalities
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DOI:
10.1093/eurheartj/ehp252
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发表时间:
2009-09-01
影响因子:
39.3
通讯作者:
Priori, Silvia G.
Priori, Silvia G.
中科院分区:
医学1区
文献类型:
--
作者:
Catalano, Oronzo;Antonaci, Serena;Priori, Silvia G.

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最近的数据表明,亚临床结构异常可能是Brugada综合征(BrS)表型的一部分,这种疾病传统上被认为发生在结构正常的心脏中。在这项研究中,我们使用心脏磁共振成像(CMRI)对心脏形态和功能进行了详细的评估。30名连续的BrS患者与30名性别(26/4男性/女性),体表面积(+/- 0.2 m2),年龄匹配(+/- 5岁)的正常志愿者进行了比较。CMRI检查包括用于脂肪浸润的长轴和短轴ECG门控屏气形态T1-TSE序列和用于动力学评估的电影SSFP序列。所有受试者均未发现脂肪浸润。与正常受试者相比,BrS患者显示轻度右心室(RV)室壁运动异常的发生率较高[15例(50%)vs. 5例(17(P = 0.006)两个以上节段的径向缩短率降低],流出道射血分数降低(49 +/- 11% vs. 55 +/- 10%; P = 0.032),流入道直径扩大(短轴为46 ± 4 mm对41 ± 5 mm,P < 0.001; 46 +/- 4 vs. 42 +/- 5 mm,四腔长轴视图中P = 0.001)和面积(22 +/- 2 vs. 20 +/- 3 cm(2); P = 0.050),以及整体RV收缩末期容积(34 +/- 10 vs. 30 +/- 6 mL/m(2); P = 0.031),但流出道尺寸、总体RV舒张末期容积、左心室参数,CMRI检测到RV的轻度结构变化的高患病率,并提示BrS的进一步病理生理复杂性。前瞻性研究,以评估这种异常的长期演变是必要的。
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.