Electroanatomic and Pathologic Right Ventricular Outflow Tract Abnormalities in Patients With Brugada Syndrome
Electroanatomic and Pathologic Right Ventricular Outflow Tract Abnormalities in Patients With Brugada Syndrome
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DOI：
10.1016/j.jacc.2018.09.037
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发表时间：
2018-12-04
影响因子：
24
通讯作者：
Bolognese, Leonardo
中科院分区：
文献类型：
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作者：
Pieroni, Maurizio;Notarstefano, Pasquale;Bolognese, Leonardo
背景Brugada综合征（BrS）结构异常的患病率和意义仍有很大争议。结论作者研究了BrS的遗传背景、电解剖异常和病理基质之间的关系。方法对30例BrS患者进行了三维电解剖单极和双极标测。20例患者接受了三维电解剖单极和双极标测引导的右心室流出道（RVOT）肌内膜活检。结果93%的患者在单极标测图上出现低电压区（LVAs），50%的患者在双极标测图上出现低电压区。单极LVA总是大于双极LVA，总是共定位，并且在所有情况下都包括RVOT。在10例（33%）患者中检测到致病突变。程序心室刺激阳性16例（53%）。在75%的患者中，RVOT组织学显示病理结果，其中80%的患者伴有心肌炎症。在接受肌内膜活检的双极标测异常患者中，9例（81%）显示心肌炎症证据。相反，83%的心肌炎症患者的双极标测图异常。心肌炎症在诱导型患者中也更为普遍（83% vs. 25%在noninducible; p = 0.032）。结论BrS的特点是定位于RVOT的电解剖和结构异常，从心外膜到内膜的病理基质梯度可能由心肌炎症驱动。这些发现将BrS重新分类为结构和电缺陷的组合，为新的风险分层和治疗策略开辟了道路。(c)2018年由美国心脏病学会基金会。
BACKGROUND The prevalence and significance of structural abnormalities in Brugada syndrome (BrS) are still largely debated.OBJECTIVES The authors investigated the relationship between genetic background, electroanatomic abnormalities, and pathologic substrate in BrS.METHODS They performed 3-dimensional electroanatomic unipolar and bipolar mapping in 30 patients with BrS. Twenty patients underwent 3-dimensional electroanatomic unipolar and bipolar mapping-guided right ventricular outflow tract (RVOT) endomyocardial biopsy. Programmed ventricular stimulation and genetic analysis were performed in all patients.RESULTS Low-voltage areas (LVAs) were observed at unipolar map in 93% of patients and at bipolar map in 50% of cases. Unipolar LVAs were always larger than bipolar LVAs, were always colocalized, and in all cases included RVOT. Disease-causing mutations were detected in 10 (33%) patients. Programmed ventricular stimulation was positive in 16 cases (53%). In 75% of patients, RVOT histology showed pathologic findings with myocardial inflammation in 80% of them. Among patients with abnormal bipolar map submitted to endomyocardial biopsy, 9 (81%) showed evidence of myocardial inflammation. Conversely, bipolar map was abnormal in 83% of patients with myocardial inflammation. Myocardial inflammation was also more prevalent among inducible patients (83% vs. 25% in noninducible; p = 0.032).CONCLUSIONS BrS is characterized by electroanatomical and structural abnormalities localized to RVOT with a gradient of the pathologic substrate from epicardium to endocardium possibly driven by myocardial inflammation. These findings reclassify BrS as a combination of structural and electrical defects opening the way to new risk stratification and therapeutic strategies. (c) 2018 by the American College of Cardiology Foundation.