Stirbys Petras
Dept. of Cardiology, Hospital of Lithuanian University of Health Sciences , Kaunas Clinic, Kaunas, Lithuania. The rest of it is non-sense.
J Atr Fibrillation. 2017 Apr 30;9(6):1554. doi: 10.4022/jafib.1554. eCollection 2017 Apr-May.
The Brugada syndrome (BrS) is associated with increased risk of ventricular arrhythmias and sudden cardiac death. It generates genetically mediated arrhythmias posing a true pathophysiological challenge. In search of the similarities between BrS and long QT syndrome some novel insights are suggested. In patients with BrS the duration of QT interval is usually normal. Some investigators have found prolonged QT interval in the syndrome's natural course or the duration of QT segment have been extended by provocative tests unmasking BrS. Thus, BrS might be characterized as "long QT sine long QT" syndrome. The existence of two functional types of myocites is suspected. Regarding structure and function the majority of ventricular myocardium is probably mostly healthy. The rest of myocardium (preferably the subepicardium of right ventricular outflow tract) due to its genotypic peculiarities demonstrates no negative influence on ventricular performance until early adulthood is reached and/or other unstable preconditions are fulfilled (nocturnal time, fever, specific drugs, etc.). Based on published findings of positive outcomes, following the epicardial ablation of the right ventricular outflow tract region, a new hypothetical concept suggesting the presence of specific, genetically affected "Brugada's myocells" is proposed. These cells as a suitable arrhythmogenic substrate reside intramurally within the subepicardial region of the outflow tract of right ventricle. In the daytime these cells likely are dormant but at rest their nocturnal proarrhythmic behavior is activated occasionally. Presumptions regarding the pathophysiology of BrS might be the focus of further discussion.
Brugada综合征（BrS）与室性心律失常及心源性猝死风险增加相关。它会引发由基因介导的心律失常，这构成了真正的病理生理学挑战。在探寻BrS与长QT综合征之间的相似性时，有了一些新的见解。BrS患者的QT间期通常正常。一些研究者发现在该综合征的自然病程中QT间期延长，或者通过激发试验使BrS显现时QT段时长会延长。因此，BrS可能被描述为“无长QT的长QT”综合征。人们怀疑存在两种功能类型的心肌细胞。就结构和功能而言，大部分心室肌可能基本健康。其余心肌（尤其是右心室流出道的心外膜下部分）由于其基因型特点，在成年早期到来之前和/或满足其他不稳定前提条件（夜间、发热、特定药物等）之前，对心室功能没有负面影响。基于已发表的积极结果，在对右心室流出道区域进行心外膜消融后，提出了一个新的假设概念，即存在特定的、受基因影响的“Brugada心肌细胞”。这些细胞作为合适的致心律失常基质位于右心室流出道心外膜下区域的壁内。在白天这些细胞可能处于休眠状态，但在夜间休息时它们偶尔会被激活而产生促心律失常行为。关于BrS病理生理学的推测可能是进一步讨论的焦点。
