Abstract
The electrophysiological signatures of the myocardium in cardiac structures, such as the atrioventricular node, pulmonary veins or the right ventricular outflow tract, are established during development by the spatial and temporal expression of transcription factors that guide expression of specific ion channels. Genome-wide association studies have shown that small variations in genetic regions are key to the expression of these transcription factors and thereby modulate the electrical function of the heart. Moreover, mutations in these factors are found in arrhythmogenic pathologies such as congenital atrioventricular block, as well as in specific forms of atrial fibrillation and ventricular tachycardia. In this review, we discuss the developmental origin of distinct electrophysiological structures in the heart and their involvement in cardiac arrhythmias.
Highlights
Components of the heart with distinct electrophysiological signatures ensure controlled electrical impulse formation and propagation, and coordinated ventricular activation generating sufficient cardiac output required to maintain body homeostasis
The combination of morphology and ion channel make-up can provide different regions of the myocardium, such as the atrioventricular node, the myocardial sleeve of the pulmonary veins or the right ventricular outflow tract (RVOT), with electrophysiological signatures that distinguish them from the working myocardium
We discuss the role of these transcription factors in atrioventricular node function, arrhythmias based on the presence of accessory pathways and arrhythmias originating in the RVOT or pulmonary veins
Summary
Components of the heart with distinct electrophysiological signatures ensure controlled electrical impulse formation and propagation, and coordinated ventricular activation generating sufficient cardiac output required to maintain body homeostasis. The combination of morphology and ion channel make-up can provide different regions of the myocardium, such as the atrioventricular node, the myocardial sleeve of the pulmonary veins or the right ventricular outflow tract (RVOT), with electrophysiological signatures that distinguish them from the working myocardium. Variations in regulatory genetic regions or mutations in genes encoding these transcription factors may alter their expression levels and predispose them to or even cause cardiac arrhythmias originating in these areas [3,4,5,6,7]. We discuss the role of these transcription factors in atrioventricular node function, arrhythmias based on the presence of accessory pathways and arrhythmias originating in the RVOT or pulmonary veins
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