The cardiac Purkinje system is capable of very rapid burst activity suggestive of its potential role in being a driver of polymorphic ventricular tachycardia (VT) (PMVT) or ventricular fibrillation (VF). It plays a pivotal role, however, not only in the triggering of but also the perpetuation of ventricular arrhythmias. A varying degree of Purkinje-myocardial complicity has been blamed in determining not only the sustained or non-sustained nature of PMVT but also the pleomorphism of the non-sustained runs. The initial part of PMVT before cascading to the whole ventricle to establish disorganized VF can give important clues for ablation of PMVT and VF. We present a case of an electrical storm after acute myocardial infarction that was successfully ablated after identifying Purkinje potentials that triggered polymorphic, monomorphic, and pleiomorphic VTs and VF.
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http://dx.doi.org/10.19102/icrm.2023.14066 | DOI Listing |
Purpose Of Review: Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a devastating heritable channelopathy that can lead to sudden cardiac death in children and young adults. This review aims to explore genetics, the cardiac and extracardiac manifestations of mutations associated with CPVT, and the challenges involved with managing phenotypically variable variants.
Recent Findings: The understanding of the genetics and mechanisms of CPVT continues to grow with recent discoveries including alternative splicing of cardiac TRDN and calmodulin gene variants.
J Cardiol Cases
October 2024
Department of Community Medicine and Medical Science, Tokushima University Graduate School of Biomedical Sciences, Tokushima, Japan.
Unlabelled: A 93-year-old man was admitted to our hospital with complaints of pain and coldness in both lower legs. He was diagnosed with arterial occlusive disease of the lower extremities. There were no obvious stenosis or occlusion of lower extremity arteries, which would be indications for surgery, and drug therapy was started.
View Article and Find Full Text PDFStem Cell Res
December 2024
Agnes Ginges Centre for Molecular Cardiology at Centenary Institute, The University of Sydney, Sydney, Australia; Faculty of Medicine and Health, The University of Sydney, Sydney, Australia. Electronic address:
Catecholaminergic polymorphic ventricular tachycardia (CPVT) is an inherited cardiac disease characterised by adrenergic-induced arrhythmias. The leading causes of CPVT are pathogenic variants in cardiac ryanodine receptor 2 (RYR2) and rarely, in cardiac calsequestrin-2 (CASQ2) genes, which are major components of Ca handling in cardiac myocytes. This resource builds upon an established induced pluripotent stem cell line generated from a family with autosomal dominant CPVT due to a heterozygous variant in CASQ2 c.
View Article and Find Full Text PDFActa Physiol (Oxf)
February 2025
Department of Biochemistry, Cell and Systems Biology, Institute of Systems, Molecular and Integrative Biology, Faculty of Health and Life Sciences, University of Liverpool, Liverpool, UK.
Aim: Long QT syndrome (LQTS) and catecholaminergic polymorphism ventricular tachycardia (CPVT) are inherited cardiac disorders often caused by mutations in ion channels. These arrhythmia syndromes have recently been associated with calmodulin (CaM) variants. Here, we investigate the impact of the arrhythmogenic variants D131E and Q135P on CaM's structure-function relationship.
View Article and Find Full Text PDFJACC Case Rep
January 2025
Division of Cardiology, University of Colorado Anschutz Medical Campus, Aurora, Colorado, USA.
Structural abnormalities within the right ventricular outflow tract (RVOT) can present similarly to Brugada syndrome. A 34-year-old woman with no medical history presented with polymorphic ventricular tachycardia/ventricular fibrillation cardiac arrest and initial electrocardiogram showed type I Brugada pattern. Cardiac magnetic resonance imaging revealed prominent tissue thickening at the RVOT with late gadolinium enhancement.
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