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Treatment Outcomes in Children With Catecholaminergic Polymorphic Ventricular Tachycardia: A Single Institutional Experience.
Korean Circ J
December 2024
Department of Pediatrics, Seoul National University Children's Hospital, Seoul National University College of Medicine, Seoul, Korea.
Background And Objectives: Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a life-threatening inherited arrhythmogenic disorder. Recently, , the major CPVT-causative gene, was associated with neuropsychiatric manifestations. We aimed to analyze the clinical presentations, neuropsychiatric manifestations, and treatment outcomes of children with CPVT.
View Article and Find Full Text PDFPrognostic Value of Strain by Speckle Tracking Echocardiography in Patients with Arrhythmogenic Right Ventricular Cardiomyopathy.
J Cardiovasc Dev Dis
December 2024
Institute of Cardiovascular Sciences, University of Birmingham, Birmingham B15 2TT, UK.
Background Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a rare genetic disorder associated with an elevated risk of life-threatening arrhythmias and progressive ventricular impairment. Risk stratification is essential to prevent major adverse cardiac events (MACE). Our study aimed to investigate the incremental value of strain measured by two-dimensional speckle-tracking echocardiography in predicting MACE in ARVC patients compared to conventional echocardiographic parameters.
View Article and Find Full Text PDFFour cardiomyopathy patients with a heterozygous DSG2 p.Arg119Ter variant.
Hum Genome Var
December 2024
Department of Cardiovascular Medicine, Osaka University Graduate School of Medicine, Suita, Osaka, 565-0871, Japan.
DSG2, encoding desmoglein-2, is one of the causative genes of arrhythmogenic cardiomyopathy. We previously identified a homozygous DSG2 p.Arg119Ter stop-gain variant in a patient with juvenile-onset cardiomyopathy and advanced biventricular heart failure.
View Article and Find Full Text PDFIntegration of a surgical bipolar ablation device within an electroanatomical mapping system during epicardial ventricular tachycardia ablation via video-assisted thoracoscopic surgery: a case report.
Eur Heart J Case Rep
December 2024
Liverpool Centre for Cardiovascular Science at University of Liverpool, Liverpool John Moores University and Liverpool Heart & Chest Hospital, William Henry Duncan Building, 6 W Derby St, Liverpool L7 8TX, UK.
Background: Epicardial ventricular tachycardia (VT) ablation is an established approach in patients with epicardial arrhythmogenic foci and is most commonly performed via percutaneous access. An alternative approach is via video-assisted thoracoscopic surgery (VATS), although reports of this technique are limited to the use of catheter-based technologies for radiofrequency ablation delivery.
Case Summary: A 55-year-old man with non-ischaemic cardiomyopathy presented with recurrent VT despite medical therapy.
Strain Analysis for Early Detection of Fibrosis in Arrhythmogenic Cardiomyopathy: Insights from a Preliminary Study.
J Clin Med
December 2024
Department of Cardiac, Thoracic, Vascular Sciences and Public Health, University of Padova, 35128 Padova, Italy.
Arrhythmogenic cardiomyopathy (ACM) is a genetic disorder characterized by fibrofatty replacement of myocardial tissue, predominantly affecting the right ventricle (RV), but often involving the left ventricle (LV) as well. The early detection of fibrosis, crucial for risk stratification, has been enhanced by advanced imaging techniques. Global longitudinal strain (GLS) has shown promise as a surrogate marker for late enhancement (LE) in identifying myocardial fibrosis, yet precise cut-off values for strain are lacking.
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