Pathomorphological criteria of arrhythmogenic heart include structural compartmentalization with primary changes in the right ventricle and interventricular septum, fibro- and lipomatosis of the myocardium, and disseminated coronary obstruction. Ischemic focuses in the conducting system are the site of formation of arrhythmogenic substance promoting the development and progression of cardiac arrhythmias. Cardioneuropathy and pathological motility of the interventricular septum lead to systolic dysfunction and contribute to asynchronous excitation and contraction of ventricles in arrhythmogenic heart.
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http://dx.doi.org/10.1023/a:1026061501921 | DOI Listing |
Cell Discov
January 2025
Centro Nacional de Investigaciones Cardiovasculares (CNIC), Madrid, Spain.
A well-balanced ion channel trafficking machinery is paramount for the normal electromechanical function of the heart. Ion channel variants and many drugs can alter the cardiac action potential and lead to arrhythmias by interfering with mechanisms like ion channel synthesis, trafficking, gating, permeation, and recycling. A case in point is the Long QT syndrome (LQTS), a highly arrhythmogenic disease characterized by an abnormally prolonged QT interval on ECG produced by variants and drugs that interfere with the action potential.
View Article and Find Full Text PDFCurr Cardiol Rep
January 2025
Department of Internal Medicine, Division of Cardiovascular Medicine, University of Michigan, Ann Arbor, MI, USA.
Purpose Of Review: To summarize the available data on the use of immunosuppression therapies for the management of hot phases of disease and recurrent myocarditis in patients with desmoplakin cardiomyopathy (DSP-CMP).
Recent Findings: Occurrence of myocarditis episodes has been associated with worsening of outcomes in DSP-CMP. Multiple case reports and small case series have described potential benefit in using anti-inflammatory and immunosuppressive medications for the treatment of those episodes.
Children (Basel)
November 2024
Department of Pediatrics, Faculty of Medicine, "Grigore T. Popa" University of Medicine and Pharmacy, 700115 Iasi, Romania.
A physically active lifestyle offers multiple benefits, including lowering the risk of cardiovascular disease, lowering body-mass index (BMI), and, last but not least, improving the quality of life. However, there are still disincentives to physical activity in children with heart diseases due to the high protection of parents and the scarcity of data in the literature. The purpose of this paper is to help pediatricians and pediatric cardiologists identify the type of physical activity allowed in children with congenital cardiac malformations, thus minimizing the risk of major adverse effects, such as acute coronary syndrome and sudden cardiac death.
View Article and Find Full Text PDFBiomolecules
December 2024
Department of Anatomy, Animal Physiology and Biophysics, Faculty of Biology, University of Bucharest, Splaiul Independentei 91-95, 050095 Bucharest, Romania.
Cenobamate is a novel third-generation antiepileptic drug used for the treatment of focal onset seizures and particularly for multi-drug-resistant epilepsy; it acts on multiple targets: GABA receptors (EC 42-194 µM) and persistent neuronal Na currents (IC 59 µM). Side effects include QT interval shortening with >20 ms, but not <300 ms. Our in vitro cardiac safety pharmacology study was performed via whole-cell patch-clamp on HEK293T cells with persistent/inducible expression of human cardiac ion channel isoforms hNav1.
View Article and Find Full Text PDFArrhythmogenic cardiomyopathy (ACM) is a genetic form of heart failure that affects 1 in 5000 people globally and is caused by mutations in cardiac desmosomal proteins including , and Individuals with ACM suffer from ventricular arrhythmias, sudden cardiac death, and heart failure. There are few effective treatments and heart transplantation remains the best option for many affected individuals. Here we performed single nucleus RNA sequencing (snRNAseq) and spatial transcriptomics on myocardial samples from patients with ACM and control donors.
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