A novel homozygous mutation in the TRDN gene causes a severe form of pediatric malignant ventricular arrhythmia

Triadin is a protein expressed in cardiac and skeletal muscle with an essential role in the structure and functional regulation of calcium release units and excitation-contraction coupling. Mutations in the triadin gene (TRDN) have been described in different forms of human arrhythmia syndromes with early onset and severe arrhythmogenic phenotype, among which the triadin knockout syndrome.
Source: Heart Rhythm - Category: Cardiology Authors: Source Type: research

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CONCLUSION: Collectively, these findings suggest that the increased ventricular arrhythmia susceptibility of type 2 diabetic mouse hearts is due to dysregulation of the sympathetic ventricular control. PMID: 31625779 [PubMed - as supplied by publisher]
Source: American Journal of Physiology. Heart and Circulatory Physiology - Category: Physiology Authors: Tags: Am J Physiol Heart Circ Physiol Source Type: research
ConclusionsAll MVT cases were successfully treated by catheter ablation. We observed high ventricular arrhythmia free rate following catheter ablation during the long-term follow-up period. BrS patients who developed MVT should consider catheter ablation.
Source: Journal of Interventional Cardiac Electrophysiology - Category: Cardiology Source Type: research
Adults with repaired Tetralogy of Fallot (rTOF) are at increased risk of ventricular tachycardia (VT) due to fibrotic remodeling of the myocardium. However, the current clinical guidelines for VT risk stratification and subsequent ICD deployment for primary prevention of sudden cardiac death in rTOF remain inadequate.
Source: Heart Rhythm - Category: Cardiology Authors: Source Type: research
This article reviews current knowledge regarding drug therapy for inherited arrhythmia syndromes (Brugada, early repolarization, long QT and short QT syndromes, and catecholaminergic polymorphic ventricular tachycardia), and acquired arrhythmias (idiopathic ventricular fibrillation, short-coupled torsade de pointes, outflow tract ventricular tachycardia, idiopathic left, papillary muscle and annular ventricular tachycardias). Expert opinion: In inherited arrhythmia syndromes, appropriate clinical and genetic diagnoses followed by proper selection and dosing of antiarrhythmic drugs are of utmost importance to prevent SCD, m...
Source: Expert Opinion on Pharmacotherapy - Category: Drugs & Pharmacology Tags: Expert Opin Pharmacother Source Type: research
We present a group of 12 patients (7 boys and 5 girls) aged 6 to 22 years. The age of detection ranged from 2 to 8 years, and patients complaint on syncope - 4 patients (33%), cleft palate - 3 patients (25%), periodic paralysis - 2 patients (16,7%), polymorphic ventricular arrhythmia - 3 patients (25%).
Source: Neuromuscular Disorders - Category: Neurology Authors: Source Type: research
We report the case of a 65-year-old man brought in by ambulance who complained of chest pain and received an out-of-hospital ECG suggestive of anterior-wall ST-segment elevation myocardial infarction. Shortly after arrival, the patient became unresponsive, with no palpable pulse, and was found to be in ventricular fibrillation. The patient’s ventricular fibrillation persisted despite repeated attempts at standard and double sequential defibrillation, multiple rounds of epinephrine, and amiodarone, magnesium, and bicarbonate. After these interventions were exhausted, a stellate ganglion blockade was conducted after an...
Source: Annals of Emergency Medicine - Category: Emergency Medicine Source Type: research
We report diagnosis modalities and evolution. PMID: 31558269 [PubMed - as supplied by publisher]
Source: Annales de Cardiologie et d'Angeiologie - Category: Cardiology Authors: Tags: Ann Cardiol Angeiol (Paris) Source Type: research
New England Journal of Medicine,Volume 381, Issue 12, Page 1180-1181, September 2019.
Source: New England Journal of Medicine - Category: Internal Medicine Authors: Source Type: research
Abstract Exacerbation of chronic heart failure (HF) is the most common cause of hospitalization in adults, which is associated with high morbidity and mortality, mainly in the mechanism of exacerbation of chronic HF or sudden cardiac death (SCD). A novelty in the treatment of HF with reduced left ventricular ejection fraction (HFrEF) in recent years has been registration of sacubitril/valsartan - a drug belonging to the angiotensin II receptor and neprilysin inhibitors (ARNI). Sacubitril/valsartan significantly reduces the severity of HF symptoms, the risk of hospitalization and death and is characterized by a goo...
Source: Polish Heart Journal - Category: Cardiology Authors: Tags: Kardiol Pol Source Type: research
Publication date: Available online 15 September 2019Source: Journal of Critical CareAuthor(s): Hollie Russell, Leonid Churilov, Lisa Toh, Glenn M. Eastwood, Rinaldo BellomoAbstractPurposeTo study the incidence, predictors and outcomes of QTc prolongation (≥500 ms) during ICU admission.MethodsProspective observational study of patients admitted to a tertiary ICU during a two-month period. We obtained daily data on QTc intervals and arrhythmias from ICU monitors. We performed univariate and multivariable analyses to compare patients who did or did not experience QTc prolongation.ResultsOf the 257 patients, 93 (36.2%) de...
Source: Journal of Critical Care - Category: Gastroenterology Source Type: research
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