• METHODS: In this study, we performed clinical exome sequencing or gene panel including cardiac arrhythmia and cardiomyopathy-associated genes by next-generation sequenc-ing in 13 unrelated patients. (bvsalud.org)
  • The possibility of cardiac arrhythmia was suspected after the patient presented to the emergency room and the electrocardiograph (ECG) monitor showed paroxysmal ventricular tachycardia during attacks. (biomedcentral.com)
  • it is characterized by syncope and a high incidence of ventricular arrhythmia (including torsades de pointes) and sudden cardiac death. (biomedcentral.com)
  • The patient has never been suspected LQTS because her normal routine 12 channel electrocardiograph (ECG) at early disease stage, until we caught clinical attacks during her initial visit to our emergency room (ER) and documented simultaneous cardiac arrhythmia. (biomedcentral.com)
  • Ventricular arrhythmia (VA) may be an isolated and completely benign finding in children, a marker of serious systemic disease or myopathy, or a mechanism for syncope and sudden cardiac death (SCD). (medscape.com)
  • Our case illustrates how long LQTS can masquerade convincingly as epilepsy and can be treated wrongly with AEDs, putting the patient at high risk of sudden cardiac death. (biomedcentral.com)
  • It is necessary to study with a broad perspective to elucidate the underly- ing molecular etiology in patients with hereditary cardiac arrhythmias. (bvsalud.org)
  • Multivariate analysis was carried out to identify age-related gender- and genotype-specific risk factors for cardiac events (comprising syncope, aborted cardiac arrest [ACA] or sudden cardiac death [SCD]) from birth through age 40 years among 971 LQT1 (n = 549) and LQT2 (n = 422) patients from the International LQTS Registry. (medscape.com)
  • It is characterized by electrocardiographic abnormalities and a high incidence of syncope and sudden cardiac death. (medscape.com)
  • Implantable Cardioverter Defibrillator (ICD) is used for those who failed medications, or present with aborted cardiac arrest. (criticalcarenow.com)
  • The number of oncological patients who may benefit from proton beam radiotherapy (PBT) or carbon ion radiotherapy (CIRT), overall referred to as particle radiotherapy (RT), is expected to strongly increase in the next future, as well as the number of cardiological patients requiring cardiac implantable electronic devices (CIEDs). (bvsalud.org)
  • Flecainide can be added for primary prevention of a cardiac arrest when beta blockers alone cannot control the onset of arrhythmias during an exercise stress test. (nih.gov)
  • This syndrome, once diagnosed by clinical profile, has been more clearly defined by specific genetic defects that cause ion channel abnormalities, resulting in a syndrome that predisposes to lethal cardiac arrhythmias. (medscape.com)
  • The management of long-QT syndrome (LQTS) patients who continue to have cardiac events (CEs) despite beta-blockers is complex. (nih.gov)
  • The congenital long-QT syndrome (LQTS) is a genetic channelopathy with variable penetrance that is associated with increased propensity for polymorphic ventricular tachyarrhythmias and sudden cardiac death (SCD) in young individuals with normal cardiac morphology. (medscape.com)
  • Indeed, sudden cardiac death in the pediatric population can be the first presentation of an underlying heart problem. (medscape.com)
  • Abnormal cardiac repolarization renders the heart susceptible to these lethal ventricular tachyarrhythmias, increasing the risk of sudden cardiac death in patients of all ages. (medscape.com)
  • Ventricular tachycardia (VT) or ventricular fibrillation (VF) is responsible for most of the sudden cardiac deaths in the United States,[1] at an estimated rate of approximately 300,000 deaths per year. (medscape.com)
  • CPVT is caused by mutations in the cardiac ryanodine receptor gene (RyR2) and calsequestrin isoform 2 gene (CASQ2). (criticalcarenow.com)
  • Sympathetic activity is thought to enhance the EADs, which, in turn, can initiate a lethal form of ventricular arrhythmia termed torsade de pointes. (medscape.com)
  • Long QT syndrome is a genetically transmitted cardiac arrhythmia caused by ion channel protein abnormalities. (medscape.com)
  • Some synonymous and nonsynonymous exonic SNPs identified in long QT syndrome-causing genes may have an effect on the cardiac repolarization process and may modulate the clinical expression of a latent long QT syndrome pathogenic mutation. (medscape.com)
  • [ 5 ] Despite the sympathetic mechanisms associated with the 2 main LQTS genotypes, previous studies that assessed efficacy of beta-blocker therapy in LQT1 and LQT2 patients have suggested important limitations of this mode of medical therapy among treated patients. (medscape.com)
  • If untreated, CPVT is highly lethal, as approximately 30% of affected individuals experience at least one cardiac arrest and up to 80% have one or more syncopal spells. (nih.gov)