Brief communicationInfants with long-QT syndrome and 2:1 atrioventricular block☆
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Cited by (73)
Genotype Predicts Outcomes in Fetuses and Neonates With Severe Congenital Long QT Syndrome
2020, JACC: Clinical ElectrophysiologyLong QT syndrome with a functional 2:1 block and multilevel conduction disease
2018, Progress in Pediatric CardiologyCitation Excerpt :In the case we describe, present also is the important but uncommon characteristic of a functional 2:1 AV block. Patients presenting as such have been reported sporadically over recent decades [8]. As in this case, the non-conducted P-waves occur at the onset of, or even prior to, the preceding T-wave [7].
Comorbid Epilepsy and Developmental Disorders in Congenital Long QT Syndrome With Life-Threatening Perinatal Arrhythmias
2016, JACC: Clinical ElectrophysiologyCitation Excerpt :Of 3,323 LQTS patients in an international registry, sudden cardiac death occurred in only 20 (0.6%), aborted cardiac arrest in 16 (0.4%), and syncope in 34 (1%) during the first year of life (1). However, these patients are known to be at a very high risk of aborted cardiac arrest or sudden death in the years to come, especially those with LQTS plus TdP or 2:1 AVB during the perinatal period (1–5). In the present study, during the follow-up, more arrhythmic events were observed in the perinatal LQTS patients despite more intensive treatment.
Na<inf>V</inf>1.5 and regulatory β subunits in cardiac sodium channelopathies
2014, Cardiac Electrophysiology Clinics2012 ACCF/AHA/HRS focused update incorporated into the ACCF/AHA/HRS 2008 guidelines for device-based therapy of cardiac rhythm abnormalities: A report of the American college of cardiology foundation/American heart association task force on practice guidelines and the heart rhythm society
2013, Journal of the American College of CardiologyLong QT Syndrome in the Very Young: Fetal Life Through Infancy
2012, Cardiac Electrophysiology ClinicsCitation Excerpt :Early studies indicated that the mortality of patients with LQTS with 2:1 AVB is very high; reaching 50% at 6 months and 67% at 2 years.11 Fatal events are associated with VT, which are often pause dependent.11 This is not surprising considering that a markedly prolonged QT interval is often seen in patients with AVB and that a QTc longer than 500 msec is independently associated with an increased risk of significant cardiac events.19
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Conclusions and opinions expressed are those of the authors and do not necessarily reflect the position or policy of the Department of Defense, the Department of the Army, the Army Medical Department, or the Health Services Command.