Presentation
The present study was designed to determine the G325R ion channel phenotype and its association with the clinical LQTS presentation. Electrophysiological testing was performed using the Xenopus oocyte expression system. [ncbi.nlm.nih.gov]
A synthesis of the available management methods is presented in Figure 4. Figure 4. Management of LQTS. [romanianjournalcardiology.ro]
[…] increased risk of an irregular heartbeat which can result in palpitations, fainting, drowning, or sudden death. [1] These episodes can be triggered by exercise or stress. [5] Other associated symptoms may include hearing loss. [1] Long QT syndrome may be present [en.wikipedia.org]
Inappropriate pacing inhibition triggered by QT prolongation due to t wave oversensing in an icd recipient presenting with long QT syndrome. Internal medicine. 2011;50:1021-1024 34. [med.niigata-u.ac.jp]
Musculoskeletal
- Fracture
Malfunction of cardiac resynchronization therapy due to subsequent fracture of the ring and tip conductors of a co-radial left ventricular bipolar lead. Intern Med. 2013;52:1189-93 [med.niigata-u.ac.jp]
Ears
- Normal Hearing
hearing, later called Romano-Ward syndrome. [en.wikipedia.org]
Workup
QT, RR, ST Intervals
- Prolonged QT Interval
While some with LQTS will have QT intervals that are very prolonged, others will have only slight QT prolongation, or even a normal QT interval at rest (concealed LQTS). [en.wikipedia.org]
On the one side, one can see the prolongation of QT interval, often associated, but not always, with an increased TDR. [romanianjournalcardiology.ro]
Treatment
Neonatal electrocardiographic screening could allow early identification of LQTS and adapted treatment and follow-up. https://hal.archives-ouvertes.fr/hal-00911278 Contributeur : Morgane Le Corre <> Soumis le : vendredi 29 novembre 2013 - 09:56:14 Dernière [hal-bioemco.ccsd.cnrs.fr]
Beta blockers are an effective treatment for LQTS caused by LQT1 and LQT2. [4] Genotype and QT interval duration are independent predictors of recurrence of life-threatening events during beta-blocker therapy. [en.wikipedia.org]
The risk stratification for a first cardiac event in life, before age of 40 and without treatment in congenital LQTS types 1, 2 and 3, depending upon QTc value and gender. [romanianjournalcardiology.ro]
Etiology
Current SIDS etiological surveys fail to diagnose LQTS. Specific questioning and electrocardiographic screening of first-degree relatives could greatly facilitate LQTS diagnosis. We propose adding these to screening modalities after a SIDS incident. [hal-bioemco.ccsd.cnrs.fr]
Etiology of sudden death in an apparently normal heart (7-10) Congenital LQTS inside the genetic anomalies of the ion channels So far, there is a spectrum of inherited arrhythmia syndromes arising from genetic defects in structures involved in the genesis [romanianjournalcardiology.ro]
Epidemiology
[…] who are untreated, the risk of death within 15 years is around 50%. [6] With careful treatment this decreases to less than 1% over 20 years. [3] Those who exhibit symptoms before the age of 18 are more likely to experience a cardiac arrest. [18] [36] Epidemiology [en.wikipedia.org]
Pathophysiology
The role of the sympathetic nervous system in the long QT syndrome: the long road from pathophysiology to therapy. Long QT syndrome. Cardiac Electrophysiology Clinics, volume 4, number 1. Editors: Priori SG, Thakur RK, Natale A. [romanianjournalcardiology.ro]
"Molecular Pathophysiology of Congenital Long QT Syndrome". Physiological Reviews. 97 (1): 89–134. doi : 10.1152/physrev.00008.2016. PMID 27807201. ^ a b Giudicessi JR, Ackerman MJ (October 2013). [en.wikipedia.org]
Prevention
Principles of management of the LQTS As not all syncopes in a patient having LQTS are automatically due to TdP, the treatment of any form of LQTS is essentially the prevention and termination of TdP and consequently the prevention of sudden cardiac death [romanianjournalcardiology.ro]
(November 2015). "2015 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death: The Task Force for the Management of Patients with Ventricular Arrhythmias and the Prevention of Sudden Cardiac [en.wikipedia.org]
- prevention of vagal nerve activation-induced shortening of the atrial action potential duration. Circ J. 2010;74:895-902 8. Izumi D, Chinushi M, Iijima K, Ahara S, Komura S, Furushima H, Hosaka Y, Sanada A, Yagihara N, Aizawa Y. [med.niigata-u.ac.jp]