Edit concept Question Editor Create issue ticket

Long QT Syndrome 5

Romano-Ward Syndrome


Presentation

  • Education Plain Text - 2015 Graduate School of Comprehensive Human Sciences, University of Tsukuba, Tsukuba, Japan 1999 - 2005 Hamamatsu University of Medicine, Hamamatsu, Japan Awards & Honors Plain Text Oct 2018 A novel CACNA1C mutation(R860Q) in a family presented[researchmap.jp]
  • 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]
Physician
  • "Congenital long QT syndrome: considerations for primary care physicians". Cleveland Clinic Journal of Medicine. 75 (8): 591–600. doi : 10.3949/ccjm.75.8.591. PMID 18756841. a b c d e f g h i j "Long QT Syndrome". NHLBI, NIH.[en.wikipedia.org]
  • Coll Cardiol 2012;59 1948-1953 篠崎毅 睡眠呼吸障害に対する夜間酸素療法をどのように用いるか Heart View 2012;16:90-93 法井薫、木村義隆、但木壮一郎、田丸貴規、山口展寛、尾上紀子、田中光昭、石塚豪、篠崎毅 腎梗塞と調律、左房径、CHADS2スコアの関係 心臓 2012;44:546-550 尾上紀子、尾形剛、藤田央、山口展寛、田中光昭、石塚豪、篠崎毅 動脈管開存症によるEisenmenger症候群に対してボセンタンが有効であった1例 Modern Physician[nsmc.hosp.go.jp]
Surgical Procedure
  • Arrhythmia prevention [ edit ] Arrhythmia suppression involves the use of medications or surgical procedures that attack the underlying cause of the arrhythmias associated with LQTS.[en.wikipedia.org]
Cheyne-Stokes Respiration
  • において睡眠呼吸療法による夜間交感神経活性の低下はslow wave sleepの増加に依存する 第44回睡眠呼吸障害研究会 2010/2/20 東京 篠崎毅、池田尚平、田丸貴規、尾上紀子、田中光昭、石塚豪 Adaptive-Servo Ventilation Improves Sleep Quality And Restores Ultradian Rhythm in Electroencephalogram in Patients With Chronic Heart Failure And Cheyne-Stokes[nsmc.hosp.go.jp]
Normal Hearing

Workup

Prolonged QT Interval
  • QT interval and congenital deafness is seen. [18] Other rare forms include Anderson-Tawil syndrome (LQT7), which combines a prolonged QT interval with periodic paralysis and abnormalities of the face and skeleton; and Timothy syndrome (LQT8) which associates[en.wikipedia.org]
  • Their propensity to prolong QT interval and to increase the TDR is dose-dependent for almost all, except quinidine32. However, the amiodarone is perceived as a relatively safe drug.[romanianjournalcardiology.ro]
2:1 Atrioventricular Block
  • 1 Atrioventricular Block Okuwaki K, Kato Y, Lin L, Nozaki Y, Ishikawa N, Takahashi-Igari M, Horigome H Heart ryhthm 2017 10 May 2017 Efficacy of walking as a lifestyle modification approach for childhood obesity.[researchmap.jp]
  • Timothy syndrome may include a functional 2/1 atrioventricular block, due to an exceedingly long QT interval5,19,48.[romanianjournalcardiology.ro]
Notched T Wave
  • T-waves in at least 3 leads 1 point Low heart rate for age (children) 0.5 points Syncope with stress 2 points Cannot receive points both for syncope and Torsades without stress 1 point Congenital deafness 0.5 points Family history Other family member[en.wikipedia.org]

Treatment

  • 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]

Prognosis

  • A Pharmacist-Directed Medication Counseling Contributes to Better Prognosis of Patients with Chronic Heart Failure.[nsmc.hosp.go.jp]

Etiology

  • 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]
Sex distribution
Age distribution

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]

Ask Question

5000 Characters left Format the text using: # Heading, **bold**, _italic_. HTML code is not allowed.
By publishing this question you agree to the TOS and Privacy policy.
• Use a precise title for your question.
• Ask a specific question and provide age, sex, symptoms, type and duration of treatment.
• Respect your own and other people's privacy, never post full names or contact information.
• Inappropriate questions will be deleted.
• In urgent cases contact a physician, visit a hospital or call an emergency service!