(Presentation of 1st Case in Italian Pediatric Literature)] Clin Pediatr (Bologna). 1963;45:656-83. 5. Ward OC. A New Familial Cardiac Syndrome In Children. J Ir Med Assoc. 1964;54:103-6. 6. Schwartz PJ, Priori SG, Napolitano C. [revistas.uis.edu.co]

A synthesis of the available management methods is presented in Figure 4. Figure 4. Management of LQTS. [romanianjournalcardiology.ro]

About half of these cases are thought to be due to Brugada syndrome. [10] At present, implantation of an automatic implantable cardiac defibrillator (ICD) is the only treatment proven effective in treating ventricular tachycardia and fibrillation and [emedicine.medscape.com]

[…] 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]

• Disability

  Please enable JS and disable any ad blocker [ouest-france.fr]

• Familial Adenomatous Polyposis

  adenomatous polyposis plakoglobin ( Naxos syndrome ) GAN ( Giant axonal neuropathy ) Other desmoplakin : Striate palmoplantar keratoderma 2 Carvajal syndrome Arrhythmogenic right ventricular dysplasia 8 plectin : Epidermolysis bullosa simplex with muscular [en.wikipedia.org]

• Tachycardia

  Flecainide suppresses bidirectional ventricular tachycardia and reverses tachycardia-induced cardiomyopathy in Andersen-Tawil syndrome. J Cardiovasc Electrophysiol 2008;19(1):95-97. 45. Noda T, Shimizu W, Satomi K et al. [romanianjournalcardiology.ro]

  At that time, their electrocardiograms showedprolongation of QT interval (QT intervals 0.55 sec, 0.52sec, 0.52 sec), and also ventricular tachycardia, the socalled torsade de pointes. [webview.isho.jp]

  The mean age of patients who die suddenly is 41 years. [13] Prognosis Brugada syndrome is a cause of polymorphic ventricular tachycardia, which may degenerate into ventricular fibrillation and cause cardiac arrest. [emedicine.medscape.com]

  Symptoms that do occur are generally caused by abnormal heart rhythms or arrhythmias, most commonly a form of ventricular tachycardia called Torsades de pointes. [en.wikipedia.org]

• Heart Disease

  Congenital deaf mutism, functional heart disease with prolongation of the QT interval, and sudden death. Am Heart J. 1957;54:59-68. 4. Romano C, Gemme G, Pongiglione R. [Rare Cardiac Arrhythmias of the Pediatric Age. II. [revistas.uis.edu.co]

  Braunwald’s heart disease: a textbook of cardiovascular medicine, 9th edition. Editors: Bonow RO, Mann DL, Zipes DP et al. Elsevier Saunders, Philadelphia, 2012,126-167. 37. Vetter VL. [romanianjournalcardiology.ro]

  Electrical Diseases of the Heart. London: Springer. pp. = 39–46. doi : 10.1007/978-1-4471-4881-4_27. ISBN 978-1-4471-4881-4. ^ Nakajima T, Kaneko Y, Kurabayashi M (2015). [en.wikipedia.org]

  Ventricular fibrillation without apparent heart disease: description of six cases. Am Heart J. 1989 Dec. 118(6):1203-9. [Medline]. Brugada J, Brugada R, Brugada P. [emedicine.medscape.com]

• Seizure

  If the arrhythmia reverts to a normal rhythm by itself then the affected person may experience a faint known as syncope, which may be associated with seizures. [en.wikipedia.org]

See Workup for more detail. [emedicine.medscape.com]

• 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]

• Torsades De Pointes

  At that time, their electrocardiograms showedprolongation of QT interval (QT intervals 0.55 sec, 0.52sec, 0.52 sec), and also ventricular tachycardia, the socalled torsade de pointes. [webview.isho.jp]

  Schwartz score to aid diagnosis of inherited Long QT syndrome. [31] Corrected QT interval (QTc) ≥ 480 ms 3 points QTc defined according to Bazett's correction 460-470 ms 2 points 450 ms and male gender 1 point Torsades de pointes 2 points T-wave alternans [en.wikipedia.org]

  The onset of the pause-dependent torsade de pointes. VPB = ventricular premature beat. Data in references 30,32. [romanianjournalcardiology.ro]

  Barnel C 2009, 60 p Severe respiratory failure and torsades de pointes induced by disopyramide in a patient with myasthenia gravis Hirose K, Yamaguchi H, Oshima Y, et al. [myobase.org]

• Abnormal ECG

  […] depolarization abnormalities and conduction delay mapped to the right ventricle. [emedicine.medscape.com]

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]

See Treatment and Medication for more detail. [emedicine.medscape.com]

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]

Incidence, clinical implications and prognosis of atrial arrhythmias in Brugada syndrome. Eur Heart J. 2004 May. 25(10):879-84. [Medline]. Take Y, Morita H, Toh N, Nishii N, Nagase S, Nakamura K, et al. [emedicine.medscape.com]

Brugada syndrome is genetically determined and has an autosomal dominant pattern of transmission in about 50% of familial cases (see Etiology). [emedicine.medscape.com]

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]

[…] alcohol or cocaine intoxication, and the use of certain medications, including sodium channel blockers, vagotonic agents, alpha-adrenergic agonists, beta-adrenergic blockers, heterocyclic antidepressants, and a combination of glucose and insulin. [13] Epidemiology [emedicine.medscape.com]

[…] 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]

Molecular Pathophysiology of Congenital Long QT Syndrome. Physiol Rev. 2017 Jan;97(1):89-134. Review. Modell SM, Bradley DJ, Lehmann MH. Genetic testing for long QT syndrome and the category of cardiac ion channelopathies. [ghr.nlm.nih.gov]

"Brugada" syndrome: clinical data and suggested pathophysiological mechanism. Circulation. 1999 Feb 9. 99(5):666-73. [Medline]. Meregalli PG, Wilde AA, Tan HL. [emedicine.medscape.com]

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]

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]

[…] of Patients with Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death of the European Society of Cardiology (ESC). [emedicine.medscape.com]