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Long QT Syndrome 15
Congenital Long QT Syndrome 15

Presentation

They cause syncope and sudden death in response to exercise or emotional stress, and can present with a sentinel event of sudden cardiac death in infancy. [uniprot.org]

There is potential to identify additional genes, gene mutations, and modifier genes due to the great variability in current presentation of the disease. [slideplayer.es]

(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]

Dominant genetic disorders occur when only a single copy of an abnormal gene is necessary for the presentation of the disease. [rarediseases.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]

Cardiovascular

  • Heart Block

    A connexin40 mutation associated with a malignant variant of progressive familial heart block type i. [med.niigata-u.ac.jp]

Neurologic

  • Excitement

    Several different factors are known to trigger the onset of symptoms including physical activity, excitement and fright, although cardiac events may occur while asleep or at rest. [rarediseases.org]

Workup

T Wave

  • Notched T Wave

    t waves on ecg (in some patients) more Prenatal Manifestations: fetal bradycardia (in some patients) Clinical features from OMIM: 616249 Drugs & Therapeutics for Long Qt Syndrome 15 Genetic Tests for Long Qt Syndrome 15 Genetic tests related to Long [malacards.org]

Treatment

It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care. Our staff consists of biologists and biochemists that are not trained to give medical advice. [uniprot.org]

Standard Therapies Treatment is aimed at preventing symptoms such as syncope or cardiac arrest. [rarediseases.org]

Prognosis

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

Prevention

Standard Therapies Treatment is aimed at preventing symptoms such as syncope or cardiac arrest. [rarediseases.org]

ICDs can treat breakthrough tachyarrhythmias to prevent sudden cardiac death. 47 Manejo segun genotipo LQT1 y LQT2 se benefician ppalmente. Con BB El beneficio de BB en LQT3 es menos claro. [slideplayer.es]

- 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]

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