LQT10

Long QT Syndrome Microchapters
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [2]

Overview

Only one mutation in one patient of this Long QT syndrome subtype has been found.

LQT10

Type	Mutation
LQT10	SCN4B

This novel susceptibility gene for LQT is SCN4B encoding the protein Na_Vβ4, an auxiliary subunit to the pore-forming Na_V1.5 (gene: SCN5A) subunit of the voltage-gated sodium channel of the heart. The mutation leads to a positive shift in inactivation of the sodium current, thus increasing sodium current. Only one mutation in one patient has so far been found.

History and Symptoms

• Seizures - due to oxygen deprivation that occurs during arrhythmia.
• Fainting - fainting or syncope is the most common symptom LQTS.
• A prodrome may occur before losing consciousness, which may consist of lightheadedness, heart palpitations, blurred vision or weakness.
• Sudden death - a fatal arrhythmia that is not quickly intervened on, may cause sudden death.

Therapy

• Beta-blockers are the first line treatment in LQTS, even in asymptomatic carriers, as they reduce the incidence of syncope and sudden cardiac death.
• Other medications to control non-malignant arrhythmias.
• Electrolytes should be repleted as neccesary.
• Avoidance of triggers (drugs, supplements, loud noises, exercise).
• LQTs is one of the few diseases where genetic testing can provide important guidance, such as in whom to place an AICD (defibrillator) for the primary prevention of cardiac events.
• Placement of a pacemaker may be indicated.
• Left stellectomy is not a cure, but is a second line therapy to reduce the risk of sudden cardiac death and is indicated if the patient does not tolerate beta blockers, as well as in young patients under the age of 12 where beta blockers are not deemed protective enough and AICD is not appropriate.
• Patients with long QT syndrome should undergo secondary prevention with AICD implantation if they sustain an aborted cardiac arrest or sudden cardiac death.