Long QT Syndrome

What is Long QT Syndrome (LQTS)?
LQTS is a disturbance of the heart’s electrical system. It is caused by abnormalities of microscopic pores (proteins) in the heart cells called ion channels.

Nearly half of patients with LQTS NEVER have a symptom!

What are the symptoms of LQTS?

• Cardiac arrest or fainting due to trademark cardiac arrhythmia called torsade de pointes
• Family history – of a SADS diagnosis, SADS symptoms, or sudden unexplained death of a family member under 50 years of age

What tests may be used to make an LQTS diagnosis?

• Echocardiogram (ECG) --to rule out a cardiomyopathy
• More than 1 resting ECG
• A treadmill stress test with an ECG
• An epinephrine, procainamide (Brugada), or catecholiminergic (CPVT) drug study - where the effects of drugs on the heart are analyzed
• A Holter or event monitor
• Genetic Testing

What are the treatment options for LQTS?

• Medication, beta blockers
• ICD
• AED
• Left Cardiac Sympathectomy Denervation (LCSD)
• Avoid drugs that can prolong the QT Interval

Long QT Syndrome Brochure
Long QT Syndrome in Spanish (El Sindrome de QT Largo)
Timothy Syndrome (a rare type of LQTS with autism and webbed toes/fingers)