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