Long QT Syndrome

Long QT syndrome is an abnormality in the hearts conduction system.  It is a rare genetic disorder that effects about 1/5000 individuals.  It causes approximately 3,000 deaths per year in the United States.  Individuals with this genetic abnormality are at risk for an abnormal heart rhythm that can result in syncope or sudden cardiac arrest.  Often times the abnormal rhythm is brought about by exercise or a startling event that causes the release of adrenalin.  Unfortunately, this condition is often responsible for sudden death in teenagers and adolescents.

How is Long QT Syndrome diagnosed?

Persons with long QT syndrome appear completely normal and often go undiagnosed until they have an emotional or physically intense event that causes them to pass out.  Some other ways physicians can diagnose this is by doing an EKG or electrocardiogram.  The electrocardiogram is a test used to visualize the electrical conduction system of the heart.  Sometimes the long QT interval shows up on this test.  Another important piece of information is knowing the patient's family history.  If there is another family member or relative who has Long QT syndrome there is a good chance that other family members have it as well.  

What is the treatment for long QT syndrome?

There are two treatment options for Long QT syndrome.  One is medical therapy with beta blockers and the other is an ICD or implantable cardioverter defibrillator.  ICDs are generally reserved for patients at high risk for sudden cardiac death.

Beta blockers are medications that help to block adrenalin to the heart.  Studies have shown that beta blockers are effective in reducing long QT syndrome in about 75% of individuals.  The other 25% of individuals will require an ICD.  To learn more about these devices click on ICD above.

One other important thing that all people with long QT syndrome need to be aware of is that certain drugs/medications can further prolong the QT interval and increase their chances for sudden cardiac arrest.