In December of 2007, I was diagnosed with a heart condition known as "Long QT Syndrome"
What is Long QT Syndrome?
Long QT Syndrome is a heart disorder that is associated with sudden unexpected fainting (loss of consciousness), seizures
(convulsions), or death. The "QT" refers to the QT interval, which is a time found on a standard electrocardiogram (EKG or
ECG). An EKG is a recording of the electrical activity of the heart. On the EKG doctors measure several intervals. The QT
interval includes the time that the heart muscle relaxes. Because people have different heart rates, the measured interval
is corrected for heart rate by a mathematical formula and the result is called the corrected QT interval (often depicted by
QTC or QTc). The normal QTc was thought to be 0.44 second or less. It is now thought that a QTc greater than 0.46 in males,
0.47 in females, is prolonged. However, we now know that some individuals can have a normal QTc at times and abnormal at others.
To have the "syndrome" other factors such as severe symptoms and family history are used to help make the diagnosis. Many
causes of long QT interval exist, such as medications, low potassium, magnesium or calcium in the blood, and abnormal heart
muscle, to name a few. Long QT Syndrome is different. People who have Long QT Syndrome usually inherit the condition from
relatives and have it from birth. Therefore, Long QT Syndrome is congenital (present at birth) and usually is hereditary (present
What is the treatment for Long QT Syndrome?
The aim of treatment is to prevent symptoms and sudden death. The choice of treatment is based on the presence
or absence of symptoms, as well as on the type of symptoms and the degree of sudden death in family members. For patients
with minimal or no symptoms, the first choice of medicine usually is Propranolol (Inderal). Propranolol is a medicine that
is in a class of drugs called beta-blockers. Propranolol is effective in preventing symptoms in approximately 80% of people
with Long QT Syndrome. Although more experience exists with Propranolol than any other treatment and it has been proven effective
in most people with Long QT Syndrome, some other medicine may occasionally be needed.
Although most people do not have intolerable side effects from Propranolol, occasionally severe sleep disturbance,
mental depression, poor school performance, or impotence may require changing to a different beta-blocker medication. Experience
is limited with other beta-blockers. However, experience in the registry suggests that Nadolol (Corgard) is an acceptable
alternative. Atenolol (Tenormin) may not be a good alternative in young people. Sometimes Propranolol or Nadolol are not effective,
or the specific type of problems suggests that another medication should be used. Examples include Mexiletine (Mexitil), Tocainide
(Tonocard), or Phenytoin (Dilantin).
When a slow heart rate is found, medication may cause further slowing and a pacemaker may be needed. The treatment
combination of medication and a pacemaker provides an attempt to protect against the fast heart rate (ventricular tachycardia
and ventricular fibrillation) and the slow heart rate.
The most aggressive treatment option is to implant a device called an automatic cardioverter-defibrillator (ICD).
In the same device, a pacemaker is included. This option is usually considered when the patient has been resuscitated from
a cardiac arrest or has had symptoms suggesting a life-threatening event. The device
contains a sophisticated computer. When the life-threatening fast heart rate ventricular tachycardia fibrillation
occurs, the device recognizes it and then responds by shocking the heart.
Rarely, people require additional treatment such as surgery. The intent of surgery is to decrease the effect of adrenaline
on the heart by cutting nerves in the spine area of the left neck.
Learn more about "Long QT Syndrome"
Click here to learn more about "Long QT Syndrome"
Sudden Arrhythmia Death Syndrome: Importance of the Long QT Syndrome
The long QT syndromes: genetic basis and clinical implications.
Long-QT Syndrome Frequently Asked Questions