Long QTBy Courtney Colen

Alternate names

• There is no alternate name • for Long QT.

Who can get this?

• Anyone can get this. If 1 person has been diagnosed in a family. All family members should be tested for LQT.

Symptoms

• This mutation is found in the heart• You cannot tell if a person has LQT by looking at them• Fainting- This is the most common sign of long QT. In

people with long QT syndrome, fainting spells are caused by the heart temporarily beating in an un normal way. These may happen when you're excited, angry or scared, or while exercising. Fainting in people with long QT can occur without warning. – Signs and symptoms before fainting include

lightheadedness, irregular heart beat, and blurred vision. It is uncommon though that warnings are given before you faint.

• Seizures- when the heart consistently beats un-normally the brain becomes deprived of air.

How common?

• Long QT is a lot more common then originally thought. Every 1 person in 5000 to 7000 has long QT. Most unexplained deaths in children and young adults seems they had long QT.

Deadly?

• This can be deadly. If found early enough it can be treated.

• Children, teenagers and young adults with unexplained fainting, unexplained near drowning or other accidents, unexplained seizures, or a history of cardiac arrest may have long QT.

Tests

• An electrocardiogram (ECG)

• Ambulatory ECG monitoring

• Event ECG recording

• A non-exercise (medication) stress test

• An electroencephalogram (EEG).

• Genetic testing

Treatment

• Treatment for long QT syndrome include medications, medical devices, or surgery.

• Medications-Beta Blockers

-Mexiletine -Potassium -Fish Oil

• Medical Devices and Surgery- -Pacemaker

-Left cardiac sympathetic denervation surgery

Support Groups

• Families with long QT may find it helpful to talk to a cardiologist with expertise in diagnosing and treating long QT, a genetics counselor, a psychiatrist or psychologist, as well as other families with the condition.

Inheritance pattern

• Long QT is Dominant not recessive

• Prolonged QT is autosomal

• It is not sex-linked

• Mutations found in 5 ion channel

What Chromosome?

• Long QT is on chromosome 11. another form LQT2 is on chromosome 7.

Interesting Facts

• There are different forms of long QT.– LQT2

• alpha subunit of the rapid delayed rectifier potassium

channel – LQT3

• alpha subunit of the sodium channel – LQT4

• anchor protein Ankyrin B – LQT5

• beta subunit MinK which co assembles with KvLQT1 – LQT6

• beta subunit MiRP1 which co assembles with HERG

Facts (con’t)

– LQT7• potassium channel, leads to Andersen-Tawil syndrome.

– LQT8• alpha subunit of the calcium channel Cav1.2 encoded by the

gene, Leads to Timothy's syndrome.

– No information about the following• LQT9 • LQT10• LQT11• LQT12• LQT13

Pictures

Works Cited

• http://www.hrsonline.org/PatientInfo/HeartRhythmDisorders/IDisorders/index.cfm

• www.mayoclinic.com/health/long-qt-syndrome/DS00434

• http://jmg.bmj.com/content/38/10/705.extract