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titlelines Long QT Syndrome
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Click the image to view an animation on Long QT Syndrome 

Long QT Syndrome

Long QT Syndrome (LQTS) is a disorder of the heart’s electrical system, in which the heart muscle cells take longer than normal to recover electrically after each beat. There are two types of LQTS: inherited and acquired.  People with inherited LQTS have an abnormal gene that controls the heart muscle protein that is responsible for electrical activity in the heart muscle cells.  Other people have normal genes, but are taking a medication or combination of medications that interfere with these same heart muscle proteins and cause acquired long QT.  Some people may have a minor genetic abnormality that does not cause obvious LQTS by itself, but they may be very sensitive to certain medications and be more prone to developing LQTS.

It is not known exactly how many people have LQTS, but the inherited type is thought to be a common cause of sudden, unexplained death in children and young adults.

The QT Interval

Click the image to view an animation on an electrocardiogram 

Electrocardiogram

A waveform on an ECG represents the electrical activity of each heart beat.

Most people are familiar with electrocardiography (ECG or EKG),a test in which electrodes are temporarily attached onto the chest, arms and legs to record the heart's electrical activity. The electrodes send information to a machine that records and prints a "picture" (an electrocardiogram) of the pattern, or rhythm, of the electrical impulses that control the heart beat. The line created for each beat on the ECG is called a "waveform."  The curves that are drawn on the ECG graph show when the heart chambers are being activated and also when they are recovering after each beat.

The letters, P, Q, R, S, and T represent different parts of each waveform.

The distance between the beginning of the Q wave and the end of the T wave (called the QT Interval) is a critical measurement. It represents the amount of time the heart's electrical system takes to recharge after each beat (repolarization). After the heart cells have repolarized, the next signal, or heartbeat, can be generated

In LQTS, the QT interval is longer than normal. People with LQTS are at risk of developing an abnormally rapid heart rhythm (arrhythmia) called "Torsades de Pointes." This dangerous arrhythmia happens when an electrical impulse triggers a new heartbeat before the ventricles have fully recovered from the previous beat (repolarized).  When this occurs, the heart muscle begins to quiver, and cannot contract effectively.  Blood is not pumped out to the body and - most important - to the brain. If the brain is starved of blood and oxygen, the indivdiual faints within seconds. If the heart does not return to a normal rhythm on its own, it may switch into deadly arrhythmia called ventricular fibrillation. Without immediate emergency treatment, death follows within minutes.

Fainting or syncope is the primary symptom - and may be the only warning sign - of LQTS. Once a person has been diagnosed with LQTS, all family members should be tested, including the parents, siblings and children of the affected individual.  Testing may include taking ECG recordings, performing an exercise treadmill test, and/or sending blood for genetic testing to look for the presence of an abnormal gene that can cause LQTS.

Some medications may cause acquired LQTS. There are at least 50 or more FDA approved medications that are known to prolong the Q-T interval. This generally is not a problem and does not lead to Torsades de Pointes or other heart rhythm disorders for most people. These medications should be prescribed carefully, however, particularly in people who are taking more than one of these medications, or in the setting of some other types of health problems. These medications must be avoided altogether by people who already have been diagnosed with LQTS.

Drugs that prolong the QT interval and/or induce Torsades De Pointes

The following list of drugs is derived from information found in the labeling of FDA-approved drugs, or in the medical literature. There may be other drugs and/or substances that also cause these heart rhythm problems that are not listed here.

Drug & Brand Names

 

Drug Class (Clinical Use)

 

Arsenic trioxide (Trisenox®) Anti-cancer/Leukemia
Bepridil (Vascor®) Anti-anginal/heart pain
Chlorpromazine (Thorazine®) Anti-psychotic/Anti-emetic/schizophrenia/nausea
Cisapride (Propulsid®) GI stimulant/heartburn
Clarithromycin (Biaxin®) Antibiotic/bacterial infection
Disopyramide (Norpace®) Anti-arrhythmic/abnormal heart rhythm
Dofetilide (Tikosyn®) Anti-arrhythmic/abnormal heart rhythm
Dolasetron (Anzemet®) Anti-nausea/nausea, vomiting
Droperidol (Inapsine®) Sedative; Anti-nausea/anesthesia adjunct, nausea
Erythromycin (E.E.S.® ,Erythrocin®) Antibiotic;GI stimulant/bacterial infection; increase GI motility
Felbamate (Felbatrol®) Anti-convulsant/seizure
Fluoxetine (Prozac®,Sarafem®) Anti-depressant/depression
Foscarnet (Foscavir®) Anti-viral/HIV infection
Fosphenytoin (Cerebyx®) Anti-convulsant/seizure
Gatifloxacin (Tequin®) Antibiotic/bacterial infection
Halofantrine (Halfan®) Anti-malarial/malaria infection
Haloperidol (Haldol®) Anti-psychotic/schizophrenia, agitation
Ibutilide (Corvert®) Anti-arrhythmic/abnormal heart rhythm
Indapamide (Lozol®) Diuretic/stimulate urine & salt loss
Isradipine (Dynacirc®) Anti-hypertensive/high blood pressure
Levofloxacin (Levaquin®) Antibiotic/bacterial infection
Levomethadyl (Orlaam®) Opiate agonist/pain control, narcotic dependence
Mesoridazine (Serentil®) Anti-psychotic/schizophrenia
Moexipril/HCTZ (Uniretic®) Anti-hypertensive/high blood pressure
Moxifloxacin (Avelox®) Antibiotic/bacterial infection
Naratriptan (Amerge®) Serotonin receptor agonist/Migraine treatment
Nicardipine (Cardene®) Anti-hypertensive/high blood pressure
Octreotide (Sandostatin®) Endocrine/acromegaly, carcinoid diarrhea
Paroxetine (Paxil®) Anti-depressant/depression
Pentamidine (NebuPent®,Pentam®) Anti-infective/pneumocystis pneumonia
Pimozide (Orap®) Anti-psychotic/Tourette's tics
Procainamide (Procan® ,Pronestyl®) Anti-arrhythmic/abnormal heart rhythm
Quetiapine (Seroquel®) Anti-psychotic/schizophrenia
Quinidine (Cardioquin®, Quiniglute®) Anti-arrhythmic/abnormal heart rhythm
Risperidone (Risperdal®) Anti-psychotic/schizophrenia
Salmeterol (Serevent®) Sympathomimetic/asthma, COPD
Sertraline (Zoloft®) Anti-depressant/depression
Sotalol (Betapace®) Anti-arrhythmic/abnormal heart rhythm
Sparfloxacin (Zagam®) Antibiotic/bacterial infection
Sumatriptan (Imitrex®) Serotonin receptor agonist/Migraine treatment
Tacrolimus (Prograf®) Immunosuppressant/Immune suppression
Tamoxifen (Nolvadex®) Anti-cancer/breast cancer
Thioridazine(Mellaril®) Anti-psychotic/schizophrenia
Tizanidine (Zanaflex®) Muscle relaxant
Venlafaxine (Effexor®) Anti-depressant/depression
Ziprasidone(Geodon®) Anti-psychotic/schizophrenia
Zolmitriptan (Zomig®) Migraine treatment

 


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