Treatment of Long QT Syndrome (LQTS)

By Dr Ananya Mandal, MD

Treatment of long QT syndrome (LQTS) aims to prevent sudden cardiac death and similarly fatal abnormal heart rhythms and fainting spells. LQTS is a genetic condition in the majority of cases and occurs from birth till death.

There are no cures for this condition and no therapy can restore a normal QT interval. However, therapy may help prevent sudden cardiac deaths and greatly improves the chances of survival.

Diagnosis of LQTS

Before treatment an accurate diagnosis is important. This includes a detailed medical, family and personal history and assessment using Electrocardiograms.

The type of LQTS is determined. The possible triggers that bring on the fainting spells are enquired of the patient.

Types of treatment of LQTS

Treatment of LQTS includes lifestyle changes, dietary changes and so forth. (1-7)

Lifestyle changes

Patients are advised of lifestyle changes. These include avoidance of strenuous exercise and water sports such as swimming and diving etc. (as they raise the risk of drowning).

Other sports should also be avoided in the susceptible individuals as they may trigger abnormal heart rhythms.

Patients may also be asked to avoid sudden jarring noises like buzzers, alarm clocks or door bells as this may trigger symptoms.

Patients are advised to wear labels or bracelets that mention their condition in case they have an unexpected black out while unescorted.

Physical activities such as walking, moderate hiking and biking, and golf are suitable but need to be cleared by the cardiologist before they are attempted.

Dietary changes

A diet that is rich in potassium, such as bananas as well as potassium supplements, may be beneficial in some types of LQTS like type 1 and type2. These should be taken under medical supervision and advice.

Some foods increase the heart rate and worsen the symptoms of LQTS. These include caffeine containing drinks like tea, coffee, fizzy drinks and high-energy drinks. These should be avoided.

Avoiding certain medications

Patients are advised to avoid certain medications that may trigger abnormal heart rhythms. This includes anti-allergy medications (antihistaminics and decongestants), diuretic pills for high blood pressure, cholesterol lowering pills, antidepressants and antipsychotics.

Medications for LQTS

Medications like beta-blockers are advised as they reduce the risk of symptoms by slowing the heart rate. These includes Propranolo, Atenolol or Nadolol.

This, however, is not advised in patients with LQTS 3 who get the symptoms of LQTS when their heart rate slows. These patients with LQTS 3 usually need sodium channel blocker medicines like Mexiletine.

Surgically implanted devices

Some patients may need implanted devices. These are surgically implanted devices include the pacemaker or an implantable cardioverter defibrillator (ICD). These devices send in small electric currents and serve to control the abnormal electric pulses that generate the arrhythmias in the heart.

The pacemakers and ICDs are implanted under the skin of the chest or abdomen surgically and connected to the heart. The use of these devices is similar in children and adults.

Surgery on the nerves that regulate the heartbeat

Some patients may also require surgery on the nerves that regulate the heart beat. This is usually suggested for patients who have dangerous heart rhythms from their LQTS and are at a high risk of sudden cardiac death.

Automated external defibrillator (AED)

Some patients may need to keep an Automated external defibrillator (AED) at hand at home or work place to manage sudden attack. This device uses electric shocks to restore a normal heart rhythm. Someone at work or at home needs to be trained to use the AED in case of an emergency.

Sleeping arrangements

Those with LQTS 3 that may lead to symptoms during sleep, patient is advised not to sleep alone or have an intercom in the bedroom that is connected to someone else's bedroom.

Regular check-ups for relatives

Regular checkups with a cardiologists and examination of all first degree relatives for LQTS is important.

Avoidance of recreational drugs

Recreational drugs such as Ecstasy and Cocaine are very dangerous to patients with LQTS and should be avoided.

When is treatment needed?

In patients who have never had unexpected or unexplained symptoms like fainting, seizures or cardiac arrest then he or she may be given no treatment but be closely observed for symptoms.

If the person or any of his family has had any symptom, treatment is recommended.

People with borderline long QT syndrome where the QT interval is between 0.43‐0.45 seconds and with other diagnosis criteria need treatment.

LQTS and pregnancy

There are no recommendations that prevent a woman with LQTS from getting pregnant. However the pregnancy needs to be closely watched for complications. The baby also needs to be monitored after birth.

Reviewed by , BA Hons (Cantab)

Further Reading

Last Updated: Aug 12, 2012

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