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Treatment And Management Of Long-QT Syndrome
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All symptomatic patients should receive treatment. It is also recommended to screen the family members of patients with long QT syndrome. The goal of therapy is to prevent symptoms. Current research suggests that treatment might best be aimed at the specific gene types of long QT syndrome. But it has not yet been proven that such therapy is effective. For the moment, the following possibilities provide the best medical care:

Beta Blockers: The first choice therapy in patients with long QT syndrome is beta-adrenergic blocking agents. Beta-blockers are effective in preventing cardiac events in approximately 70% of patients, while cardiac events continue to occur despite beta-blocker therapy in the remaining 30% of patients.

Implantable Cardioverter Defibrillator (ICD): Patients who have experienced cardiac arrest, particularly if already on drug treatment, or who continue to have syncope in spite of medications, might best be treated with the implantable defibrillator.

ICDs will not prevent the precipitation of torsades but will prevent sudden cardiac death when torsades is prolonged or degenerates to ventricular fibrillation. Therefore, to prevent the precipitation of torsades, the use of Beta Blockers should be continued along with the implantation of the cardioverter-defibrillator.

According to a new study released early from in the December 25 issue of The Journal of the American Medical Association, ICDs with rate-responsive dual-chamber pacing seem not to offer clinical advantage over back-up ventricular-only pacing.

Left thoracic sympathectomy. This procedure has mainly been used in Europe, for patients who were not responding to Beta blockers. It now has been largely replaced with permanent pacemaker and cardioverter-defibrillator implantation.

Asymptomatic patients. In asymptomatic patients, preventive therapy is required. The reason is that it cannot be predicted with any accuracy which patient will subsequently have symptoms and which one will not. It is important to point out that 30% to 40% of sudden deaths occur at the first event.  The treatment option is the long-term use of Beta blockers agent. It is generally recommended to treat all asymptomatic patients younger than  40 years old at the time of. However, on the other hand, some investigators have recommended treating asymptomatic patients only if they have high-risk characteristics.

Last update: January 2003

Sources: Ijaz A. Khan. Long QT Syndrome: Diagnosis and Management: Am Heart J 143(1):7-14, 2002.

Long QT Syndrome by Wojciech Zareba

Sudden Arrhythmia Death Syndromes Foundation 

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