Treatment And Management Of Long-QT Syndrome
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:
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.
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
This procedure has mainly been used in
for patients who were not responding to Beta blockers. It now has been
largely replaced with permanent pacemaker and cardioverter-defibrillator
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
Khan. Long QT Syndrome: Diagnosis and Management:
Heart J 143(1):7-14, 2002.
Long QT Syndrome by Wojciech Zareba
Sudden Arrhythmia Death Syndromes Foundation
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by QTsyndrome.ch. All rights reserved.