Definition:
Short QT syndrome is a congenital abnormality in the normal rhythm of the heart. The heart of an individual with Short QT Syndrome takes less time than normal to recharge between beats. Left untreated, the irregular heartbeats will manifest signs and symptoms starting from dizziness and fainting, later on cardiac arrest and worse may lead to sudden death.
Diagnosis:
Characteristic history and findings on Echocardiogram and electrophysiologic testing can provide diagnosis of Short QT Syndrome.
Treatment:
At present, implantation of implantable cardioverter-defibrillator is the most effective treatment option for individuals with Short QT Syndrome. This device is a battery-powered electrical impulse generator aimed at detecting abnormal electrical activity of the heart and corrects it by delivering a jolt of electricity.
Symptoms and Signs:
The most common complaint of persons with Short QT Syndrome is unexplained palpitations, irregular heartbeats and loss of consciousness. The characteristic sign of Short QT Syndrome is the presence of very short QT interval on electrocardiogram (ECG).
Some cases of Short QT Syndrome remain to be asymptomatic leading to unexplained or sudden death.
Causes:
Short QT Syndrome is inherited in an autosomal dominant pattern, which means that one copy of the altered gene in each cell in enough to cause the disorder. There were studies proven that Short QT Syndrome is caused by mutations in the KCNH2, KCNJ2, and KCNQ1 genes.
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