CONGENITAL LQTS - AN ELECTROCARDIOGRAPHIC AND GENOTYPE CORRELATION
Abstract
The congenital Long QT Syndrome (LQTS) is characterized by abnormally prolonged ventricularrepolarization due to inherited defect in cardiac sodium and potassium channels, which
predisposes the patients to syncope, ventricular arrhythmias, and sudden cardiac death. Early
diagnosis and preventive treatment are instrumental to prevent sudden cardiac death in patients
with the congenital LQTS. The diagnostic criteria for congenital LQTS are based on certain
electrocardiographic findings and clinical history. Recently genotype specific electrocardiographic
pattern in the congenital LQTS has also been described. Recent studies suggest feasibility of
genotype specific treatment of LQTS and, in near future, mutation specific treatment will probably
become a novel approach to this potentially fatal syndrome. We describe two cases that fulfilled
the electrocardiographic and historical diagnostic criteria with morphology on electrocardiogram
(ECG) suggestive of LQT1 genotype.
Keywords: Congenital LQTS, ECG, genotype, pregnancy
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