Long QT syndrome

It is a genetic arrhythmogenic disease, which is characterized by QT prolongation in the absence of structural damage to the heart. Women are more often affected by this disease.

LQTS (long QT syndrome) is caused by mutation in gene encoding individual subunits of the ion channels of the heart muscle. Failure of the ion channels results in impaired myocardial repolarization, which affects the EKG by prolonging the QT interval.

The risk of LQTS lies in the increased risk of malignant arrhythmias (especially ventricular tachycardia such as torsade de pointes or ventricular fibrillation) and sudden cardiac death.^[1]^[2]^[3]

We distinguish 2 basic forms of LQTS:^[3]^[4]

Roman-Ward syndrome (RWS): AD inherited form, contains a set of 6 different molecular genotypes.
Jervell-Lange-Nielsen syndrome (JLNS): The AR inherited form associated with congenital deafness, contains 2 different molecular genotypes.

LQTS treatment includes Beta-blockers, pacing, or implantation ICD.

thumb|800px|center|Nejznámější formy LQTS