Silvia Mišíková
Rarely occurring heart rhythm disorders are arrhythmogenic syndromes. These are hereditary, primarily electrical disorders.
They are also referred to as channelopathies because they are caused by mutations of genes encoding ion channels.
It is estimated that they cause the sudden death to approximately 10% of patients. A long QT interval and Brugada
syndrome constitute three quarters of all inherited arrhythmogenic syndromes. Substantially less frequent are
the short QT interval syndrome, catecholaminergic polymorphic ventricular tachycardia, early repolarization syndrome,
and idiopathic ventricular fibrillation. The exact diagnosis of arrhythmogenic syndromes is based on molecular-genetic
examination. Patients with abrupt sudden death meet the ICD (implantable cardioverter-defibrillator) implantation
indication in the first class of indication criteria. For other patients, the ICD implantation decision depends on the
specific diagnosis and risk of sudden cardiac death.