Kardiale Ionenkanalerkrankungen

Abstract

Cardiac channelopathies are caused by mutations of ion channels and other functional proteins. Without causing structural heart disease, they can cause sudden cardiac death by disturbing cellular repolarization. Channelopathies can be distinguished by the proteins carrying the specific mutation. More relevant for clinical practice is a nomenclature based on ECG and clinical criteria. Frequent channelopathies are the different QT syndromes, the Brugada syndrome and catecholaminergic polymorphic tachycardia. Therapy options consist of pharmacological and/or ICD therapy. Only part of the patient population with the specific mutation or characteristic ECG pattern is symptomatic; therefore and because of possible complications, ICD therapy has to be considered with caution. Generally, ICD therapy is indicated after survival of sudden cardiac death. The decision to implant an ICD for primary prevention is more difficult and should be based on a thorough risk stratification.