Inherited Cardiac Arrhythmias and Channelopathies.

Author information

1: Cardiovascular Disease, Summa Health System, 95 Arch Street, Suite 300, Akron, OH 44304, USA.
2: Cardiovascular Disease Fellowship, Summa Health Heart and Vascular Institute, Summa Health System, 95 Arch Street, Suite 350, Akron, OH 44304, USA. Electronic address: costantinio@summahealth.org.

Abstract

With recent advances in genetic diagnostics, many inherited diseases, which can cause life-threatening arrhythmias, are being better characterized. Many of these diseases are caused by genetic disorders that affect the function of the ion channels that regulate the action potential or the function of important cardiac muscle regulatory proteins. This article summarizes the diseases that we have learned about, such as the long QT syndrome, Brugada syndrome, and catecholaminergic polymorphic ventricular tachycardia. The article examines the diagnosis, genetic screening of patients and their relatives, management, and referral to a specialist for further therapy.

KEYWORDS:

Brugada syndrome; Catecholaminergic polymorphic ventricular tachycardia; Channelopathies; Inherited cardiac arrhythmias; Long QT syndrome; Short QT syndrome