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Inherited arrhythmias and conduction disorders 

Inherited arrhythmias and conduction disorders
Chapter:
Inherited arrhythmias and conduction disorders
Author(s):

Perry Elliott

, Pier D. Lambiase

, and Dhavendra Kumar

DOI:
10.1093/med/9780198829126.003.0009
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date: 26 May 2022

Long QT syndrome is primarily a disorder of repolarization with three principal ion channel currents accounting for the condition. Management revolves around optimization of beta blockade and avoidance of triggers with implantable cardiac defibrillators (ICD) recommended in high risk cases. Brugada syndrome is thought to be caused by Na channel mutations although the aetiology is not fully established. Controversy exists regarding optimal risk stratification approaches. Catecholaminergic polymorphic ventricular tachycardia is caused by abnormalities in calcium handling leading to bidirectional ventricular tachycardia/VF and can be managed using medication –principally beta blockers and occasionally sympathectomy with ICD in cardiac arrest survivors with specific caveats. Inherited conduction disorders should always be considered in patients presenting at a younger age with conduction disease paying specific attention to lamin mutations where an ICD will need to be considered.

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