[Frontiers in Bioscience E1, 519-527, June 1, 2009]

Cardiac conduction disorders in children

Thora S. Steffensen¹, Enid Gilbert Barness¹

¹Department of Pathology and Cell Biology, University of South Florida and Tampa General Hospital, 2 Columbia Drive, Tampa FL 33606


1. Abstract
2. Introduction
3. Cardiac conduction disorders in children
3.1. Histiocytoid cardiomyopathy
3.2. Arrhythmogenic right ventricular dysplasia
3.3. Isolated noncompaction of the left ventricle myocardium
3.4. Long QT syndrome
3.5. Brugada syndrome
3.6. Congenital short QT syndrome
3.7. Catecholaminergic polymorphic ventricular tachycardia
4. Summary
5. References


Conduction disorders result in cardiac arrhythmias that may be fatal. Histiocytoid cardiomyopathy, Arrhythmogenic right ventricular dysplasia, Isolated noncompaction of the left ventricle, Long QT syndrome (LQTS) and Brugada syndrome, are all well described. Congenital short QT syndrome is a new familial primary electrical disease of the heart, which is characterized by abnormally short QT interval and paroxysmal atrial and ventricular tachyarrhythmias, including sudden cardiac death. An autosomal dominant mode of inheritance has been suggested. Catecholaminergic polymorphic ventricular tachycardia is an inherited disease and occurs in the absence of structural heart disease or known associated syndromes. Although the histological appearance of some of these disorders may be diagnostic, molecular analysis is necessary to define clearly the particular type of cardiomyopathy.