Open AccessCongenital Long QT syndrome and torsade de pointes
Author(s) -
ElSherif Nabil,
Turitto Gioia,
Boutjdir Mohamed
Publication year - 2017
Publication title -
annals of noninvasive electrocardiology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.494
H-Index - 48
eISSN - 1542-474X
pISSN - 1082-720X
DOI - 10.1111/anec.12481
Subject(s) - medicine , afterdepolarization , qt interval , long qt syndrome , cardiology , ventricular action potential , channelopathy , repolarization , electrophysiology
Since its initial description by Jervell and Lange‐Nielsen in 1957, the congenital long QT syndrome (LQTS) has been the most investigated cardiac ion channelopathy. A prolonged QT interval in the surface electrocardiogram is the sine qua non of the LQTS and is a surrogate measure of the ventricular action potential duration ( APD ). Congenital as well as acquired alterations in certain cardiac ion channels can affect their currents in such a way as to increase the APD and hence the QT interval. The inhomogeneous lengthening of the APD across the ventricular wall results in dispersion of APD . This together with the tendency of prolonged APD to be associated with oscillations at the plateau level, termed early afterdepolarizations ( EAD s), provides the substrate of ventricular tachyarrhythmia associated with LQTS , usually referred to as torsade de pointes (TdP) VT . This review will discuss the genetic, molecular, and phenotype characteristics of congenital LQTS as well as current management strategies and future directions in the field.