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. 2008 Jan;1(1):18-26.
doi: 10.4103/0974-2069.41051.

Long QT syndrome: A therapeutic challenge

Maully Shah  1 , Christopher Carter
Affiliations

Long QT syndrome: A therapeutic challenge

Maully Shah et al. Ann Pediatr Cardiol. 2008 Jan.

Abstract

Congenital long QT syndrome (LQTS) is one of the most common cardiac channelopathies and is characterized by prolonged ventricular repolarization and life-threatening arrhythmias. The mortality is high among untreated patients. The identification of several LQTS genes has had a major impact on the management strategy for both patients and family members. An impressive genotype-phenotype correlation has been noted and genotype identification has enabled genotype specific therapies. Beta blockers continue to be the primary treatment for prevention of life threatening arrhythmias in the majority of patients. Other therapeutic options include pacemakers, implantable cardioverter defibrillators, left cardiac sympathetic denervation, sodium channel blocking medications and lifestyle modification.

Keywords: Long QT syndrome; torsade de pointes.

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Conflict of interest statement

Conflict of Interest: None declared

Figures

Figure 1
Figure 1
Surface ECG showing torsades de pointes
Figure 2
Figure 2
Surface ECG showing prolonged QTc interval with broad based T waves often seen in LQT1
Figure 3
Figure 3
Surface ECG showing prolonged QTc with relatively low amplitude notched T waves seen in LQT2
Figure 4
Figure 4
Surface ECG showing prolonged QTc with an isoelectric ST segment and peaked T waves seen in LQT3, also shows a low atrial rhythm
See this image and copyright information in PMC

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