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Long QT syndrome: A therapeutic challenge


The Cardiac Center, The Children's Hospital of Philadelphia, Philadelphia, USA

Correspondence Address:
Christopher Carter
The Cardiac Center, The Children's Hospital of Philadelphia 34th and Civic Center Blvd Philadelphia, PA 19104
USA
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0974-2069.41051

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Year : 2008  |  Volume : 1  |  Issue : 1  |  Page : 18-26

 

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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.






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The Cardiac Center, The Children's Hospital of Philadelphia, Philadelphia, USA

Correspondence Address:
Christopher Carter
The Cardiac Center, The Children's Hospital of Philadelphia 34th and Civic Center Blvd Philadelphia, PA 19104
USA
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0974-2069.41051

Rights and Permissions

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.






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