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Placement of an implantable cardioverter-defibrillator in an infant with congenital long QT syndrome: Anesthetic considerations

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Author(s): Kansara Bhuvnesh | Singh Ajmer | Kaushal Sunil | Saxena Anil

Journal: Annals of Cardiac Anaesthesia
ISSN 0971-9784

Volume: 14;
Issue: 2;
Start page: 122;
Date: 2011;
Original page

Keywords: Congenital long QT syndrome | implantable cardioverter-defibrillator | anesthesia

ABSTRACT
Sudden cardiac arrest (SCA) in children is a rare, but catastrophic event. Children with cardiac pathology at particular risk include those with congenital long QT syndrome (CLQTS) and hypertrophic cardiomyopathy. CLQTS is a genetic disorder of the cardiac ion channels and is associated with significant risk of malignant ventricular arrhythmias and SCA. For symptomatic, untreated patients, the mortality rate is approximately 20% for the first year and 50% at ten years. Use of an implantable cardioverter-defibrillator (ICD) is recommended for the prevention of SCA in this patient population. We report a case of CLQTS, who after successful resuscitation from SCA, underwent ICD placement at our center.

Tango Jona
Tangokurs Rapperswil-Jona

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