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

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.