Abstract
We describe the use of permanent pacing and implantable defibrillator therapy and their outcome in two consecutive infants with malignant neonatal long QT syndrome. Patient 1 was prenatally diagnosed with fetal hydrops associated with sustained tachycardia. The electrocardiogram (ECG) 1 h after birth showed first degree atrioventricular (AV) block and a QTc (Bazett’s formula) of 0.51 s1/2. Six hours after birth, complete AV dissociation developed, followed by runs of polymorphic ventricular tachycardia and torsade de pointes. A ventricular pacemaker was implanted to prevent the bradycardia-induced arrhythmias. She was discharged on propanolol and mexiletine and remained symptom-free for 10 months. At that time she had a cardiac arrest due to ventricular fibrillation and was successfully resuscitated. Left cervical sympathectomy was performed. Five days later she experienced two further episodes of ventricular tachycardia and fibrillation, and was successfully resuscitated. A cardioverter defibrillator was implanted surgically at 17 months of age following the next episode of ventricular fibrillation (Medtronic Jewel II SN). An epicardial patch was wrapped around the heart. After implantation propanolol was continued.
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