904-50 Pacemaker Cardioverter Defibrillators in Young Patients

Abstract

Although life-threatening ventricular dysrhythmias are uncommon in young patients, there are underlying heart diseases that may predispose these patients to ventricular tachycardia or ventricular fibrillation (VT\VF). To test the hypothesis that tiered therapy with a pacemaker-cardioverter defibrillator (PCD) is a therapeutic option for young patients at risk for sudden death, we reviewed our experience with these devices. Nine patients, aged 9–33 years (17.7 ± 7, mean ± SD) at the time of implantation, received PCD's between March, 1993, and the present. Post-operative diagnoses included complete repair of tetralogy of Fallot (2), Senning for D-transposition of the great arteries (1), double outlet right ventricle, status post ventricular septal defect closure baffling the left ventricle to the aorta (1), and Fontan for single ventricle with pulmonary stenosis (1). Other diagnoses included Wolff-Parkinson-White (WPW) (1), WPW and dilated cardiomyopathy (1), arrhythmogenic right ventricular dysplasia (1), and long QT syndrome (1). All patients had inducible or clinical unstable VT\VF before implantation except one patient with WPW who had a family history of sudden death. Seven of the patients had failed antiarrhythmics before implantation. Defibrillator electrode configurations consisted of epicardial patches (4), transvenous alone (3), and transvenous with subcutaneous patch(es) (2). In 10.4 ± 5 (range 2–17) months of follow-up, overdrive pacing was successful in one patient, and there have been appropriate defibrillator discharges in five patients (56%). Seven of the nine patients remain on antiarrhythmics. There was only one complication, a moderate pericardial effusion two months following transvenous lead implantation in a 24.9 kg nine-year old. No system has required revision to date. In relatively short-term follow-up over 50% of our patients successfully have used tiered therapy from pacemaker-cardioverter defibrillators. This implies that these therapies protect selected young patients from sudden cardiac death, potentially greatly increasing their life span.