Ventricular pacing in single ventricles-A bad combination.

BACKGROUND: Chronic ventricular pacing (VP) is associated with systolic dysfunction in a subset of pediatric patients with heart block and structurally normal hearts. The effect of chronic VP in congenital heart disease is less well understood, specifically in the single-ventricle (SV) population. OBJECTIVE: To determine the longitudinal effect of VP in SV patients. METHODS: SV patients with heart block and dual-chamber pacemakers requiring >50% VP were compared with nonpaced (controls) SV patients matched for age, sex, and SV morphology. Patients were excluded if a prepacing echocardiogram was not available. Echocardiogram and clinical parameters were compared at baseline (prepacing) and at last follow-up in the paced group, and in controls when they were at ages similar to those of their paced-group matches. RESULTS: Twenty-two paced and 53 control patients from 2 institutions were followed for similar durations (6.6±5 years vs 7.6±7.6 years; P = .59). There was no difference between groups regarding baseline ventricular function or the presence of moderate-to-severe atrioventricular valvar regurgitation (AVVR). Paced patients were more likely to develop moderate-to-severe systolic dysfunction (68% vs 15%; P < .01) and AVVR (55% vs 8%; P < .001) and require heart failure medications (65% vs 21%; P < .001). Chronic VP was also associated with a higher risk of transplantation or death (odds ratio, 4.9; 95% confidence interval, 1.05-22.7; P = .04). CONCLUSIONS: SV patients requiring chronic VP are at higher risk of developing moderate-to-severe ventricular dysfunction and AVVR with an increased risk of death or transplantation compared with controls. New strategies to either limit VP or improve synchronization in this vulnerable population is imperative.

Quality of life in pediatric patients with implantable cardioverter defibrillators.

Psychosocial and quality-of-life (QOL) outcomes in adult patients with implantable cardioverter-defibrillators (ICDs) are well studied. Minimal research exists regarding pediatric adjustment, despite a potentially more challenging adjustment process. The purpose of the present study was to examine psychosocial and QOL functioning of pediatric ICD patients from patient and parent self-reports. Children and primary caregiver dyads from several university hospitals were analyzed using the PedsQL, the Device Severity Index, the ICD and Avoidance Survey, and demographic information. Sixty children (25 female, 35 male) were enrolled. The present pediatric sample reported lower psychosocial and physical QOL scores than healthy children's normative scores. In comparison with a sample of chronically ill children, pediatric ICD patients reported lower physical QOL. Parent-observed QOL reports revealed lower psychosocial and physical QOL than parent-observed healthy norms and lower psychosocial and physical QOL than chronically ill norms. There were no QOL differences by ICD shocks or medical severity. Female patients reported lower psychosocial, physical, and cardiac QOL scores. Children reported better QOL than parent observations on psychosocial and physical health. Finally, 84.7% of children reported avoidance behaviors since ICD implantation, with female children avoiding places more than male children. In conclusion, pediatric ICD patients are comparable to children with other chronic illnesses with the exception of lower physical QOL. Similar to adult samples, female children reported poorer QOL and were more likely to engage in avoidance behaviors. Patients indicated better QOL perceptions than their parents' reports. ICD discharges and medical severity did not negatively affect QOL.