The risk of NEC in neonates with congenital heart disease is substantial. Factors associated with an elevated risk of NEC in infants with heart disease include premature birth, hypoplastic left heart syndrome, truncus arteriosus, and episodes of poor systemic perfusion or shock. Heightened suspicion is warranted in newborns with these risk factors.

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Our study was designed to characterize the patterns of growth, in the medium term, of children with functionally univentricular hearts managed with a hemi-Fontan procedure in infancy, followed by a modified Fontan operation in early childhood. Failure of growth is common in patients with congenital cardiac malformations, and may be related to congestive heart failure and hypoxia. Repair of simple lesions appears to reverse the retardation in growth. Palliation of the functionally single ventricular physiology with a staged Fontan operation reduces the adverse effects of hypoxemia and prolonged ventricular volume overload. The impact of this approach on somatic growth is unknown. Retrospectively, we reviewed the parameters of growth of all children with functionally univentricular hearts followed primarily at our institution who had completed a staged construction of the Fontan circulation between January 1990 and December 1995. Measurements were available on all children prior to surgery, and annually for three years following the Fontan operation. Data was obtained on siblings and parents for comparative purposes. The criterions of eligibility for inclusion were satisfied by 65 patients. The mean Z score for weight was -1.5 +/- 1.2 at the time of the hemi-Fontan operation. Weight improved by the time of completion of the Fontan circulation (-0.91 +/- 0.99), and for the first two years following the Fontan operation, but never normalized. The mean Z scores for height at the hemi-Fontan and Fontan operations were -0.67 +/- 1.1 and -0.89 +/- 1.2 respectively. At most recent follow-up, with a mean age of 6.1 +/- 1.3 years, and a mean time from the Fontan operation of 4.4 +/- 1.4 years, the mean Z score for height was -1.15 +/- 1.2, and was significantly less than comparable Z scores for parents and siblings. In our experience, children with functionally univentricular hearts who have been palliated with a Fontan operation are significantly underweight and shorter than the general population and their siblings.

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Background-The purpose of this study was to evaluate the long-term outcome of all pediatric epicardial pacing leads. Methods and Results-All epicardial leads and 1239 outpatient visits between January 1, 1983, and June 30, 2000, were retrospectively reviewed. Pacing and sensing thresholds were reviewed at implant, at 1 month, and at subsequent 6-month intervals. Lead failure was defined as the need for replacement or abandonment due to pacing or sensing problems, lead fracture, or phrenic/muscle stimulation. A total of 123 patients underwent 207 epicardial lead (60 atrial/147 ventricular, 40% steroid) implantations (median age at implant was 4.1 years [range 1 day to 21 years]). Congenital heart disease was present in 103 (84%) of the patients. Epicardial leads were followed for 29 months (range 1 to 207 months). The 1-, 2-, and 5-year lead survival was 96%, 90%, and 74%, respectively. Compared with conventional epicardial leads, both atrial and ventricular steroid leads had better stimulation thresholds 1 month after implantation; however, only ventricular steroid leads had improved chronic pacing thresholds (at 2 years: for steroid leads, 1.

The incidences of superficial and deep pacemaker infections were 4.9% and 2.3%, respectively. Trisomy 21 and pacemaker revisions were significant risk factors in the development of infection after pacemaker implantation. For primary pacemaker implantation, the risk of infection requiring system removal is low (0.3%).

ABSTRACT. Objective. To review and describe pneumatosis intestinalis (PI) in children who have undergone thoracic organ transplantation and evaluate potential risk factors.Methods. We retrospectively reviewed abdominal radiographs obtained from June 1992 through September 2000 in all pediatric (age <21 years) thoracic organ recipients who survived at least 1 week after transplantation. In this group, a case was defined as an episode of radiographically confirmed PI; those without PI were assigned as controls. Variables analyzed included demographic data, gastroenteritis history (stool cultures or symptoms of gastroenteritis), and transplant-related factors (ie, graft type, rejection history, immunosuppression regimen). Significance was defined as P < .05.Results. Over this 8-year period, PI occurred in 8 (7%) of 116 patients (0.86% annual risk). No child had >1 diagnosed episode of PI. Of these 8 cases, 7 presented with 1 or more abdominal symptoms. Three of these children had rotavirus antigen isolated in their stool, 2 others were noted to have stool positive for Clostridium difficile toxin, and in the other 3, no pathogen was identified. All cases were treated with a regimen of intravenous antibiotics and total parenteral nutrition. There were no deaths; however, 1 patient developed an Aspergillus pulmonary infection during his course of antibiotic therapy, and another underwent an exploratory laparotomy without bowel resection. Significant risk factors included black race (unadjusted odds ratio: 16), younger age at presentation (age <5 years; unadjusted odds ratio: 9), higher steroid dose (steroid dose >0.5 mg/kg/d; unadjusted odds ratio: 7), and a higher tacrolimus level at presentation (tacrolimus level >1; unadjusted odds ratio: 6). PI did not occur with a steroid dose <0.4 mg/kg/d. Variables not associated with increased risk for developing PI included gender, graft type, total white blood cell count, recent antibiotic use, concurrent use of an antimetabolite, cytomegaloviral infection, past use of extracorporeal membrane oxygenation, and graft rejection history.Conclusions. Significant risk factors for the development of PI in our pediatric thoracic organ transplantation population included black race, younger age, higher daily steroid dosing, and a high tacrolimus level at presentation. In the children diagnosed with PI, there were no related deaths, significant gastrointestinal sequelae, or complications. These findings suggest that in this population, PI will often have a benign course when treated aggressively, and that steroid dosing should be reduced to <0.5 mg/kg/d whenever possible. Pediatrics 2002;109(5). URL: http://www.pediatrics.org/cgi/content/ full/109/5/e78; pediatric heart transplantation, pediatric lung transplantation, pediatric heart-lung transplantation, pneumatosis intestinalis, steroids.

Epicardial leads can be safely placed in Fontan patients at no additional risk compared to patients with biventricular physiology. Sensing and pacing qualities were relatively constant in both the Fontan and non-Fontan groups over the first 2 years after implantation.