Objectives To assess self-reported quality of life (QOL) in a large multicenter cohort of adolescent and young adults surviving Fontan. Study design Cross-sectional. The Pediatric Quality of Life ...Inventory (PedsQL) was administered to 408 survivors of Fontan ages 13-25 years enrolled in the Pediatric Heart Network Fontan Follow-up Study. Subjects also completed either the Child Health Questionnaire (age <19 years) or Short Form Health Survey (age ≥19 years). PedsQL data were compared with matched controls without a chronic health condition. Correlations between the measures were examined. Results Mean PedsQL scores for subjects receiving Fontan were significantly lower than those for the control group for physical and psychosocial QOL ( P < .001). Overall, 45% of subjects receiving Fontan had scores in the clinically significant impaired range for physical QOL with 30% in the impaired range for psychosocial QOL. For each 1 year increase in age, the physical functioning score decreased by an average of 0.76 points ( P = .004) and the emotional functioning score decreased by an average of 0.64 points ( P = .03). Among subjects ≥19 years of age, the physical functioning score decreased by an average of 2 points for each year increase in age ( P = .02). PedsQL scale scores were significantly correlated with conceptually related Child Health Questionnaire ( P < .001) and Short Form Health Survey scores ( P < .001). Conclusions Survivors of Fontan are at risk for significantly impaired QOL which may decline with advancing age. Routine assessment of QOL is essential to inform interventions to improve health outcomes. The PedsQL allowed QOL assessment from pediatrics to young adulthood. Trial registration ClinicalTrials.gov : NCT00132782.
Objectives To assess variation in feeding practice at hospital discharge after the Norwood procedure, factors associated with tube feeding, and associations among site, feeding mode, and growth ...before stage II. Study design From May 2005 to July 2008, 555 subjects from 15 centers were enrolled in the Pediatric Heart Network Single Ventricle Reconstruction Trial; 432 survivors with feeding data at hospital discharge after the Norwood procedure were analyzed. Results Demographic and clinical variables were compared among 4 feeding modes: oral only (n = 140), oral/tube (n = 195), nasogastric tube (N-tube) only (n = 40), and gastrostomy tube (G-tube) only (n = 57). There was significant variation in feeding mode among sites (oral only 0%-81% and G-tube only 0%-56%, P < .01). After adjusting for site, multivariable modeling showed G-tube feeding at discharge was associated with longer hospitalization, and N-tube feeding was associated with greater number of discharge medications ( R2 = 0.65, P < .01). After adjusting for site, mean pre–stage II weight-for-age z -score was significantly higher in the oral-only group (−1.4) vs the N-tube–only (−2.2) and G-tube–only (−2.1) groups ( P = .04 and .02, respectively). Conclusions Feeding mode at hospital discharge after the Norwood procedure varied among sites. Prolonged hospitalization and greater number of medications at the time of discharge were associated with tube feeding. Infants exclusively fed orally had a higher weight-for-age z score pre–stage II than those fed exclusively by tube. Exploring strategies to prevent morbidities and promote oral feeding in this highest risk population is warranted.
Background Data routinely captured in clinical registries may be leveraged to enhance efficiency of prospective research. The quality of registry data for this purpose has not been studied, however. ...We evaluated the completeness and accuracy of perioperative data within congenital heart centers’ local surgical registries. Methods Within 12 Pediatric Heart Network (PHN) sites, we evaluated 31 perioperative variables (and their subcategories, totaling 113 unique fields) collected via sites’ local clinical registries for submission to The Society of Thoracic Surgeons Database, compared with chart review by PHN research coordinators. Both used standard STS definitions. Data were collected on 10 subjects for 2 to 5 procedures/site and adjudicated by the study team. Completeness and accuracy (agreement of registry data with medical record review by PHN coordinator, adjudicated by the study team) were evaluated. Results A total of 56,500 data elements were collected on 500 subjects. With regard to data completeness, 3.1% of data elements were missing from the registry, 0.6% from coordinator-collected data, and 0.4% from both. Overall, registry data accuracy was 98%. In total, 94.7% of data elements were both complete/non-missing and accurate within the registry, although there was variation across data fields and sites. Mean total time for coordinator chart review per site was 49.1 hours versus 7.0 hours for registry query. Conclusions This study suggests that existing surgical registry data constitute a complete, accurate, and efficient information source for prospective research. Variability across data fields and sites also suggest areas for improvement in some areas of data quality.
Background Although decellularized cryopreserved valved allografts (DCAs) have reduced immunogenicity, proof of clinical superiority over standard cryopreserved allografts (SCAs) is lacking. To ...assess functional results and durability, we studied a group of patients with DCAs implanted between 2000 and 2005 and compared them with a similar group with SCAs. Methods From July 2000 until January 2005, 47 patients underwent insertion of a DCA between the right ventricle and pulmonary arteries. The DCA patients were compared with 47 age-matched and diagnosis-matched controls receiving SCAs. All patients received pulmonary allografts and were matched for valve position (orthotopic versus heterotopic). We analyzed each group for survival, reoperation, reintervention (surgical or catheter-based), stenosis, and regurgitation. Results There were no differences between groups with respect to weight, age, valve size, or survival. Actuarial freedom from reintervention at 8 years was 79% for DCAs as compared with 63% for SCAs ( p = 0.31, log-rank). Echocardiogram in the DCA group (median 66 months) showed a slightly lower median peak gradient of 16 mm Hg (range, 0 to 82 mm Hg) as compared with 22 mm Hg (range, 0 to 63) in the SCA group (median 61 months, p = 0.051, Wilcoxon). However, when conduits 18 mm or less in diameter were compared, DCA patients had a median peak gradient of 10 mm Hg (range, 0 to 43) compared with 25 mm Hg in SCAs (range, 0 to 55 mm Hg, p = 0.03). There were no differences in the degree of allograft insufficiency in either group. Conclusions Decellularized cryopreserved valved allografts have a nonsignificant trend toward lower peak valve gradient and reintervention in comparison with SCAs. Small valve sizes (18 mm or less) show a slight but significant improvement in peak gradient, but no advantage in valve insufficiency. These findings and a significantly higher cost (>$3,000) make further direct comparisons necessary before widespread use of DCAs can be justified.
Objective Previous reports of neonatal coarctation repair demonstrate a high rate of recurrent arch obstruction in small neonates. This study assesses the effect of patient size on reintervention and ...survival in neonates and infants undergoing repair of simple aortic coarctation. Methods From 1996 to 2006, 167 neonates and infants younger than 90 days with simple coarctation underwent repair. Median patient age was 16 days (range, 1–85 days). Median patient weight was 3.4 kg (range, 0.8–6.0 kg), with 29 patients weighing less than 2.5 kg. All 167 patients included in the study underwent repair through a left thoracotomy. Results There was 1 early death (1/167, 0.6%). Median follow-up of 4.8 years (range, 0–11.8 years) demonstrated 2 late deaths unrelated to recurrent coarctation. Eighteen patients underwent intervention for recurrent arch obstruction a median of 0.48 years postoperatively (range, 0.14–9.8 years). All were treated with balloon angioplasty and have required no additional intervention. Actuarial freedom from reintervention was 90% at 1 year and 89% at 5 years for infants weighing more than 2.5 kg and 89% at 1 year and 86% at 5 years ( P = .31) for infants weighing less than 2.5 kg. There was no difference between survival or reintervention for neonates 30 days of age or younger compared with infants 31 to 90 days of age. Use of polypropylene sutures and female sex did correlate with increased reintervention. Conclusions Low weight does not affect survival or reintervention rates after coarctation repair in neonates and infants less than 3 months of age. Balloon angioplasty is an effective treatment for recurrent obstruction after coarctation repair in infancy. In the current era, timing of the operation should be based on clinical status.
The objective of the present study was to characterize the outcomes and resource utilization of all infants born with hypoplastic left heart syndrome (HLHS) in the Intermountain West. This was a ...retrospective cohort study of all infants born with HLHS in the Intermountain West from January 1995 and January 2010. The cohort was divided into 3 eras: era 1, 1995 to 1999; era 2, 2000 to 2004; and era 3, 2005 to 2010. Cox proportional hazards regression analysis was performed to assess mortality. The lifetime hospitalization days and charges were also determined. Of the 245 infants identified, 65% were male infants and 172 (70%) underwent Stage 1 palliation. The transplant-free survival rate for the entire cohort was 33% at 14 years. The 1-year transplant-free survival rate for the surgical cohort was 60% in era 3. The infants whose initial presentation included shock, restrictive or intact atrial septum, chromosomal defects, or multiorgan dysfunction had an increased risk of death. A recent era of birth, greater birthweight, and older gestational age were associated with improved survival. The factors associated with mortality after stage 1 included surgical procedure type (Blalock-Taussig vs Sano shunt, hazard ratio 2.1), requirement for postoperative extracorporeal membrane oxygenation (hazard ratio 4.2), postoperative renal dysfunction (hazard ratio 3.0), anomalous pulmonary venous return (hazard ratio 2.9), and moderate or greater tricuspid valve regurgitation at any point (hazard ratio 2.0). For patients who had undergone stage 1, 2, or 3 palliation, the median cumulative lifetime hospitalization was 32, 48, and 65 days, and the median cumulative lifetime charges for hospitalization were $201,812, $253,183, and $296,213, respectively. In conclusion, although hospital-based studies of HLHS have shown significantly improved survival after surgical palliation, population-based studies have shown that HLHS continues to have a high mortality and high resource utilization.
Objective The purpose of this analysis was to evaluate a novel strategy for reporting adverse events in the Pediatric Heart Network’s randomized surgical trial of systemic–pulmonary artery shunt ...versus right ventricle–pulmonary artery conduit in infants with hypoplastic left heart syndrome. The strategy was developed to align the reporting process with the needs of a surgical trial while maintaining participant safety. Methods Adverse event reporting was analyzed for 2 groups of study subjects: those randomized to a trial arm during a period in which a standard adverse event reporting system was used (period 1) and those randomized after institution of a system that focused serious adverse event reporting on 6 sentinel events (period 2). The analysis encompassed the period from randomization (Norwood surgery) to hospital discharge from stage II surgery. Adverse event rates were compared using a Poisson regression model for the number of events per subject. Results From period 1 to period 2, the rate of serious adverse events requiring expedited reporting decreased as expected (0.42 vs 0.14/subject/month of follow-up; P < .001). Subjects with a serious (sentinel) adverse event in period 2 had a significantly higher rate of death and cardiac transplantation. Conclusions The new adverse event reporting system successfully targeted subjects at highest risk, while decreasing the administrative burden associated with adverse event reports. This methodology may be of benefit in trials evaluating surgical or device-based interventions and in critically ill populations where many common clinical events would qualify as serious adverse events in the context of a drug trial.
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