Alexandra Ehrlich

Association of Interstage Home Monitoring With Mortality, Readmissions, and Weight Gain A Multicenter Study from the National Pediatric Cardiology Quality Improvement Collaborative

Background— Daily home monitoring of oxygen saturation and weight has been reported to improve outcomes for patients with single-ventricle heart disease during the period between stage I palliation and stage II palliation. However, these studies have been limited to single institutions and used historical control subjects. Our objective was to determine the association of various interstage home monitoring strategies with outcomes using a multicenter cohort with contemporary control subjects. Methods and Results— We performed a retrospective cohort study using prospectively collected data from the National Pediatric Cardiology Quality Improvement Collaborative from 2008 to 2012. We compared interstage mortality, unscheduled readmissions, and change in weight-for-age Z score for various home monitoring strategies of oxygen saturation (n=494) or weight (n=472), adjusting for sex, syndrome, tricuspid regurgitation, arch obstruction, and shunt type. Overall interstage mortality was 8.1%, and 47% had ≥1 unscheduled readmission. We did not find any associations of home oxygen saturation or weight monitoring with mortality or readmission. Although there was no difference in weight-for-age Z score for daily (0.33±0.12) versus weekly (0.34±0.18, P=0.98) weight monitoring, daily home weight monitoring was superior to no home weight monitoring (−0.15±0.18; P<0.01). Conclusions— Home weight monitoring is associated with improved weight gain during the interstage period, but we did not find any benefits in other clinical outcomes for either home oxygen saturation monitoring or home weight monitoring.

Single Ventricle Palliation in Low Weight Patients Is Associated With Worse Early And Midterm Outcomes

BACKGROUND While low weight is an established risk factor for operative mortality after single ventricle (SV) palliation, its influence on late outcomes is not well understood. We examined current-era effects of low weight at time of surgery on hospital mortality, progression through palliative stages, and survival. METHODS Five hundred and thirty infants with SV underwent first-stage palliation (2002 to 2012). Competing risk analysis modeled events after initial surgery and after Glenn. Regression models examined the effect of low weight 2.5 kg or less (n = 77 of 530, 14.5%) on early and late outcomes. RESULTS Initial palliation was Norwood (n = 284, 54%), modified Blalock-Taussig shunt (n = 173, 33%), and pulmonary artery band (n = 73, 14%). Competing risk analysis showed that at 6 months after initial palliation the proportion of patients who had died or received transplantation was 40% in patients 2.5 kg or less and 20% in patients greater than 2.5 kg (p < 0.001). Consequently, the proportion of patients who had progressed to Glenn was 33% in patients 2.5 kg or less and 59% in patients greater than 2.5 kg (p < 0.001). Subsequent to Glenn, progression toward Fontan was unaffected by initial weight. In addition to increased hospital mortality (odds ratio 1.86, 95% confidence interval [CI] 0.93% to 3.70%, p = 0.08); adjusted hazard analysis showed that weight 2.5 kg or less was associated with diminished late survival (hazard ratio 1.65, 95% CI 1.085% to 2.53%, p = 0.02) and that was evident for all palliation types and most SV morphologies. CONCLUSIONS Low weight at time of first-stage SV palliation is associated with an increase in both hospital mortality and interstage attrition, with subsequently fewer patients progressing toward the Glenn operation. The increased death hazard in low weight SV patients persists for almost 1 year after initial palliation, suggesting the need for more vigilant monitoring and out-patient care in those high-risk patients.

Transcatheter Versus Surgical Closure of Atrial Septal Defects in Children: A Value Comparison.

OBJECTIVES The purpose of this study was to determine whether a transcatheter procedure or surgical closure offers a better value proposition for atrial septal defect (ASD) closure. BACKGROUND Secundum ASDs are common congenital heart defects with both transcatheter and surgical treatment options. Although both options have been shown to have excellent results in children, the relative value of the 2 procedures is unclear. METHODS Using data from the Pediatric Hospital Information System for 2004 to 2012, we compared the value of transcatheter versus surgical ASD closure for children ages 1 to 17 years, with value being defined as outcomes relative to costs. Total charges for procedure-related encounters were converted to costs using hospital-specific cost-to-charge ratios, and all costs were adjusted for inflation to reflect 2012 dollars. RESULTS There were 4,606 transcatheter procedures and 3,159 surgeries at 35 childrens hospitals. Those undergoing transcatheter closure were more likely to be older (5.6 years vs. 4.5 years, p < 0.0001). There was no mortality in either group. Children with a surgical procedure had a longer length of stay (4.0 days vs. 1.5 days, p < 0.0001), were more likely to have an infection (odds ratio: 3.73, p < 0.0001) or procedural complication (odds ratio: 6.66, p < 0.0001). Costs for transcatheter procedure encounters were lower than costs for surgical encounters (mean of

TRANSCATHETER VERSUS SURGICAL CLOSURE OF ATRIAL SEPTAL DEFECTS IN CHILDREN: A VALUE COMPARISON

19,128 vs.

Current Outcomes of the Norwood Operation in Patients With Single-Ventricle Malformations Other Than Hypoplastic Left Heart Syndrome

25,359, p < 0.0001). CONCLUSIONS Both transcatheter and surgical ASD closure had excellent short-term outcomes, but transcatheter procedures had lower lengths of stay, rates of infection, and complications, resulting in lower overall costs. For children who are eligible, transcatheter ASD closure provides better short-term value than surgery.

Late Outcomes of Infants Supported by Extracorporeal Membrane Oxygenation Following the Norwood Operation

Secundum atrial septal defects (ASDs) are common congenital heart defects with both transcatheter and surgical treatment options. While both options have been shown to have excellent results in children, the relative value of the two procedures is unknown. The purpose of this study was to determine

Improvement in Tricuspid Annular Plane Systolic Excursion with Pulmonary Hypertension Therapy in Pediatric Patients

Background: Subsequent to increased experience with the Norwood operation in children with hypoplastic left heart syndrome (HLHS), its application has expanded to allow palliation of single-ventricle (SV) malformations other than HLHS. We describe current palliation outcomes in this group of SV patients. Methods: Between 2002 and 2012, 65 of the 303 Norwood operations were performed in non-HLHS SV patients. Competing risk analysis modeled events after Norwood and after subsequent Glenn and examined risk factors affecting outcomes. Results: Competing risk analysis showed that one year following Norwood, 24% of patients had died or received transplantation, 72% had undergone Glenn, and 4% were alive awaiting Glenn/Kawashima. Five years following Glenn, 9% of patients had died, 68% had undergone Fontan, and 23% were alive awaiting Fontan. Overall seven-year survival following Norwood was 68%. On multivariable analysis, mortality risk factors were unplanned cardiac reoperation (hazard ratio [HR]: 4.0 [1.5-10.6], P = .006), right dominant ventricle morphology (HR: 3.3 [1.3-8.3], P = .012), and postoperative extracorporeal membrane oxygenation (HR: 3.1 [1.1-9.0], P = .035). Conclusions: Operative death and interstage mortality continue to be problematic following Norwood palliation for non-HLHS SV variants. Outcomes seem comparable to those reported for HLHS, however they are influenced by underlying pathology; children with dominant left ventricle morphology (tricuspid atresia and double inlet left ventricle) have superior survival compared to those with dominant right ventricle morphology (mitral atresia, unbalanced atrioventricular septal defect, and most patients with atrial isomerism). Unplanned reoperations for technical imperfections diminish survival. Large multicenter studies might be warranted to better identify high-risk patients and provide guidance toward improving their survival.

Novel Electrocardiographic Screening Criterion for Hypertrophic Cardiomyopathy in Children

Background: Hospital survival for infants who require extracorporeal membrane oxygenation (ECMO) following the Norwood operation is 30% to 60%. However, little is known about late outcomes of hospital survivors and their ability to progress through subsequent palliative stages. Methods: Between 2002 and 2012, 38 (13.4%) of the 284 neonates with hypoplastic left heart syndrome or other single ventricle variants received ECMO support following Norwood. We examined factors affecting hospital death and compared postdischarge events between hospital survivors who received postoperative ECMO (n = 16 of 38) and a control of hospital survivors who did not receive ECMO (220 of 246). Results: Unplanned cardiac reoperation was the only predictor of postoperative ECMO requirement. Overall, 22 (58%) of the 38 patients were weaned from ECMO support and 16 (42%) of the 38 survived to hospital discharge. The ECMO duration was a significant factor for hospital mortality (odds ratio = 1.52 per 1-day increase [1.03-2.24], P = .035). Following discharge, 15 (94%) of the 16 underwent Glenn and 1 (6%) of the 16 had interstage mortality. In the control group, 194 (88%) of the 220 underwent Glenn and 26 (12%) of the 220 had interstage mortality or received transplantation (P = .499). Following Glenn, 3 (20%) of the 15 patients had interstage mortality or received transplantation and 12 (80%) of the 15 proceeded to Fontan or were alive awaiting Fontan. In the control group, 23 (12%) of the 194 had interstage mortality or received transplantation and 171 (88%) proceeded to Fontan or were alive awaiting Fontan (P = .357). Overall, 81% of hospital survivors were alive 5 years following discharge in both ECMO and non-ECMO groups. Conclusions: ECMO support following Norwood is associated with high probability of hospital death. Nonetheless, interstage mortality, progression to subsequent palliative stages, intermediate survival, and freedom from heart transplantation are comparable to those in patients who did not require postoperative ECMO support.

Elevated tricuspid regurgitant velocity as a marker for pulmonary hypertension in children with sickle cell disease: less prevalent and predictive than previously thought?

Tricuspid annular plane systolic excursion (TAPSE) has emerged as a novel and reliable measure of right ventricular (RV) function. The purpose of this study was to determine the effect of pulmonary hypertension (PH) therapy on TAPSE in pediatric patients and compare TAPSE to other quantitative measures of RV function.

Tissue Plasminogen Activator Use in Children: Bleeding Complications and Thrombus Resolution.

Electrocardiography is often advocated as a screening tool in children for hypertrophic cardiomyopathy (HC). We sought to establish an electrocardiographic screening tool to identify children with HC. We hypothesized that a pediatric-specific electrocardiographic criterion would perform better than the popular criteria used for screening children for left ventricular hypertrophy and HC. The earliest available electrocardiogram for children (n=108) with HC (ages 7 to 21 yrs) was reviewed. We sought to compare the diagnostic accuracy of 4 screening algorithms: (1) Sokolow-Lyon criterion (SV1+RV5/RV6>35 mm), (2) Cornell criterion (RaVL+SV3>28 mm in men, 20 mm in women), (3) total 12-lead voltage criterion (R wave to the nadir of Q/S wave>175 mm), and (4) pediatric-specific criterion (RaVL+SV2>23 mm). The same criteria were applied to a cohort of age-matched and gender-matched controls without cardiac disease. Statistically significant correlations were found between children with HC and positive screen using all 4 criteria. However, comparison of receiver operating characteristic demonstrated an area under the curve of 0.67 for Sokolow-Lyon criterion, 0.70 for Cornell criterion, 0.83 for total 12-lead criterion, and 0.82 for pediatric-specific criterion. Pediatric-specific criterion had superior sensitivity in gene-positive children and superior overall specificity than total 12-lead criterion. In conclusion, our study demonstrates that the pediatric-specific criterion employing leads RaVL+SV2 is more accurate in identifying children with HC in comparison with other popular screening criteria.