Assessing enrollment of eligible infants in the national pediatric cardiology quality improvement collaborative (NPC-QIC) through linkage to the pediatric cardiac critical care consortium (PC4) registry.

BACKGROUND: The National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC) lacks a rigorous enrollment audit process, unlike other collaborative networks. Most centers require individual families to consent to participate. It is unknown whether there is variation across centers or biases in enrollment. METHODS: We used the Pediatric Cardiac Critical Care Consortium (PC4) registry to assess enrollment rates in NPC-QIC for those centers participating in both registries using indirect identifiers (date of birth, date of admission, gender, and center) to match patient records. All infants born 1/1/2018-12/31/2020 and admitted 30 days of life were eligible. In PC4, all infants with a fundamental diagnosis of hypoplastic left heart or variant or who underwent a surgical or hybrid Norwood or variant were eligible. Standard descriptive statistics were used to describe the cohort and center match rates were plotted on a funnel chart. RESULTS: Of 898 eligible NPC-QIC patients, 841 were linked to 1,114 eligible PC4 patients (match rate 75.5%) in 32 centers. Match rates were lower in patients of Hispanic/Latino ethnicity (66.1%, p = 0.005), and those with any specified chromosomal abnormality (57.4%, p = 0.002), noncardiac abnormality (67.8%, p = 0.005), or any specified syndrome (66.5%, p = 0.001). Match rates were lower for patients who transferred to another hospital or died prior to discharge. Match rates varied from 0 to 100% across centers. CONCLUSIONS: It is feasible to match patients between the NPC-QIC and PC4 registries. Variation in match rates suggests opportunities for improvement in NPC-QIC patient enrollment.

Incidence and outcome of arrhythmias and electrical disease in patients with Trisomy 18.

Patients with Trisomy 18 have a high incidence of cardiac anomalies and are associated with early death. Because of early mortality, electrical system disease and arrhythmia has been difficult to delineate and the incidence remain unknown. We sought to describe the association and clinical outcomes of electrical system disease and cardiac tachy-arrhythmias in patients with Trisomy 18. This was a retrospective, single institutional study. All patients with Trisomy 18 were included in the study. Patient characteristics, congenital heart disease (CHD), conduction system and clinical tachy-arrhythmia data were collected on all patients. Outcomes including cardiac surgical interventions, electrical system interventions and death were collected until the time of study. Patients with tachy-arrhythmias/electrical system involvement were compared to those without to identify potential associated variables. A total of 54 patients with Trisomy 18 were included in analysis. The majority of patients was female and had associated CHD. AV nodal conduction system abnormalities with either first or second degree AV block were common (15%) as was QTc prolongation (37%). Tachy-arrhythmias were common with 22% of patients having at least one form of tachy-arrhythmia and associated with concomitant conduction system disease (p = 0.002). Tachy-arrhythmias were typically treatable with monitoring or medication with eventual resolution without need for procedural intervention. Although early death was common, there were no causes of death associated with tachy-arrhythmia or conduction system disease. In conclusion, patients with Trisomy 18 have a high incidence of conduction system abnormalities and burden of clinical tachy-arrhythmias. Although frequent, electrical system disease did not affect patient outcome or difficultly of care delivery.

The NEONATE score predicts freedom from interstage mortality or transplant in a modern cohort.

BACKGROUND: Derived from the National Pediatric Cardiology Quality Improvement Collaborative registry, the NEONATE risk score predicted freedom from interstage mortality or heart transplant for patients with single ventricle CHD and aortic arch hypoplasia discharged home following Stage 1 palliation. OBJECTIVES: We sought to validate the score in an external, modern cohort. METHODS: This was a retrospective cohort analysis of single ventricle CHD and aortic arch hypoplasia patients enrolled in the National Pediatric Cardiology Quality Improvement Collaborative Phase II registry from 2016 to 2020, who were discharged home after Stage 1 palliation. Points were allocated per the NEONATE score (Norwood type-Norwood/Blalock-Taussig shunt: 3, Hybrid: 12; extracorporeal membrane oxygenation post-op: 9, Opiates at discharge: 6, No Digoxin at discharge: 9, Arch Obstruction on discharge echo: 9, Tricuspid regurgitation ≥ moderate on discharge echo: 12; Extra oxygen plus ≥ moderate tricuspid regurgitation: 28). The composite primary endpoint was interstage mortality or heart transplant. RESULTS: In total, 1026 patients met inclusion criteria; 61 (6%) met the primary outcome. Interstage mortality occurred in 44 (4.3%) patients at a median of 129 (IQR 62,195) days, and 17 (1.7%) were referred for heart transplant at a 167 (114,199) days of life. The median NEONATE score was 0(0,9) in those who survived to Stage 2 palliation compared to 9(0,15) in those who experienced interstage mortality or heart transplant (p < 0.001). Applying a NEONATE score cut-off of 17 points that separated patients into low- and high-risk groups in the learning cohort provided 91% specificity, negative predictive value of 95%, and overall accuracy of 87% (85.4-89.5%). CONCLUSION: In a modern cohort of patients with single ventricle CHD and aortic arch hypoplasia, the NEONATE score remains useful at discharge post-Stage 1 palliation to predict freedom from interstage mortality or heart transplant.

Randomized Controlled Trial of Interactive Spaced Education to Support Constipation Management by Pediatric Primary Care Providers.

INTRODUCTION: Integrating continuing medical education and quality improvement (QI) initiatives is challenging. We aimed to compare one method, Interactive Spaced Education (ISE/QI), with standard (sTD/Qi) education embedded within a constipation management Qi initiative. METHODS: We conducted a randomized, controlled study to compare ISE/QI and STD/QI education. Pediatric primary care providers (PCPs) were recruited from a network of local private practices. The QI initiative was implemented with all providers before education interventions. ISE/QI participants received questions by email weekly, provided answers, received feedback, and repeated questions over a 4-month period. The STD/QI group received a Power Point with the same educational content. Pre- and post-surveys evaluated usability, self-assessed confidence, and practice changes while quizzes evaluated knowledge. Process control charts tracked subsequent visits to gastroenterology (GI). RESULTS: Of the 212 eligible PCPs, 101 (48%) enrolled, with 49 PCPs in the ISE/QI arm and 52 in STD/QI education arm. Quiz scores improved in the ISE/QI arm with a strong effect size (Cohen d 1.76). Mean increase in confidence managing difficult cases was higher in the ISE/QI group (1.84 vs 1.21, P  = 0.030). ISE/QI participants were more likely to rate the activity better than most online education (odds ratio [OR] 18.1, P < 0.0001) and incorporate practice changes (OR 3.35, P = 0.0152). Visits to GI decreased among the entire population, but the effect on GI visits within each education arm was mixed. CONCLUSIONS: ISE/QI improved knowledge and confidence managing difficult cases. ISE/QI participants reported higher likelihood to change practice, but no differences were seen in GI referrals.

Outcomes in Infants with Supraventricular Tachycardia: Risk Factors for Readmission, Recurrence and Ablation.

Supraventricular tachycardia (SVT) is the most common arrhythmia in infants. Once diagnosed, infants are admitted for antiarrhythmic therapy and discharged after observation. There are limited data on risk factors for readmission and readmission rates, while on medication. The objective of this study was to investigate risk factors for readmission and outcomes in infants diagnosed with SVT. This is a single-center retrospective study over a 10-year period of infants under 6 months of age with documented SVT. Infants with congenital heart disease requiring surgical or catheter intervention, gestational age less than 32 weeks or diagnosis of atrial flutter or fibrillation were excluded. The primary outcome was readmission within 31 days of hospital discharge. Long term need for ablation and eventual discontinuation of medications were assessed. Ninety patients were included. Beta blockers were the initial therapy in 66 and 28 required a medication change. Nineteen were readmitted within 31 days of discharge. The only clinical factor associated with early readmission was presence of ventricular pre-excitation (6/19 vs. 8/71, p = 0.03). Patients who were readmitted within 31 days had a longer length of treatment (12 [11.5, 22.0] vs. 10 [7.5, 12.0] months, p = 0.007) and were more likely to undergo ablation (4/19 vs. 2/71, p = 0.017). In this cohort of infants with SVT, readmission was common and ventricular pre-excitation was identified as a risk factor for readmission. Infants who were readmitted within 31 days of discharge had longer length of antiarrhythmic therapy and were more likely to undergo catheter ablation.

Socioeconomic Impact on Outcomes During the First Year of Life of Patients with Single Ventricle Heart Disease: An Analysis of the National Pediatric Cardiology Quality Improvement Collaborative Registry.

Socioeconomic status (SES) affects a range of health outcomes but has not been extensively explored in the single ventricle population. We investigate the impact of community-level deprivation on morbidity and mortality for infants with single ventricle heart disease in the first year of life. Retrospective cohort analysis of infants enrolled in the National Pediatric Cardiology Improvement Collaborative who underwent staged single ventricle palliation examining mortality and length of stay (LOS) using a community-level deprivation index (DI). 974 patients met inclusion criteria. Overall mortality was 20.5%, with 15.7% of deaths occurring between the first and second palliations. After adjusting for clinical risk factors, the DI was associated with death (log relative hazard [Formula: see text] = 8.92, p = 0.030) and death or transplant (log relative hazard [Formula: see text] = 8.62, p = 0.035) in a non-linear fashion, impacting those near the mean DI. Deprivation was associated with LOS following the first surgical palliation (S1P) (p = 0.031) and overall hospitalization during the first year of life (p = 0.018). For every 0.1 increase in the DI, LOS following S1P increased by 3.35 days (95% confidence interval 0.31-6.38) and total hospitalized days by 5.08 days (95% CI 0.88-9.27). Community deprivation is associated with mortality and LOS for patients with single ventricle congenital heart disease. While patients near the mean DI had a higher hazard of one year mortality compared to those at the extremes of the DI, LOS and DI were linearly associated, demonstrating the complex nature of SES factors.

Progressive QRS Duration and Ventricular Dysfunction in Pediatric Patients with Chronic Ventricular Pacing.

Pacemakers are a mainstay of therapy for patients with congenital and acquired heart block, but ventricular pacing is related to ventricular dysfunction. We sought to evaluate patient and device characteristics associated with ventricular dysfunction in pediatric patients with chronic ventricular pacing. This was a retrospective cohort of pediatric patients with heart block and chronic ventricular pacing. Patient, ECG, and device characteristics were analyzed to determine factors associated with ventricular dysfunction. Longitudinal ECG and echocardiogram parameters were obtained to track changes in QRS and systemic ventricular systolic function over time. In total, 82 patients were included (median age at implant 0.81 years). Over a follow-up time of 6.1 years, 18% developed ventricular dysfunction. Patients with dysfunction had greater current QRS duration (p = 0.002) compared to those with preserved function with a similar time from device implantation. There was no difference between lead location or age at device implantation. QRS duration increased with time from implant and the resultant ΔQRS was associated with ventricular dysfunction (p = 0.01). QRS duration >162 ms was associated with a 5.8 (2-9)-fold increased risk for dysfunction. Transvenous leads were associated with longer QRS duration with no difference compared to epicardial leads in development of ventricular dysfunction. This study demonstrated that the absolute paced QRS duration and Δpaced QRS were association with long-term ventricular dysfunction independent of how long a given patient was paced. Patients in high-risk categories may benefit from close echocardiographic monitoring. Whether permissive junctional rhythm or His bundle/biventricular pacing decreases the rate of dysfunction needs further study.

Characteristics of Interstage Death After Discharge from Stage I Palliation.

BACKGROUND: Interstage mortality (IM) remains high for patients with single-ventricle congenital heart disease (SVCHD) in the period between Stage 1 Palliation (S1P) and Glenn operation. We sought to characterize IM. METHODS: This was a descriptive analysis of 2184 patients with SVCHD discharged home after S1P from 60 National Pediatric Cardiology Quality Improvement Collaborative sites between 2008 and 2015. Patients underwent S1P with right ventricle-pulmonary artery conduit (RVPAC), modified Blalock-Taussig-Thomas shunt (BTT), or Hybrid; transplants were excluded. RESULTS: IM occurred in 153 (7%) patients (median gestational age 38 weeks, 54% male, 77% white), at 88 (IQR 60,136) days of life, and 39 (IQR 17,84) days after hospital discharge; 13 (8.6%) occurred ≤ 30 days after S1P. The mortality rate for RVPAC was lower (5.2%; 59/1138) than BTT (9.1%; 65/712) and Hybrid (20.1%; 27/134). More than half of deaths occurred at home (20%) or in the emergency department (33%). The remainder occurred while inpatient at center of S1P (cardiac intensive care unit 36%, inpatient ward 5%) or at a different center (5%). Fussiness and breathing problems were most often cited as harbingers of death; distance to surgical center was the biggest barrier cited to seeking care. Cause of death was unknown in 44% of cases overall; in the subset of patients who underwent post-mortem autopsy, the cause of death remained unknown in 30% of patients, with the most common diagnosis being low cardiac output. CONCLUSIONS: Most IM occurred in the outpatient setting, with non-specific preceding symptoms and unknown cause of death. These data indicate the need for research to identify occult causes of death, including arrhythmia.

Implementation of a Pediatric Chest Pain Local Consensus Guideline Decreases the Total Tests Performed Without Negatively Affecting the Yield of Abnormal Cardiac Results.

Pediatric chest pain is common and though usually benign often leads to unnecessary diagnostic testing. There is limited evidence as to whether a local consensus guideline can decrease testing frequency without negatively affecting the overall yield. In addition, it is unknown whether the addition of pulmonary function testing to a cardiopulmonary exercise test increases the diagnostic yield in pediatric patients with chest pain. A retrospective chart review was performed on all new pediatric patients who presented with chest pain at our academic center's pediatric cardiology clinic 18 months before and after the implementation of a standard management guideline. Data from the encounter-associated echocardiogram, cardiopulmonary exercise test, and pulmonary function test, when available, were analyzed. There were no significant differences in patient volume or demographic characteristics in the 18 months before (n = 768) and after (n = 778) guideline implementation. There were significant reductions in the number of ordered echocardiograms (n = 131; 17% vs. n = 75; 9.6%, p < 0.001) and cardiopulmonary exercise tests (n = 46; 6% vs. n = 29; 4%, p = 0.04) with no concerning pathology discovered in either group. Associated pulmonary function testing performed prior to with exercise testing discovered abnormalities in 19% of the total patients tested. The implementation of a local consensus guideline for pediatric chest pain results in fewer unnecessary tests ordered. There was no concerning pathology before or after guideline implementation, therefore conclusions regarding the diagnostic yield of these guidelines are unfeasible. The addition of pulmonary function testing to cardiopulmonary exercise tests increases the potential diagnostic yield in these patients.

The Fontan outcomes network: first steps towards building a lifespan registry for individuals with Fontan circulation in the United States.

The Fontan Outcomes Network was created to improve outcomes for children and adults with single ventricle CHD living with Fontan circulation. The network mission is to optimise longevity and quality of life by improving physical health, neurodevelopmental outcomes, resilience, and emotional health for these individuals and their families. This manuscript describes the systematic design of this new learning health network, including the initial steps in development of a national, lifespan registry, and pilot testing of data collection forms at 10 congenital heart centres.

Can noninvasive testing identify benign patterns of suggested pre-excitation on electrocardiogram?

BACKGROUND: The presence of anterograde conduction through an accessory pathway (AP) has been linked to sudden cardiac death. Unfortunately, pre-excitation associated with classic pathways can be difficult to differentiate from benign APs such as nodofascicular fibers. OBJECTIVE: Identifying characteristics on electrocardiogram (ECG) and exercise that differentiate classic and benign AP connections in suggested pre-excitation patterns. METHODS: Retrospective review of patients presenting between 1995 and 2017 with ventricular pre-excitation on ECG, determined to have either typical left-lateral AP during electrophysiology study (EPS), or benign, or no AP determined by either transesophageal electrophysiology study (TEP), or EPS. RESULTS: A total of 96 patients were included, 14.2 years (4-24), 45% female, 90% Caucasian. Of these, 60 (63%) had a classic APs identified on EPS and 58 (97%) underwent successful ablation. Conversely, 36 (37%) had benign pathways identified. ECG findings differed between the groups: PR-interval 102 versus 120 ms (P < .0001), QRS-duration 110 versus 102 ms (P < .0001), QRS-axis 74 versus 59 degrees (P = .0005), and QRS onset to peak R/S in limb leads 64 versus 42 ms (P < .0001), and precordial leads 66 versus 46 ms (P < .0001). Change in QRS duration during exercise differed between the groups: 25 versus 2 ms (P < .0001) and ECG characteristics identified the presence of an AP with 97% sensitivity and 94% negative predictive value. CONCLUSION: Classic and benign APs exhibit different ECG characteristics, though clinical overlap does not allow for absolute differentiation. These data may help with risk stratification decision making though does not obviate the need for additional invasive testing.

Enhancing efficiency and scientific impact of a clinical trials network: the Pediatric Heart Network Integrated CARdiac Data and Outcomes (iCARD) Collaborative.

Recent years have seen an exponential increase in the variety of healthcare data captured across numerous sources. However, mechanisms to leverage these data sources to support scientific investigation have remained limited. In 2013 the Pediatric Heart Network (PHN), funded by the National Heart, Lung, and Blood Institute, developed the Integrated CARdiac Data and Outcomes (iCARD) Collaborative with the goals of leveraging available data sources to aid in efficiently planning and conducting PHN studies; supporting integration of PHN data with other sources to foster novel research otherwise not possible; and mentoring young investigators in these areas. This review describes lessons learned through the development of iCARD, initial efforts and scientific output, challenges, and future directions. This information can aid in the use and optimisation of data integration methodologies across other research networks and organisations.

Longitudinal Health Care Cost in Hypoplastic Left Heart Syndrome Palliation.

Management of hypoplastic left heart syndrome (HLHS) is resource intensive. Heath care systems are pressured to provide value to patients by improving outcomes while decreasing costs. A single-center retrospective cohort of infants with HLHS who underwent Norwood procedure or hybrid Norwood from 2004 to 2014 and survived to first outpatient follow up were studied. The primary outcome was total cost through 12 months with a sub-analysis of patients with 60 months of data. Costs were calculated using internal cost accounting system and reported by cost center. Of the 152 HLHS patients identified, 69 met inclusion criteria. Stage I hospitalization (n = 69), with a median length of stay 34 days [interquartile range (IQR) 24-58 days], resulted in a median cost of $203,817 (IQR $136,236-272,453). Of survivors at 12 months (n = 55), the median cost was $369,393 (IQR $216,289-594,038) generated in part by a median of 67 (40-126 days) hospitalized days during that year. A subgroup analysis of patients who reached 60 months of age (n = 29) demonstrated a median total cost of $391,812 (IQR $293,801-577,443) and a median of 74 lifetime hospitalized days (IQR 58-116 days). High cost centers included intensive care (41%), non-ICU hospital (17%), operative services (11%), catheterization lab (9%), and pharmacy (9%). Using multiple regression analysis, significant drivers of cost included reoperation, length of hospitalization, low birthweight, and use of ECMO. Costs related to HLHS management are driven both by care-related complications such as surgical re-intervention and patient factors such as low birth weight.

Assessment of Growth 6 Years after the Norwood Procedure.

At 6 years of age, patients with hypoplastic left heart syndrome had mean age-adjusted z-scores for weight and height below the normative population, and body mass index was similar to the normative population. Males had the greatest increase in z-scores for body mass index. TRIAL REGISTRATION: ClinicalTrials.gov: NCT00115934.

Early Repolarization in Normal Adolescents is Common.

In adults with structurally normal hearts, the early repolarization pattern (ERP) on electrocardiogram (ECG) may be associated with an increased risk of sudden cardiac death. The prevalence and significance of the ERP in children is unknown. This study examines the prevalence of the ERP, the population in which it is found, and whether there exists any correlation with increased LV mass or family history of significant cardiac events. This was a secondary review of data obtained from healthy adolescents undergoing a limited ECG and transthoracic echocardiogram (TTE) as part of a cardiac screening study. Subjects were excluded if ECG revealed known arrhythmic syndromes or TTE revealed structural abnormalities. ERP was defined as (1) notching or slurring of the terminal QRS; (2) elevation of the QRS-ST junction ≥1 mV; and (3) upwardly concave positive T-wave. Left ventricular (LV) mass was defined as mass/height2.7. Patient demographics, LV mass, family history of sudden death, arrhythmia, and/or ICD/pacemaker placement were compared for subjects with and without ERP on ECG. Data from 575 subjects (median age 15, range 13-18; 36% female; 93% Caucasian) were reviewed. The incidence of ERP was 40% (n = 228) and was seen in the inferior, lateral, or combination of these leads in 42, 10, and 48% of subjects with ERP, respectively. There was no difference in gender (p = 0.7), race (p = 0.7), age (p = 0.3), history of syncope (p = 0.2), LV mass (p = 0.8), family history of (a) sudden death (p = 0.5), (b) arrhythmia (p = 0.2), or (c) ICD/pacemaker requirement (p = 0.8) in subjects with ERP versus those without. However, a greater percentage of patients with ERP were noted to play football, when compared to those without ERP (34 vs. 13%, p < 0.001). ERP is common in healthy adolescents, and does not correlate with concerning personal/family history or elevated LV mass. Longitudinal studies are required to determine whether ERP in childhood confers an increased mortality risk.

Variability of antithrombotics use in patients with hypoplastic left heart syndrome and its variants following first- and second-stage palliation surgery: a national report using the National Pediatric Cardiology Quality Improvement Collaborative registry.

Purpose Patients with hypoplastic left heart syndrome and its variants following palliation surgery are at risk for thrombosis. This study examines variability of antithrombotic practice, the incidence of interstage shunt thrombosis, and other adverse events following Stage I and Stage II palliation within the National Pediatric Cardiology Quality Improvement Collaborative registry. METHODS: We carried out a multicentre, retrospective review using the National Pediatric Cardiology Quality Improvement Collaborative registry including patients from 2008 to 2013 across 52 surgical sites. Antithrombotic medications used at Stage I and Stage II discharge were evaluated. Variability of antithrombotics use at the individual patient level and intersite variability, incidence of shunt thrombosis, and other adverse events such as cardiac arrest, seizure, stroke, and need for cardiac catheterisation intervention in the interstage period were identified. Antithrombotic strategies for hybrid Stage I patients were evaluated but they were excluded from the variability and outcomes analysis. RESULTS: A total of 932 Stage I and 923 Stage II patients were included in the study: 93.8% of Stage I patients were discharged on aspirin and 4% were discharged on no antithrombotics, and 77% of Stage II patients were discharged on aspirin and 17.5% were discharged on no antithrombotics. Only three patients (0.2%) presented with interstage shunt thrombosis. The majority of patients who died during interstage or required shunt dilation and/or stenting were discharged home on aspirin. CONCLUSION: Aspirin is the most commonly used antithrombotic following Stage I and Stage II palliation. There is more variability in the choice of antithrombotics following Stage II compared with Stage I. The incidence of interstage shunt thrombosis and associated adverse events was rare.

Arrhythmic Burden and Ambulatory Monitoring of Pediatric Patients with Cardiomyopathy.

BACKGROUND: Pediatric patients with cardiomyopathy (CM) are at risk for sudden cardiac death (SCD), likely driven by arrhythmic etiologies. OBJECTIVES: Describe arrhythmia burden and Holter utility in pediatric CM including: hypertrophic CM (HCM), dilated CM (DCM), and restrictive CM (RCM). METHODS: Retrospective cohort study of patients <21 years with CM. Patient demographics, arrhythmic history, and genetic status were reviewed including outcomes of death, aborted SCD, and device shocks. Holter findings were analyzed over the prior 5 years including clinically significant findings and resulting changes to management. Analysis for the composite outcomes of death, aborted SCD, and appropriate shock were performed using logistic regression with backward elimination. RESULTS: One hundred and forty-six patients were included: 83 HCM, 54 DCM, and nine RCM (mean 13 ± 6 years). A total of 23% of patients had defibrillators. There were six deaths (two SCD), four patients with appropriate device therapies, and four aborted SCD episodes. In total, 305 Holter monitors were reviewed. Six Holters had significant findings, all nonsustained ventricular tachycardia. Two Holters resulted in changes in management, both defibrillator implantations. Twelve patients had one or more of the conditions defining the composite outcome. Using logistic regression, clinical history of ventricular arrhythmia, frequent premature ventricular complexes, and CM type were included as potential independent predictors in the final model and clinical ventricular arrhythmia and RCM disease were associated with the composite outcome. CONCLUSIONS: SCD and device therapies were relatively rare. Routine Holter screening rarely demonstrated significant findings or changed clinical care. Clinical history of ventricular arrhythmia was associated with poor clinical outcome.

Variation in Antiarrhythmic Management of Infants Hospitalized with Supraventricular Tachycardia: A Multi-Institutional Analysis.

Supraventricular tachycardia (SVT) is the most frequent form of symptomatic tachyarrhythmia in infants. The purposes of this study were to describe practice patterns of the management of infants hospitalized with SVT and factors associated with 30-day hospital readmission. This was a multi-institutional, retrospective review of the pediatric health information system database of SVT hospitalizations from 2003 to 2013. High-volume centers (HVC) were defined as those at the upper quartile of admissions. Infants with an ICD-9 code of paroxysmal SVT were included. Antiarrhythmics investigated included amiodarone, atenolol, digoxin, esmolol, flecainide, procainamide, propafenone, propranolol, and sotalol. Frequency of antiarrhythmic use based on center volume was the primary end point. Rate of 30-day SVT readmission was the secondary end point. Analysis of factors associated with readmission was assessed by Chi-square analysis and expressed as odds ratio and 95 % confidence interval. A total of 851 patients (60 % male, 44 % neonates) were hospitalized at 43 hospitals. Propranolol, digoxin, and amiodarone were the most frequently utilized antiarrhythmics. HVCs represented 12 hospitals comprising 494 (58 %) patients. Although HVCs were more likely to utilize propranolol (OR 2.5, CI 1.5-4.1), there was no significant difference in the 30-day readmission rate between patients treated at HVCs versus non-HVCs (p = 0.9). The majority of infants with SVT are treated with a small number of antiarrhythmic medications during index hospitalization. Although hospital-to-hospital variation in antiarrhythmic choice exists, there appears to be no difference in readmission. The remaining practice variation may be related to intrinsic patient characteristics.

Quality improvement through collaboration: the National Pediatric Quality improvement Collaborative initiative.

PURPOSE OF REVIEW: The National Pediatric Quality Improvement Collaborative (NPCQIC) was established to improve outcomes and quality of life in children with hypoplastic left heart syndrome and other single ventricle lesions requiring a Norwood operation. The NPCQIC consists of a network of providers and families collecting longitudinal data, conducting research, and using quality improvement science to decrease variations in care, develop and spread best practices, and decrease mortality. RECENT FINDINGS: Initial descriptive investigation of the collaborative data found interstage care process variations, different surgical strategies, diverse feeding practices, and variable ICU approaches between centers and within sites. Analysis and evaluation of these practice variations have allowed centers to learn from each other and implement change to improve processes. There has been an improvement in performance measures and most importantly, a 39.7% reduction in mortality. SUMMARY: The NPCQIC has shown, in a rare disease such as hypoplastic left heart syndrome that a network based on multicenter collaboration, patient (parent) engagement, and quality improvement science can facilitate change in practices and improvement in outcomes.

Autonomic dysfunction: a driving force for myocardial fibrosis in young Duchenne muscular dystrophy patients?

Cardiac manifestations of Duchenne muscular dystrophy (DMD) include progressive cardiac dysfunction and an elevated resting heart rate (HR). We hypothesized this elevated HR reflects autonomic dysfunction that can be identified by heart rate variability (HRV) analyses which will be associated with myocardial fibrosis by cardiac magnetic resonance imaging (cMR). DMD patients (N = 74) and controls (N = 17) had time and frequency domain HRV analyses calculated via Holter monitoring. Cardiac magnetic resonance imaging was performed on DMD cases only. χ (2) test, T test, ANOVA, and logistic regression were used to perform comparisons between groups. A p value of <0.05 was used for statistical significance. DMD cases had higher resting average HR than controls (99.4 ± 8.9, 85.4 + 6.2, p < 0.001). Among HRV variables, decreases were seen in the following: standard deviation of R to R intervals, the percent RR intervals differing by >50 ms from previous RR interval, the root-meansquare of successive differences of RR intervals, the standard deviation of the mean R to R segment (SDANN), low frequency, and high frequency domain, all p values 0.001. Maximum HR and SDANN most significantly associated with positive LGE on cMR (p = 0.008, p = 0.016). DMD cases on beta blocker had an average HR lower than those not on beta blocker (p = 0.009), but with no difference in HRV analysis. DMD patients have reduced HRV and therefore autonomic dysfunction prior to the onset of heart failure which is associated with myocardial fibrosis.