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To test associations between parent-reported confidence to avoid hospitalization and caregiving strain, activation, and health-related quality of life (HRQOL).In this prospective cohort study, enrolled parents of children with medical complexity (n = 75) from 3 complex care programs received text messages (at random times every 2 weeks for 3 months) asking them to rate their confidence to avoid hospitalization in the next month. Low confidence, as measured on a 10-point Likert scale (1 = not confident; 10 = fully confident), was defined as a mean rating5. Caregiving measures included the Caregiver Strain Questionnaire, Family Caregiver Activation in Transition (FCAT), and caregiver HRQOL (Medical Outcomes Study Short Form 12 [SF12]). Relationships between caregiving and confidence were assessed with a hierarchical logistic regression and classification and regression trees (CART) model.The parents were mostly mothers (77%) and were linguistically diverse (20% spoke Spanish as their primary language), and 18% had low confidence on average. Demographic and clinical variables had weaker associations with confidence. In regression models, low confidence was associated with higher caregiver strain (aOR, 3.52; 95% CI, 1.45-8.54). Better mental HRQOL was associated with lower likelihood of low confidence (aOR, 0.89; 95% CI, 0.80-0.97). In the CART model, higher strain similarly identified parents with lower confidence. In all models, low confidence was not associated with caregiver activation (FCAT) or physical HRQOL (SF12) scores.Parents of children with medical complexity with high strain and low mental HRQOL had low confidence in the range in which intervention to avoid hospitalization would be warranted. Future work could determine how adaptive interventions to improve confidence and prevent hospitalizations should account for strain and low mental HRQOL.

Objective Because children with medical complexity (CMC) display very different health trajectories, needs, and resource utilization than other children, it is unclear how well traditional conceptions of population health apply to CMC. We sought to identify key health outcome domains for CMC as a step toward determining core health metrics for this distinct population of children. Methods We conducted and analyzed interviews with 23 diverse national experts on CMC to better understand population health for CMC. Interviewees included child and family advocates, health and social service providers, and research, health systems, and policy leaders. We performed thematic content analyses to identify emergent themes regarding population health for CMC. Results Overall, interviewees conveyed that defining and measuring population health for CMC is an achievable, worthwhile goal. Qualitative themes from interviews included: 1) CMC share unifying characteristics that could serve as the basis for population health outcomes; 2) optimal health for CMC is child specific and dynamic; 3) health of CMC is intertwined with health of families; 4) social determinants of health are especially important for CMC; and 5) measuring population health for CMC faces serious conceptual and logistical challenges. Conclusions Experts have taken initial steps in defining the population health of CMC. Population health for CMC involves a dynamic concept of health that is attuned to individual, health-related goals for each child. We propose a framework that can guide the identification and development of population health metrics for CMC.

To evaluate the associations between parent confidence in avoiding hospitalization and subsequent hospitalization in children with medical complexity (CMC); and feasibility/acceptability of a texting platform, Assessing Confidence at Times of Increased Vulnerability (ACTIV), to collect repeated measures of parent confidence.This prospective cohort study purposively sampled parent-child dyads (n = 75) in 1 of 3 complex care programs for demographic diversity to pilot test ACTIV for 3 months. At random days/times every 2 weeks, parents received text messages asking them to rate confidence in their child avoiding hospitalization in the next month, from 1 (not confident) to 10 (fully confident). Unadjusted and adjusted generalized estimating equations with repeated measures evaluated associations between confidence and hospitalization in the next 14 days. Post-study questionnaires and focus groups assessed ACTIV's feasibility/acceptability.Parents were 77.3% mothers and 20% Spanish-speaking. Texting response rate was 95.6%. Eighteen hospitalizations occurred within 14 days after texting, median (IQR) 8 (2-10) days. When confidence was5 vs ≥5, adjusted odds (95% CI) of hospitalization within 2 weeks were 4.02 (1.20-13.51) times greater. Almost all (96.8%) reported no burden texting, one-third desired more frequent texts, and 93.7% were very likely to continue texting. Focus groups explored the meaning of responses and suggested ACTIV improvements.In this demographically diverse multicenter pilot, low parent confidence predicted impending CMC hospitalization. Text messaging was feasible and acceptable. Future work will test efficacy of real-time interventions triggered by parent-reported low confidence.

BACKGROUND AND OBJECTIVES: Defining and measuring health for children with medical complexity (CMC) is poorly understood. We engaged a diverse national sample of stakeholder experts to generate and then synthesize a comprehensive list of health outcomes for CMC. METHODS: With national snowball sampling of CMC caregiver, advocate, provider, researcher, and policy or health systems experts, we identified 182 invitees for group concept mapping (GCM), a rigorous mixed-methods approach. Respondents (n = 125) first completed Internet-based idea generation by providing unlimited short, free-text responses to the focus prompt, “A healthy life for a child or youth with medical complexity includes: ___.” The resulting 707 statements were reduced to 77 unique ideas. Participants sorted the ideas into clusters based on conceptual similarity and rated items on perceived importance and measurement feasibility. Responses were analyzed and mapped via GCM software. RESULTS: The cluster map best fitting the data had 10 outcome domains: (1) basic needs, (2) inclusive education, (3) child social integration, (4) current child health-related quality of life, (5) long-term child and family self-sufficiency, (6) family social integration, (7) community system supports, (8) health care system supports, (9) a high-quality patient-centered medical home, and (10) family-centered care. Seventeen outcomes representing 8 of the 10 domains were rated as both important and feasible to measure (“go zone”). CONCLUSIONS: GCM identified a rich set of CMC outcome domains. Go-zone items provide an opportunity to test and implement measures that align with a broad view of health for CMC and potentially all children.

OBJECTIVES: We sought to examine the effect of a caregiver coaching intervention, Plans for Action and Care Transitions (PACT), on hospital use among children with medical complexity (CMC) within a complex care medical home at an urban tertiary medical center. METHODS: PACT was an 18-month caregiver coaching intervention designed to influence key drivers of hospitalizations: (1) recognizing critical symptoms and conducting crisis plans and (2) supporting comprehensive hospital transitions. Usual care was within a complex care medical home. Primary outcomes included hospitalizations and 30-day readmissions. Secondary outcomes included total charges and mortality. Intervention effects were examined with bivariate and multivariate analyses. RESULTS: From December 2014 to September 2016, 147 English- and Spanish-speaking CMC CONCLUSIONS: Among CMC within a complex care program, a health coaching intervention designed to identify, prevent, and manage patient-specific crises and postdischarge transitions appears to lower hospitalizations and charges. Future research should confirm findings in broader populations and care models.

Infants with univentricular congenital heart disease (CHD), including those with hypoplastic left heart syndrome (HLHS), regularly pose dilemmas in decision-making because their anatomy and physiology are often unique and variable. The typical staged surgical course for infants with complex univentricular anatomy with systemic outflow obstruction begins with the Norwood (stage 1) operation or variant shortly after birth, followed several months later by superior cavopulmonary anastomosis (stage 2 palliation) with an ultimate goal of a Fontan-type operation several years later.1–3 Improvement in surgical and postoperative management has led to considerable improvement in early post-Norwood survival in the recent era.4–7 However, after the Norwood procedure and before stage 2 palliation, a high-risk time period termed interstage, mortality has been previously been reported at 10% to 15%.8–10 The rare nature of this disorder has limited robust learning about successful strategies to improve survival undertaken by single-surgical centers, and a gap exists in our ability to further improve mortality in this population. The National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC), the first multicenter learning network within pediatric cardiology,11 was established with the goal of improving care and outcomes for children with univentricular heart after the Norwood operation and specifically to (1) improve interstage mortality, (2) decrease interstage growth failure, and (3) reduce interstage hospital readmissions for major medical events. There were several perceived challenges to success in changing clinical outcomes before starting the NPC-QIC collaborative. A primary challenge in collaboration among multiple sites can be agreement on best practices that should be implemented. This is especially true for rare diseases, such as univentricular heart disease, where evidence-based clinical guidelines are not available to clinicians. As noted above, major variation persists in management practices among individuals and institutions caring for children with HLHS and other …

BACKGROUND AND OBJECTIVES:Children with medical complexity (CMC) account for disproportionately high hospital use, and it is unknown if hospitalizations may be prevented. Our objective was to summarize evidence from (1) studies characterizing potentially preventable hospitalizations in CMC and (2) interventions aiming to reduce such hospitalizations.METHODS:Our data sources include Medline, Cochrane Central Register of Controlled Trials, Web of Science, and Cumulative Index to Nursing and Allied Health Literature databases from their originations, and hand search of article bibliographies. Observational studies (n = 13) characterized potentially preventable hospitalizations, and experimental studies (n = 4) evaluated the efficacy of interventions to reduce them. Data were extracted on patient and family characteristics, medical complexity and preventable hospitalization indicators, hospitalization rates, costs, and days. Results of interventions were summarized by their effect on changes in hospital use.RESULTS:Preventable hospitalizations were measured in 3 ways: ambulatory care sensitive conditions, readmissions, or investigator-defined criteria. Postsurgical patients, those with neurologic disorders, and those with medical devices had higher preventable hospitalization rates, as did those with public insurance and nonwhite race/ethnicity. Passive smoke exposure, nonadherence to medications, and lack of follow-up after discharge were additional risks. Hospitalizations for ambulatory care sensitive conditions were less common in more complex patients. Patients receiving home visits, care coordination, chronic care-management, and continuity across settings had fewer preventable hospitalizations.Conclusions:There were a limited number of published studies. Measures for CMC and preventable hospitalizations were heterogeneous. Risk of bias was moderate due primarily to limited controlled experimental designs. Reductions in hospital use among CMC might be possible. Strategies should target primary drivers of preventable hospitalizations.

Background Growth failure is common in infants with single ventricle. This study evaluated the use of a learning network, the National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC), to spread optimized nutritional practices and improve infant growth. Methods A previously identified Nutritional Bundle was spread among NPC-QIC sites. Primary outcome interstage weight-for-age z-score change (ΔWAZ) between discharge from stage 1 palliation (S1) and stage 2 surgical palliation (S2). Variation among sites in interstage ΔWAZ was evaluated before (Period 1) and after (Period 2) spread of Nutritional Bundle. We performed an analysis of NPC-QIC registry infants presenting for S2 at sites previously shown to have significant variation in interstage patient growth. Results Four hundred seven infants from 15 sites underwent S2 between 2008 and 2013: 158 in Period 1 (December 2008-December 2010) and 249 in Period 2 (December 2010-April 2013). Median age at S2 was 4.9 months (2.6-12.8) with no difference between periods. There was significant variation in interstage ΔWAZ among sites in Period 1 (P = .01) but not in Period 2 (P = .39). More patients had an interstage ΔWAZ Conclusions Spread of optimal nutritional practices led to decreased variation in interstage growth with most improvement observed at sites with the worst baseline growth outcomes.

Among infants with single ventricle congenital heart disease (SVD) requiring Stage I palliation (S1P), the impact of prenatal diagnosis (PD) on outcomes has been variably characterized. We investigated the impact of PD in a large multi-center cohort of survivors of S1P in the National Pediatric Cardiology Quality Improvement Collaborative (NPCQIC) registry. Retrospective analysis of demographic and outcomes data among infants enrolled in the NPCQIC database; eligibility includes SVD requiring S1P and survival to discharge. From 43 contributing surgical centers, 591 infants had data available through time of BDG (519) or interstage death (55). Median gestational age was 39 weeks (31–46), and 66 % had variants of hypoplastic left heart syndrome. PD was made in 445 (75 %), with significant variation by center (p = 0.004). While infants with PD had slightly lower gestational age at birth (p

The objective of this study is to identify predictors of prolonged intensive care unit (ICU) length of stay (LOS) for single ventricle patients following Stage I palliation. We hypothesize that peri-operative factors contribute to prolonged ICU stay among children with hypoplastic left heart syndrome (HLHS) and its variants. In 2008, as a part of the Joint Council on Congenital Heart Disease initiative, the National Pediatric Cardiology-Quality Improvement Collaborative established a data registry for patients with HLHS and its variants undergoing staged palliation. Between July 2008 and August 2011, 33 sites across the United States submitted discharge data essential to this analysis. Data describing the patients, their procedures, and their hospital experience were entered. LOS estimates were generated. Prolonged LOS in the ICU was defined as stay greater than or equal to 26 days (i.e., 75th percentile). Statistical analyses were carried out to identify pre-operative, operative, and post-operative predictors of prolonged LOS in the ICU. The number of patients with complete discharge data was 303, and these subjects were included in the analysis. Univariate and multivariate analyses were performed. Multivariate analysis revealed that lower number of enrolled participants (e.g., 1-10) per site, the presence of pre-operative acidosis, increased circulatory arrest time, the occurrence of a central line infection, and the development of respiratory insufficiency requiring re-intubation were associated with prolonged LOS in the ICU. Prolonged LOS in the ICU following Stage I palliation in patients with HLHS and HLHS variant anatomy is associated with site enrollment, circulatory arrest time, pre-operative acidosis, and some post-operative complications, including central line infection and re-intubation. Further study of these associations may reveal strategies for reducing LOS in the ICU following the Norwood and Norwood-variant surgeries.

Objective To test the hypothesis that missing primary care follow-up plans in the discharge summary is associated with higher 30-day readmissions. Study design This retrospective cohort study included pediatric patients discharged from Mattel Children's Hospital, University of California, Los Angeles between July 2008 and July 2010. Exclusions included deaths, transfers, neonatal discharges, stays under 24 hours, and patients over 18 years of age. Bivariate and propensity weighted multivariate logistic regressions tested relationships between 30-day readmission and patient demographics, illness severity, and documentation of primary care provider (PCP) follow-up plans at discharge. Results There were 7794 index discharges (representing 5056 unique patients), with 1457 readmissions within 30 days (18.7%). Average length of stay was 6.3 days. Being 15-18 years old, (OR 1.42 [1.02-1.96]), having public insurance (OR 1.48 [1.20-1.83]), or having higher All-Patient Refined Diagnosis-Related Group severity scores (for severity=4 vs 1, OR 6.88 [4.99-9.49]) was associated with increased odds of 30-day readmission. After adjusting for insurance status, Asian (OR 1.46 [1.01-2.12]) but not Black or Hispanic, race/ethnicity was associated with greater odds of readmission. Fifteen percent of 172 medical records from a randomly selected month in 2010 documented PCP follow-up plans. After adjusting for demographics, length of stay and severity, documenting PCP follow-up plans was associated with significantly increased odds of 30-day readmission (OR 4.52 [1.01-20.31]). Conclusion Readmission rates are complex quality measures, and documenting primary care follow-up may be associated with higher rather than lower 30-day readmissions. Additional studies are needed to understand the inpatient-outpatient transition.

BACKGROUND Improvement in hospital transitional care has become a major national priority, although the impact on children's postdischarge outcomes is unclear. OBJECTIVE To characterize common handoff practices between hospital and primary care providers (PCPs), and test the hypothesis that common handoff practices would be associated with fewer unplanned readmissions. DESIGN, SETTING, AND PATIENTS This prospective cohort study enrolled randomly selected pediatric patients during an acute hospitalization at a tertiary children's hospital in 2012-2014. MEASUREMENTS Primary care and patient data were abstracted from administrative, caregiver, and PCP questionnaires on admission through 30 days postdischarge. The primary outcome was 30-day unplanned readmission to any hospital. Logistic regression assessed relationships between readmissions and 11 handoff communication practices. RESULTS We enrolled 701 children, from which 685 identified PCPs. Complete data were collected from 84% of PCPs. Communication practices varied widely--verbal handoffs occurred rarely (10.7%); PCP notification of admission occurred for 50.8%. Caregiver experience scores, using an adapted Care Transitions Measure-3, were high but were unrelated to readmissions. Thirty-day unplanned readmissions to any hospital were unrelated to most handoff practices. Having PCP follow-up appointments scheduled prior to discharge was associated with more readmissions (adjusted odds ratio, 2.20; 95% confidence interval, 1.08-4.46). CONCLUSION Despite their presumed value, common handoff practices between hospital providers and PCPs may not lead to reductions in postdischarge utilization for children. Addressing broader constructs like caregiver self-efficacy or social determinants is likely necessary. Journal of Hospital Medicine 2017;12:29-35.

The medical home has been widely promoted as a model of primary care with the potential to transform the health care delivery system. Although this model was initially focused on children with chronic conditions, the American Academy of Pediatrics has endorsed a generalization of the model, promoting the statement, "Every child deserves a medical home." Recently, other major professional and governmental organizations have embraced this more inclusive vision, and the medical home concept has been promoted in provisions of the Affordable Care Act. Yet, rigorous evaluations of the value of the medical home, within pediatrics and beyond, have been limited, and the results have been mixed. Early results from large demonstration projects in adults have generally noted modest improvements in quality without accompanying reductions in cost. At this critical period in health care, with widespread interest in health care delivery and payment reform, these results present a potential threat to the medical home. Understanding possible reasons for these early findings is crucial to sustaining the spread of the medical home beyond its first 50 years. With this aim, we review the history of the medical home and trends in child health, and we explore the concepts of value and complexity as they pertain to pediatric health care delivery. We propose that, because of the demographic characteristics and economics of child health and current policy imperatives with regard to health care, a strong value proposition for the medical home in pediatrics involves children with medical complexity.

Objective Interventions to reduce disproportionate hospital use among children with medical complexity (CMC) are needed. We conducted a rigorous, structured process to develop intervention strategies aiming to reduce hospitalizations within a complex care program population. Methods A complex care medical home program used 1) semistructured interviews of caregivers of CMC experiencing acute, unscheduled hospitalizations and 2) literature review on preventing hospitalizations among CMC to develop key drivers for lowering hospital utilization and link them with intervention strategies. Using an adapted version of the RAND/UCLA Appropriateness Method, an expert panel rated each model for effectiveness at impacting each key driver and ultimately reducing hospitalizations. The complex care program applied these findings to select a final set of feasible intervention strategies for implementation. Results Intervention strategies focused on expanding access to familiar providers, enhancing general or technical caregiver knowledge and skill, creating specific and proactive crisis or contingency plans, and improving transitions between hospital and home. Activities aimed to facilitate family-centered, flexible implementation and consideration of all of the child's environments, including school and while traveling. Tailored activities and special attention to the highest utilizing subset of CMC were also critical for these interventions. Conclusions A set of intervention strategies to reduce hospitalizations among CMC, informed by key drivers, can be created through a structured, reproducible process. Both this process and the results may be relevant to clinical programs and researchers aiming to reduce hospital utilization through the medical home for CMC.

The Pediatric Medical Home Program at UCLA enrolled 41 patients in a primary care model focused on providing intensive care coordination for medically complex, ethnically diverse children with special health care needs (CSHCN) in our Pediatric Resident Continuity clinic. We sought to determine the effect of our program on parental satisfaction, and to compare differences in parental satisfaction between English and Spanish speaking patients. The Medical Home Family Index, developed by the Center for Medical Home Improvement, was administered to a total of 22 participating parents, in the family's primary language by a native speaker, at various times after enrollment in the program. Survey data and language effects were analyzed. The 36 standardized mean scores for the 15 Spanish speaking families were significantly higher (8.5 points higher) than the mean scores from the seven English speaking families (p = 0.003). Although no statistically significant differences were noted in individual questions between Spanish and English speakers, a trend towards more positive responses by Spanish speakers was noted in questions regarding physician-patient communication (p = 0.054) and family-centeredness (p = 0.053). Our results suggest that a primary care model focused on providing intensive care coordination produces positive parental perceptions of the organization and delivery of primary care services in a medically complex population of CSHCN. The main finding of the study is that utilizing the AAP's approach to the medical home model, emphasizing family-centered and culturally competent care, can produce higher satisfaction scores in Spanish speaking parents when compared to English speaking parents.

The National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC) was established by the Joint Council on Congenital Heart Disease to dramatically improve the outcomes of care for children with congenital heart disease (CHD) through a national collaborative network of multidisciplinary clinical teams and families, working together to collect longitudinal data, use improvement science methods and conduct research intended to accelerate the development and translation of new knowledge into practice. The initial project selected for this learning network is focused on care processes and outcomes of the initial interstage period for infants with hypoplastic left heart syndrome. A practice-based registry is being used to understand variation in care and outcomes of infants and children with complex CHD. The NPC-QIC has effectively recruited and engaged a large number of U.S. centers caring for infants with complex CHD and provides the infrastructure needed to support the implementation of practice changes across the collaborative that will ultimately improve outcomes in this high-risk group of patients. We describe here the development and early years of NPC-QIC as well as the challenges this collaborative faces moving forward.

Background and Methods. As the first multicenter quality improvement collaborative in pediatric cardiology, the Joint Council on Congenital Heart Disease National Pediatric Cardiology Quality Improvement Collaborative registry collects information on the clinical care and outcomes of infants discharged home after first-stage palliation of single-ventricle heart disease, the Norwood operation, and variants. We sought to describe the preoperative and intraoperative characteristics of the first 100 patients enrolled in the National Pediatric Cardiology Quality Improvement Collaborative registry. Results. From 21 contributing centers, 59% of infants were male, with median birth weight of 3.1 kg (1.9–5.0 kg); the majority had hypoplastic left heart syndrome (71%). A prenatal diagnosis of congenital heart disease was made in 75%; only one had fetal cardiac intervention. Chromosomal anomalies were present in 8%, and major noncardiac organ system anomalies were present in 9%. Preoperative risk factors were common (55%) but less frequent in those with prenatal cardiac diagnosis (P= .001). Four patients underwent a preoperative transcatheter intervention. Substantial variation across participating sites was demonstrated for choice of initial palliation for the 93 patients requiring a full first-stage approach, with 50% of sites performing stage I with right ventricle to pulmonary artery conduit as the preferred operation; 89% of hybrid procedures were performed at a single center. Significant intraoperative variation by site was noted for the 83 patients who underwent traditional surgical stage I palliation, particularly with use of regional perfusion and depth of hypothermia. Conclusions. In summary, there is substantial variation across surgical centers in the successful initial palliation of infants with single-ventricle heart disease, particularly with regard to choice of palliation strategy, and intraoperative techniques including use of regional perfusion and depth of hypothermia. Further exploration of the relationship of such variables to subsequent outcomes after hospital discharge may help reduce variability and improve long-term outcomes.

Objective To study the integration of comprehensive care coordination for children with complex disease in our resident education clinic at University of California Los Angeles by analyzing alterations in medical resource use. Study design The Pediatric Medical Home Project at University of California Los Angeles was designed to include 4 basic elements: 1) 60-minute intake appointment; 2) follow-up appointments twice the length of a standard visit; 3) access to a "family liaison"; and 4) a family notebook ("All about Me" binder). From the initial cohort of 43 patients, encounter data on 30 were analyzed to determine use of outpatient, urgent, emergency department (ED), and inpatient services. Encounters for each patient were compared for a period of 1 year before and 1 year after enrollment. Results The average number of ED visits per patient decreased from 1.1 ± 1.7 before enrollment to 0.5 ± 0.9 after medical home enrollment ( P = .02). However, no significant change was found in use of any of the other health care resources studied. Conclusions Incorporating a program of care coordination according to the principles of the medical home into an outpatient pediatric residency teaching clinic may not only serve as a training vehicle for pediatric residents, but also create favorable alterations in medical resource use.

To investigate cardiac physiology at the onset of heart beating in embryonic mouse hearts, we performed optical imaging of membrane potential (Vm) and/or intracellular calcium (Cai). Action potentials and Cai transients were detected in ∼50% of mouse embryo hearts at E8.5, but in all hearts at E9.0, indicating that beating typically starts between E8–E9. Beating was eliminated by Ca channel blocker nifedipine and the If blocker ZD7288, unaffected by tetrodotoxin and only mildly depressed by disabling sarcoplasmic (SR) and endoplasmic (ER) reticulum Ca cycling. From E8.5 to E10, conduction velocity increased from 0.2–1 mm/s to >5 mm/s in first ventricular and then atrial tissue, while remaining slow in other areas. Arrhythmias included atrioventricular reentry induced by adenosine. In summary, at the onset of beating, If-dependent pacemaking drives both AP propagation and Cai transient generation through activation of voltage-dependent Ca channels. Na channels and intracellular Ca cycling have minor roles. Developmental Dynamics 239:1941–1949, 2010. © 2010 Wiley-Liss, Inc.

This review includes a brief discussion, from the perspective of the pediatric cardiologist, of the rationale for creation and maintenance of multi-institutional databases of outcomes of the treatment of patients with congenital and paediatric cardiac disease, together with a history of the evolution of such databases, and a description of the current state of the art. A number of projects designed to have broad-based impact are currently in the design phase, or have already been implemented. Not surprisingly, most of the efforts thus far have focused on catheterization procedures and interventions, although some work examining other aspects of paediatric cardiology practice is also beginning. This review briefly describes several European and North American initiatives related to databases for pediatric and congenital cardiology including the Central Cardiac Audit Database of the United Kingdom, national database initiatives for pediatric cardiology in Switzerland and Germany, various database initiatives under the leadership of the Working Groups of The Association for European Paediatric Cardiology, the IMPACT Registry™ (IMproving Pediatric and Adult Congenital Treatment) of the National Cardiovascular Data Registry® of The American College of Cardiology Foundation® and The Society for Cardiovascular Angiography and Interventions (SCAI), the Mid-Atlantic Group of Interventional Cardiology (MAGIC) Catheterization Outcomes Project, the Congenital Cardiac Catheterization Project on Outcomes (C3PO), the Congenital Cardiovascular Interventional Study Consortium (CCISC), and the Joint Council on Congenital Heart Disease (JCCHD) National Quality Improvement Initiative. These projects, each leveraging multicentre data and collaboration, demonstrate the enormous progress that has occurred over the last several years to improve the quality and consistency of information about nonsurgical treatment for congenital cardiac disease. The paediatric cardiology field is well-poised to move quickly beyond outcome assessment and benchmarking, to collaborative quality improvement.

It is important for pediatric cardiologists to remember arrhythmogenic right ventricular dysplasia (ARVD) in the differential diagnosis of syncope in the pediatric population. Although the prevalence of ARVD is estimated to be relatively low and the true incidence is unknown, there are several characteristic findings that can be obtained by noninvasive modalities such as ECG, echocardiogram, and MRI that can lead to a diagnosis despite a nonspecific clinical presentation. We examined the diagnostic steps and current treatment and preventive options in a child with ARVD to highlight these features for clinicians who often evaluate children with syncope.

Developing myocardium is more dependent on glycolysis than adult myocardium, yet the effects of selectively inhibiting glycolysis versus oxidative phosphorylation on embryonic heart function have not been well characterized. Accordingly, we investigated how selective metabolic inhibition affects membrane voltage and intracellular Ca (Cai) transients in embryonic mouse hearts, including their susceptibility to arrhythmias. A total of 136 isolated embryonic mouse hearts were exposed to either 1) 2-deoxyglucose (2DG; 10 mM) or iodoacetate (IAA; 0.1 mM) with 10 mM pyruvate in place of glucose to selectively inhibit glycolysis or 2) the mitochondrial uncoupler protonophore carbonyl cyanide p-(trifluoromethoxy)phenylhydrazone (FCCP; 500 nM) with 10 mM glucose present to selectively inhibit oxidative phosphorylation. Using confocal imaging, we found that mitochondrial membrane potential monitored with tetramethylrhodamine methyl ester (200 nM) remained stable with 2DG or IAA but depolarized within 5 min after exposure to FCCP. IAA and FCCP decreased heart rate, inhibited Cai transient amplitude, shortened action potential duration at 80% repolarization (APD80), and prolonged atrioventricular conduction time to similar extents. Although 2DG decreased heart rate and Cai transient amplitude, it did not significantly affect APD80 and AV conduction time. In addition, spontaneous arrhythmias occurred in 77 of 136 embryonic hearts (57%) after exposure to IAA (28/53) or FCCP (49/83). There were no significant differences in the types or incidence of arrhythmias induced by IAA and FCCP. These data support the idea that both glycolysis and oxidative phosphorylation play critical metabolic roles in regulating cardiac function in the embryonic mouse heart.

ObjectiveTo identify subgroups of U.S. children with special health care needs (CSHCN) and characterize key outcomes.Data sourceSecondary analysis of 2009-2010 National Survey of CSHCN.Study designLatent class analysis grouped individuals into substantively meaningful classes empirically derived from measures of pediatric medical complexity. Outcomes were compared among latent classes with weighted logistic or negative binomial regression.Principal findingsLCA identified four unique CSHCN subgroups: broad functional impairment (physical, cognitive, and mental health) with extensive health care (Class 1), broad functional impairment alone (Class 2), predominant physical impairment requiring family-delivered care (Class 3), and physical impairment alone (Class 4). CSHCN from Class 1 had the highest ED visit rates (IRR 3.3, p&nbsp

BACKGROUND AND OBJECTIVE: Despite considerable attention, little is known about the degree to which primary care medical homes influence early postdischarge utilization. We sought to test the hypothesis that patients with medical homes are less likely to have early postdischarge hospital or emergency department (ED) encounters. METHODS: This prospective cohort study enrolled randomly selected patients during an acute hospitalization at a children’s hospital during 2012 to 2014. Demographic and clinical data were abstracted from administrative sources and caregiver questionnaires on admission through 30 days postdischarge. Medical home experience was assessed by using Maternal and Child Health Bureau definitions. Primary outcomes were 30-day unplanned readmission and 7-day ED visits to any hospital. Logistic regression explored relationships between outcomes and medical home experiences. RESULTS: We followed 701 patients, 97% with complete data. Thirty-day unplanned readmission and 7-day ED revisit rates were 12.4% and 5.6%, respectively. More than 65% did not have a medical home. In adjusted models, those with medical home component “having a usual source of sick and well care” had fewer readmissions than those without (adjusted odds ratio 0.54, 95% confidence interval 0.30–0.96). Readmissions were higher among those with less parent confidence in avoiding a readmission, subspecialist primary care providers, longer length of index stay, and more hospitalizations in the past year. ED visits were associated with lack of parent confidence but not medical home components. CONCLUSIONS: Lacking a usual source for care was associated with readmissions. Lack of parent confidence was associated with readmissions and ED visits. This information may be used to target interventions or identify high-risk patients before discharge.

Objective Children with medical complexity (CMC) are a small group that utilizes large amounts of health care resources. Although parents are the primary healthcare decision-makers for their children, little is known from their perspective about why CMC are hospitalized. We sought to understand what parents think about factors leading to hospitalization and whether any recent hospitalizations might have been avoidable. Methods We conducted qualitative, semistructured interviews with 35 parents of hospitalized CMC who receive care in the Pediatric Medical Home Program, a complex care program at University of California, Los Angeles. Interviews were conducted in English and in Spanish, audio-recorded, transcribed and translated, then coded in ATLAS.ti (Scientific Software Development Gmbh, Berlin, Germany) for qualitative analysis. We sorted qualitative codes into groups with shared concepts, to generate emergent themes. Results Parents described their experiences leading up to their children's hospitalization, but no one suggested that the hospitalization was potentially avoidable. Most parents perceived their children as having higher susceptibility because of underlying conditions, perceived the symptoms they observed as high-risk, and described seeking emergent care only when they no longer were comfortable at home. Decisions about where to seek care were influenced by health care system factors such as accessibility and continuity of care. Most parents expressed a desire to learn more about their children's conditions and how best to care for them at home. Conclusions Parents of CMC believe that hospitalizations are largely unavoidable because of higher susceptibility and higher risk. Increasing parents' self-efficacy in caring for children at home might influence their decisions to seek emergent care.

Background— During development, AV conduction switches from base-to-apex to apex-to-base conduction after emergence of the conduction system. We hypothesize that after this transition, the bulk of the AV ring, although no longer required for AV conduction, remains transiently able to conduct, providing a potential arrhythmia substrate. We studied AV conduction during this transition and its sensitivity to autonomic modulation. Methods and Results— Simultaneous voltage and Ca 2+ mapping with RH-237 and Rhod-2 was performed with 2 CCD cameras in embryonic mouse hearts (n=43). Additionally, isolated calcium mapping was performed in 309 hearts with fluo-3AM. Propagation patterns in voltage and Ca 2+ mapping coincided. Arrhythmias were uncommon under basal conditions, with AV block in 14 (4%) and junctional rhythms in 4 (1%). Arrhythmias increased after stimulation with isoproterenol—junctional rhythm in 9 (3%) and ventricular rhythms in 22 (6%)—although AV block decreased (3 hearts, 1%). Adding carbachol after isoproterenol caused dissociated antegrade and retrograde AV ring conduction in 30 (8.6%) of E10.5 and E11.5 hearts, occurring preferentially in the right and left sides of the ring, respectively. In 2 cases, reentry occurred circumferentially around the AV ring, perpendicular to normal propagation. Reentry persisted for multiple beats, lasting from 3 to 22 minutes. No episodes of AV ring reentry occurred in E9.5 hearts. Conclusions— AV ring reentry can occur by spatial dissociation of antegrade and retrograde conduction during combined adrenergic and muscarinic receptor stimulation. Critical maturation (>E9.5) seems to be required to sustain reentry.

Little is known regarding the risk factors for early and late death after hospital discharge among pediatric patients undergoing cardiac surgery.Statewide hospital discharge data from California in 1989 to 1999 were used to study outcomes of children18 years old who had a procedure code (by International Classification of Diseases, Ninth Revision, Clinical Modification) indicating cardiac surgery. The outcome variable was death occurring after hospital discharge. Postdischarge deaths were identified by linking hospital discharge data to statewide death registry data. Cardiac surgical procedures were grouped into 23 categories to adjust for risk involved with the procedures. We used logistic regression to evaluate risk factors for postdischarge mortality, including variables age, sex, race and ethnicity, type of insurance, home income, date and month of surgery, type of admission, hospitals case volume, and the various types of procedures.There were 25,402 cardiac surgery cases with 1505 inhospital deaths. Of 23,897 hospital discharges, 148 deaths (0.62%) occurred within 365 days after discharge, including 37 deaths within 30 days; 44 deaths at 31 to 90 days; and 67 deaths at 91 to 365 days. Logistic regression showed young age was an important risk factor for postdischarge death with an odds ratio of 4.8 for neonates and 3.5 for infants, compared with children1 year old. Another significant risk factor was the type of procedure. For death30 days after discharge, Norwood operation (odds ratio 8.4 compared with closure of ventricular septal defect) was a risk factor. For death that occurred between 31 and 365 days, significant risk factors were truncus arteriosus repair, total anomalous pulmonary vein repair, aortopulmonary shunt, and open valvotomy. Sex, race/ethnicity, home income, and hospital case volume were not significant predictors of postdischarge deaths.Many demographic and socioeconomic variables affecting inhospital death were not significant predictors for postdischarge death. Important risk factors for postdischarge death were young age and the type of surgery performed.

We successfully implanted 11 pacemakers, 6 defibrillators, and 1 biventricular pacemaker in 18 pediatric patients (15 female; 4 to 15 years, average age: 9) using the retropectoral transvenous approach with a hidden axillary incision. The average follow-up period was 24 months (range 49 months). Eight patients had congenital structural heart conditions (d-transposition of great arteries S/P Mustard operation, d-transposition of great arteries S/P arterial switch operation, truncus arteriosus, right ventricular diverticula, ventricular septal defect, hypertrophic cardiomyopathy). Four patients had acquired heart conditions (dilated cardiomyopathy, myocarditis). Excellent sensing and pacing thresholds were achieved in all attempted implantations. There was no pneumothorax. There was one lead dislodgement. One lead fracture distant from the subclavian vein occurred 4 months after implantation. Implantation of pacemakers and defibrillators via axillary incisions can be safe and effective in pediatric patients. This approach avoids skin erosion when implanting large devices such as defibrillators or biventricular devices in small patients with limited muscle mass while achieving superior aesthetic results. The axillary or extrathoracic venous entry site avoids subclavian crush syndrome.

Many non-medical variables such as patient demographics and socioeconomic status have been shown to affect cardiovascular outcomes. Patient characteristics such as age, gender, race and ethnicity are frequently considered risk factors for cardiac surgery. Type of insurance, income and level of education may also have significant impact on service utilization and health outcomes of children with heart disease. Furthermore, many healthcare system characteristics, such as hospital case volume and surgeon's case volume, have been shown to affect patient outcomes. In this article, we review the effect of these non-medical variables on outcomes of children undergoing cardiac surgery, and discuss issues related to improving patient outcomes by recognizing patients at higher risk.

Theoretically, regionalization has the effect of concentrating specialized medical services into ‘Centers of Excellence’ thereby enhancing volumes to improve outcomes via the volume–outcome relation. Several examples of the volume–outcome relation have been demonstrated in the field of heart surgery for congenital heart disease in the pediatric population. However, translating this theoretical construct into practice by promoting or achieving regionalization of pediatric cardiac surgical services can prove to be a challenging undertaking. In this article we review four strategies for achieving regionalization: national mandates, state standards, expert panels and market-based strategies. The feasibility of applying each strategy to pediatric cardiac surgery is discussed. When possible, specific examples of the use of each strategy have been given.

Care for children with congenital heart disease requires specialized services and various healthcare resources. The purpose of this article is to provide an updated overview of healthcare resources for infants and children with heart disease. In 2001, there were 1609 certified pediatric cardiologists in the United States. The ratio was approximately 45,000 children younger than 18 years per pediatric cardiologist. It is estimated that more than 19,000 cardiac surgeries are performed in children younger than 18 years in the United States each year. This article also reviews the effect of patient characteristics on access to healthcare and use of pediatric cardiac services, and discusses issues related to optimal use of these resources and the development of an organized approach toward service management by regionalization. The authors believe that improved access to high-quality facilities and providers coupled with thoughtful changes in the healthcare delivery system represent an excellent opportunity for optimizing outcomes for children with heart disease.

Background— The purpose of this study was to investigate whether sex disparity in cardiovascular outcomes exists in children who undergo cardiac surgery. Methods and Results— Statewide hospital discharge data from California from 1995 to 1997 were used. Children P P Conclusions— For children undergoing cardiac surgery, female sex was associated with 51% higher odds of death than male sex. The mechanism by which female sex acts as a risk factor requires further investigation.

Objective. The association between high case volumes and better patient outcomes has been demonstrated for many surgical procedures and medical treatments, including surgery for children with congenital heart disease. To simulate the effects of regionalization of pediatric cardiac surgery, we assessed the impact of reducing the number of pediatric cardiac centers on surgical mortality and patient’s travel distance. Methods. This study used abstracted statewide hospital discharge data from California from 1995 to 1997. Case volume and in-hospital mortality for pediatric cardiac surgeries at each hospital were calculated. All hospitals that performed ≥10 pediatric cardiac surgeries in 1995 to 1997 were included in the analysis. To simulate regionalization, we “closed” the hospital with the lowest case volume and redistributed patients from this hospital to the nearest remaining hospitals. The number of in-hospital deaths was then recalculated using the original mortality rate of each remaining hospital multiplied by its new case volume. A multivariate logistic regression was conducted to determine the odds ratios of mortality of various types of surgery compared with closure of ventricular septal defect. This result was used for adjusting for the case-mix of the hospitals. Regionalization simulation analysis was repeated, and the number of deaths was recalculated using this adjustment of hospital case-mix. We also examined the increase in travel distance of patients to the hospitals as a result of the regionalization simulation. Results. In California, 6592 children underwent cardiac surgeries in 1995 to 1997 with 352 in-hospital deaths (overall mortality rate: 5.34%). A quadratic regression model demonstrated that a high surgical volume was associated with a low mortality rate. We found demarcations between low- and medium-volume hospitals at 70 cases per year and medium- and high-volume hospitals at 170 cases per year. With adjustment for hospital case-mix, we found that 41 deaths could be avoided when all patients from low-volume hospitals were referred, and 83 deaths could be avoided when all patients from low- and medium-volume hospitals were referred to high-volume hospitals (overall mortality rate decreased to 4.08%). The average travel distance for pediatric cardiac surgery was 45.4 miles, which increased by 12.7 miles when all surgeries were referred to high-volume hospitals. When only the 733 high-risk patients were referred from low- and medium-volume hospitals to high-volume hospitals, 49 deaths could be avoided, yielding an overall mortality rate of 4.60%. Conclusions. Theoretical regionalization of pediatric cardiac surgery is associated with a reduction in surgical mortality from 5.34% to 4.08% when all cases were referred to high-volume hospitals, or decrease to 4.60% when high-risk cases were referred. Although regionalization is associated with an important decrease in the number of deaths, it also increases the travel distance for patients. Additional studies on the costs and benefits of regionalization are needed to determine the best strategies to improve outcomes for children who undergo cardiac surgery.

Growth failure is common in infants with single ventricle. This study evaluated the use of a learning network, the National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC), to spread optimized nutritional practices and improve infant growth.A previously identified Nutritional Bundle was spread among NPC-QIC sites.interstage weight-for-age z-score change (ΔWAZ) between discharge from stage 1 palliation (S1) and stage 2 surgical palliation (S2). Variation among sites in interstage ΔWAZ was evaluated before (Period 1) and after (Period 2) spread of Nutritional Bundle. We performed an analysis of NPC-QIC registry infants presenting for S2 at sites previously shown to have significant variation in interstage patient growth.Four hundred seven infants from 15 sites underwent S2 between 2008 and 2013: 158 in Period 1 (December 2008-December 2010) and 249 in Period 2 (December 2010-April 2013). Median age at S2 was 4.9 months (2.6-12.8) with no difference between periods. There was significant variation in interstage ΔWAZ among sites in Period 1 (P = .01) but not in Period 2 (P = .39). More patients had an interstage ΔWAZ0 in Period 1 (43%) than Period 2 (32%) (P = .03). In Period 1, the median interstage ΔWAZ was0 in six sites while in Period 2 no site had median interstage ΔWAZ0. Sites with the worst patient growth in Period 1 had marked improvement in Period 2 (P = .02, .06, and .06, respectively).Spread of optimal nutritional practices led to decreased variation in interstage growth with most improvement observed at sites with the worst baseline growth outcomes.

Understanding a care coordination framework, its functions, and its effects on children and families is critical for patients and families themselves, as well as for pediatricians, pediatric medical subspecialists/surgical specialists, and anyone providing services to children and families. Care coordination is an essential element of a transformed American health care delivery system that emphasizes optimal quality and cost outcomes, addresses family-centered care, and calls for partnership across various settings and communities. High-quality, cost-effective health care requires that the delivery system include elements for the provision of services supporting the coordination of care across settings and professionals. This requirement of supporting coordination of care is generally true for health systems providing care for all children and youth but especially for those with special health care needs. At the foundation of an efficient and effective system of care delivery is the patient-/family-centered medical home. From its inception, the medical home has had care coordination as a core element. In general, optimal outcomes for children and youth, especially those with special health care needs, require interfacing among multiple care systems and individuals, including the following: medical, social, and behavioral professionals; the educational system; payers; medical equipment providers; home care agencies; advocacy groups; needed supportive therapies/services; and families. Coordination of care across settings permits an integration of services that is centered on the comprehensive needs of the patient and family, leading to decreased health care costs, reduction in fragmented care, and improvement in the patient/family experience of care.

Background Although amiodarone has been referred to as a class III antiarrhythmic agent, it also possesses electrophysiologic characteristics of the three other classes (classes I and IV and minor class II effects). Previous studies have demonstrated that amiodarone inhibits Ca2+ channel current in intact cardiac myocytes. However, it is not clear whether this response reflects a pure class IV effect (direct Ca2+ channel inhibition) or a class II effect (β-adrenergic receptor blockade) of amiodarone. Methods In the current study, the effects of amiodarone on Ca2+ current were studied in the absence of sympathetic regulation using a Xenopus oocyte expression system. The L-type Ca2+ channel α1C subunit was coexpressed with the α2Δ and β2a subunits in enzymatically digested Xenopus oocytes. Ca2+ currents were recorded using the cut-open oocyte preparation. Results We found that perfusion of 10 μM isoproterenol produced no significant change in peak Ca2+ current (from 223±33 to 210±29 nA, mean±SEM, n=5, P=not significant), indicating the absence of a functional stimulatory sympathetic signal pathway in these oocytes. After 10 minutes of exposure to 10 μM amiodarone, Ca2+ current amplitude was significantly decreased from 174±33 to 100±26 nA (n=8, P2+ channel blocker. On the other hand, neither amiodarone nor nifedipine significantly altered the Ca2+ current activation or inactivation kinetics. Conclusions These results demonstrate that amiodarone inhibits Ca2+ current in the absence of a functional intrinsic β-adrenergic stimulatory system and, therefore, represents a true class IV effect.

Ventricular tachyarrhythmia is less frequent than supraventricular tachycardia in pediatric patients. Symptoms of ventricular tachycardia in children range widely from feeding problems to syncope and sudden death. Electrocardiographic features include wide QRS complex tachycardia and, often, atrioventricular association. Direct current cardioversion is the first-line therapy for patients who are hemodynamically unstable. Intravenous amiodarone or lidocaine is used in patients who are stable or in patients after successful cardioversion. Long-term medical management is needed in many children and radiofrequency ablation should be considered in symptomatic or life-threatening cases.

Objective. Previous studies have shown that children with congenital heart disease (CHD) who live in nonurban areas or who do not have private insurance are at risk for delayed referral to a pediatric cardiologist. However, the effect of these factors on the age at which cardiac surgery is performed has not been evaluated. This study is designed to evaluate the factors that influence the age at which definitive surgical repair is performed. Methods. Data on hospital discharges for 1995 and 1996 in California were obtained from the Office of Statewide Health Planning and Development database. Children Results. In 1995–1996, 666 children underwent ASD closure (mean age: 5.1 years; median: 4.0 years), 582 VSD closure (mean age: 2.8; median: 1.1 years), 394 TOF repair (mean age: 1.7; median: .9 years), and 177 AVC repair (mean age: 1.1; median: .6 years). Comparing median and mean age at surgery, we found: AVC Conclusions. Many medical and nonmedical variables play important roles in determining age for definitive repair of CHD in children. Type of insurance, a recognized surrogate for access to care, may play an important role. In addition, centers with higher surgical case volume were more likely to operate at a younger age. Finally, children in urban areas tend to be older at the time of surgery for ASD, VSD, and TOF.

Abstract —Cardiomyopathy associated with HIV-1 infection is a well-recognized complication. However, it is unknown whether direct cardiomyocyte infection is involved in the pathogenesis of the cardiomyopathy. An HIV-1–based lentiviral vector and wild-type HIV-1 were used to infect human fetal cardiac myocytes in a primary culture. Quantitative polymerase chain reaction, viral p24 antigen determination, and immunofluorescence were used to detect the synthesis of HIV-1 DNA and proteins after the infection. High-efficiency infection occurred using the HIV-1–based lentiviral vector, although no infection occurred with the wild-type HIV-1 strain. Dual-labeling immunofluorescence for HIV-1 proteins and myosin confirmed that cardiomyocytes were infected. This in vitro analysis suggests that direct myocyte infection with wild-type HIV-1 may not be involved in the pathogenesis of HIV-1 cardiomyopathy. However, HIV-1–based vectors may prove useful for ex vivo cardiovascular gene therapy.

To determine whether precise left-sided accessory pathway localization is possible from the coronary sinus, electrocardiogram (ECG) characteristics from the coronary sinus pair demonstrating earliest activation via the accessory pathway were compared to simultaneous mitral annular ablation catheter ECGs at successful ablation sites in 48 patients. To define the coronary sinus-mitral annular relation, the coronary sinus to mitral annulus distance (D) was measured at sequential distances from the coronary sinus os in 10 cadaver hearts. Mitral annular ECGs demonstrated earliest activation via the accessory pathway more frequently than the earliest coronary sinus pair (p0.001), more frequent continuous electrical activity (p0.001), and more frequent accessory pathway potentials (p0.01). D was10 mm at 20, 40, and 60 mm, respectively, from the coronary sinus os. Coronary sinus ECGs do not precisely localize left-sided accessory pathways, which may be due in part to an average anatomic separation of more than 10 mm between the coronary sinus and accessory pathways bridging the mitral annulus.

This overview of cardiac ion channel development does not suggest any particular theme underlying the expression or regulation of all channel subtypes. Calcium and potassium channels generally exhibit increased expression in more mature hearts. However, this increase in channel number or activity as determined under voltage clamp conditions may not be translated into increased activity in vivo. Concomitant changes in other physiological factors such as local intracellular Ca2+ accumulation, increased resting membrane potential and decreased heart rate in mature heart may inhibit or augment channel activity. Na(+)-Ca2+ exchange activity appears to decrease with development, possibly reflecting its decreasing role in both systolic and diastolic Ca2+ regulation. Na+ channel activity follows a middle course, exhibiting little change in channel conductance. The reported shift in the voltage dependence of channel inactivation toward more negative membrane potentials may reflect a concomitant shift in the resting membrane potential in mature heart. However, this change is in the direction opposite to that reported for L-type Ca2+ channel inactivation, suggesting that the regulation of these channels is not modulated by a common factor such as membrane surface charge. A detailed characterization of multiple channel subtypes in mature myocardium has resulted in significant advances in models of the cardiac action potential and excitation-contraction coupling. Recently, developmental changes in ion channel physiology have been described, setting the stage for a comparable elucidation of the ontogeny of the cardiac action potential. Ca2+ and K+ channel currents generally become more prominent with development. In contrast, developmental changes in Na+ currents are less dramatic and Na(+)-Ca2+ exchange currents appear to decrease with age. These changes may, in part, be reflected by the increasingly important role of transsarcolemmal Ca2+ influx in triggering Ca2+ release from the SR in mature heart as compared to its direct role of providing Ca2+ for cell contraction in immature heart. These developmental changes in ion channel expression and function are likely to have a significant effect on the generation of the action potential in individual myocytes. Developmental changes in the characteristics of the action potential may then have a major influence on the initiation, propagation and termination of autonomic, triggered, and re-entrant arrhythmias. Progress in this area provides an essential foundation for the evolution of a systematic approach to pediatric arrhythmias comparable to that under development for mature heart [3].

Patients with a systemic-to-pulmonary artery shunt and positive findings on traditional imaging modalities such as chest X-ray, echocardiography, or cardiac angiography often can benefit from additional noninvasive imaging with magnetic resonance imaging (MRI). Diagnostic dilemmas encountered include: pseudoaneurysms, contained fluid collection (seroma) surrounding a shunt, and stenosis of the shunt anastomoses. MRI studies using traditional cardiac-triggered spin-echo (SE) imaging and the newer breathhold MRI studies with k-space segmented gradient-recalled echo (GRE) imaging can greatly help resolve diagnostic dilemmas. By combining different MR imaging techniques it becomes possible to clearly distinguish between pseudoaneurysms and seroma, to exclude an active leak and to sometimes visualize the distal anastomosis with more precision than conventional angiography. MRI is often able to add information needed for clinical decision making prior to surgical repair.

One of the most notable trends in child health has been the increase in the number of children with special health care needs, including those with complex chronic conditions. Care of these children accounts for a growing fraction of health care resources. We examine recent developments in health care, especially with regard to medical transport and prehospital care, that have emerged to adapt to this remarkable demographic trend. One such development is the focus on care coordination, including the dissemination of the patient-centered medical home concept. In the prehospital setting, the need for greater coordination has catalyzed the development of the emergency information form. Training programs for prehospital providers now incorporate specific modules for children with complex conditions. Another notable trend is the shift to a family-centered model of care. We explore efforts toward regionalization of care, including the development of specialized pediatric transport teams, and conclude with recommendations for a research agenda.

Optimal health care is achieved when each person, at every age, receives medically and developmentally appropriate care. The goal of a planned health care transition is to maximize lifelong functioning and well-being for all youth, including those who have special health care needs and those who do not. This process includes ensuring that high-quality, developmentally appropriate health care services are available in an uninterrupted manner as the person moves from adolescence to adulthood. A well-timed transition from child- to adult-oriented health care is specific to each person and ideally occurs between the ages of 18 and 21 years. Coordination of patient, family, and provider responsibilities enables youth to optimize their ability to assume adult roles and activities. This clinical report represents expert opinion and consensus on the practice-based implementation of transition for all youth beginning in early adolescence. It provides a structure for training and continuing education to further understanding of the nature of adolescent transition and how best to support it. Primary care physicians, nurse practitioners, and physician assistants, as well as medical subspecialists, are encouraged to adopt these materials and make this process specific to their settings and populations.

The National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC) is the first quality improvement collaborative in pediatric cardiology, and its registry captures information on interstage care and outcomes of infants after the Norwood procedure. The purpose of this study was to evaluate variation in interstage outpatient clinical care practices for infants discharged home after the Norwood procedure.Data for the first 100 infants enrolled in the NPC-QIC registry were evaluated. The care domains assessed for variation included: (1) discharge communication with outpatient cardiologist and primary care physician (PCP); (2) nutrition plan at hospital discharge; and (3) planned use of home surveillance strategies.One hundred infants were discharged home between July 2008 and February 2010, from 21 participating US pediatric cardiac programs. Median age at discharge was 29 (11-188) days. Interstage outpatient care was provided at the Norwood center for 62 infants, at other centers for 25, and at a combination of centers for 13. Complete discharge communication (defined as written communication of medication list, nutrition plan, and red flag checklist) was relayed to only 45 outpatient cardiologists and to 26 PCPs. Nutrition route at discharge was exclusively oral in 49, combined oral and nasogastric (NG)/nasojejunal (NJ) in 38, exclusively NG/NJ in six, combined oral and gastrostomy tube (GT) in six, and exclusively GT in one infant. Home surveillance strategies were utilized for 81 infants (oximetry and weight monitoring in 77, oximetry alone in four), with no home surveillance in 19 infants.Considerable variation exists in interstage outpatient care after the Norwood procedure in the care domains of discharge communication, nutrition, and home surveillance. Standardizing care around evidence-based practices may improve the outcomes for these very high-risk children.

The National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC) has established a national data registry for patients with hypoplastic left heart syndrome and its variants undergoing staged palliation. The goal of this collaborative is to better understand current care practices and to improve outcomes in children with these severe and complex forms of congenital heart disease. In this study, we describe the postoperative intensive care course, and its variations, for the first 100 patients enrolled into the registry.Patients were enrolled from 21 contributing sites and were discharged home after stage I palliation between July 2008 and February 2010. Following stage I palliation, enrolled participants remained in the intensive care unit for a median duration of 11 days (range: 3-68 days). Duration of intensive care unit stay varied and was greatest for those patients with aortic atresia versus aortic hypoplasia (P= 0.04) and for those who underwent a modified Blalock-Taussig shunt as part of their palliation. The duration of intensive care unit stay also varied by contributing site (medians ranged from 8 to 18 days). Participants requiring reoperation had significantly prolonged lengths of stay (P= .0003). Inotropic agent use among univentricular registry participants also varied by site. The majority of recipients received milrinone (87%), dopamine (64%), and epinephrine (62%). Cardiac catheterization following surgery occurred in 20 patients. Fifteen percent of participants underwent an interventional procedure. Complication following stage I palliation was also fairly common.Considerable variation exists in the postoperative course and management of univentricular patients following stage I palliation. Variation in length of intensive care unit stay, inotropic agent use, need for reoperation or cardiac catheterization, and postoperative complications are described. Further studies to determine etiologies for observed variation may result in improved standards of care and better outcomes during the interstage period.