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Objective: Individuals with single ventricle physiology who are palliated with superior cavopulmonary anastomosis (Glenn surgery) may develop pulmonary arteriovenous malformations. The traditional tools for pulmonary arteriovenous malformation diagnosis are often of limited diagnostic utility in this patient population. We sought to measure the pulmonary capillary transit time to determine its value as a tool to identify pulmonary arteriovenous malformations in patients with single ventricle physiology., Methods: We defined the angiographic pulmonary capillary transit time as the number of cardiac cycles required for transit of contrast from the distal pulmonary arteries to the pulmonary veins. Patients were retrospectively recruited from a single quaternary North American paediatric centre, and angiographic and clinical data were reviewed. Pulmonary capillary transit time was calculated in 20 control patients and compared to 20 single ventricle patients at the pre-Glenn, Glenn, and Fontan surgical stages (which were compared with a linear-mixed model). Correlation (Pearson) between pulmonary capillary transit time and haemodynamic and injection parameters was assessed using angiograms from 84 Glenn patients. Five independent observers calculated pulmonary capillary transit time to measure reproducibility (intraclass correlation coefficient)., Results: Mean pulmonary capillary transit time was 3.3 cardiac cycles in the control population, and 3.5, 2.4, and 3.5 in the pre-Glenn, Glenn, and Fontan stages, respectively. Pulmonary capillary transit time in the Glenn population did not correlate with injection conditions. Intraclass correlation coefficient was 0.87., Conclusions: Pulmonary angiography can be used to calculate the pulmonary capillary transit time, which is reproducible between observers. Pulmonary capillary transit time accelerates in the Glenn stage, correlating with absence of direct hepatopulmonary venous flow.

Family-centered rounding has emerged as the gold standard for inpatient paediatrics rounds due to its association with improved family and staff satisfaction and reduction of harmful errors. Little is known about family-centered rounding in subspecialty paediatric settings, including paediatric acute care cardiology.In this qualitative, single centre study, we conducted semi-structured interviews with providers and caregivers eliciting their attitudes toward family-centered rounding. An a priori recruitment approach was used to optimise diversity in reflected opinions. A brief demographic survey was completed by participants. We completed thematic analysis of transcribed interviews using grounded theory.In total, 38 interviews representing the views of 48 individuals (11 providers, 37 caregivers) were completed. Three themes emerged: rounds as a moment of mutual accountability, caregivers' empathy for providers, and providers' objections to family-centered rounding. Providers' objections were further categorised into themes of assumptions about caregivers, caregiver choices during rounds, and risk for exacerbation of bias and inequity.Caregivers and providers in the paediatric acute care cardiology setting echoed some previously described attitudes toward family-centered rounding. Many of the challenges surrounding family-centered rounding might be addressed through access to training for caregivers and providers alike. Hospitals should invest in systems to facilitate family-centered rounding if they choose to implement this model of care as the current state risks erosion of provider-caregiver relationship.

Hospitalised children have become more medically complex and increasingly require specialised teams and units properly equipped to care for them. Within paediatric cardiology, this trend, which is well demonstrated by the expansion of cardiology-specific ICUs, has more recently led to the development of acute care cardiology units to deliver team-based and condition-focused inpatient care. These care teams are now led by paediatric cardiologists with particular investment in the acute care cardiology environment. Herein, we describe the foundation and development of an Acute Care Cardiology Advanced Training Fellowship to meet the clinical, scholarly, and leadership training needs of this emerging care environment.

Background: Pain following surgery for cardiac disease is ubiquitous, and optimal management is important. Despite this, there is large practice variation. To address this, the Paediatric Acute Care Cardiology Collaborative undertook the effort to create this clinical practice guideline., Methods: A panel of experts consisting of paediatric cardiologists, advanced practice practitioners, pharmacists, a paediatric cardiothoracic surgeon, and a paediatric cardiac anaesthesiologist was convened. The literature was searched for relevant articles and Collaborative sites submitted centre-specific protocols for postoperative pain management. Using the modified Delphi technique, recommendations were generated and put through iterative Delphi rounds to achieve consensus., Results: 60 recommendations achieved consensus and are included in this guideline. They address guideline use, pain assessment, general considerations, preoperative considerations, intraoperative considerations, regional anaesthesia, opioids, opioid-sparing, non-opioid medications, non-pharmaceutical pain management, and discharge considerations., Conclusions: Postoperative pain among children following cardiac surgery is currently an area of significant practice variability despite a large body of literature and the presence of centre-specific protocols. Central to the recommendations included in this guideline is the concept that ideal pain management begins with preoperative counselling and continues through to patient discharge. Overall, the quality of evidence supporting recommendations is low. There is ongoing need for research in this area, particularly in paediatric populations.

Background: The Pediatric Acute Care Cardiology Collaborative (PAC3) is a learning network focused on improving acute care cardiology patient outcomes. Data submission timeliness is a vulnerability for PAC3 and most clinical registries, directly affecting collaborative benchmarking, quality improvement (QI) and research projects., Objective: PAC3 conducted a collaborative-wide QI project addressing data timeliness and efficiency. Data analysis of submitted cases from September 2019 to February 2020 revealed nine 'High Performer' centers who submitted cases within 67 days of hospital discharge (the limit for timeliness) >90% of the time and eight 'High Potential' sites who submitted timely cases <75% of the time. The primary aim was to increase case submission timeliness in 'High Potential' centers from 41% to 80% by December 2020. The secondary aim was to maintain timeliness in 'High Performer' sites., Method: During the intervention phase (March-December 2020), plan-do-study-act (PDSA) cycles included webinars, facilitated exploratory conversations, data review and development of a best practice guide ('Getting Started Toolkit'). On-boarded 'New Centers' starting in 2020 were also invited to test intervention effectiveness. Balancing measures included data collector job satisfaction and stress and resubmission rates., Results: 'High Performer' and 'High Potential' centers submitted 11 358 cases from November 2019 to December 2020. Timely submission rates for 'High Potential' centers improved from 40.6% to 74.6% and were maintained at >90% for 'High Performer' centers. 'New Centers' averaged 92.6% timely case submissions during their first 6 months. Data collector job satisfaction and stress were not impacted, and the resubmission rates did not increase., Conclusion: PAC3's multicenter QI project increased data submission timeliness in a large pediatric subspecialty registry. The lessons learned and the Toolkit developed can be applied in other registries to improve data submission efficiency, with resultant improvement in benchmarking, QI, research, length of stay and outcomes., (© The Author(s) 2022. Published by Oxford University Press on behalf of International Society for Quality in Health Care. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

Competing Interests: Competing interests: None declared.

The burden of clinically-apparent cardiac injury secondary to coronavirus disease 2019 (COVID-19) in school-age children is unclear. Over 12 months at a large academic pediatric hospital in the Midwestern portion of the United States, there were 1481 COVID-19 positive non-hospitalized otherwise healthy schoolaged children with 195 having cardiac testing performed. While incidental findings occurred, no definitive COVID-19 related cardiac pathology was discovered. Additionally, only ~3% of children had acute cardiac symptoms necessitating evaluation by pediatric cardiology. School-age children who were not hospitalized for COVID-19 have a very low risk of having clinically significant cardiac damage and are more likely to discover incidental findings., Competing Interests: None, (Sociedad Argentina de Pediatría.)

Background: Increased arterial stiffness is associated with diastolic dysfunction in adults. Data in youth are lacking, so we examined the impact of arterial stiffness on diastolic function in youth. Methods: We obtained diastolic function and augmentation index, pulse wave velocity, brachial artery distensibility, and carotid stiffness on 612 youth [10-24 years, 65% female, 38% normal weight, 36% obese, and 26% with type 2 diabetes mellitus (T2DM)]. Participants were classified as compliant (C) vs. stiff (S) arteries based on seven arterial stiffness parameters [Global Stiffness Index (GSI), S = GSI > 4). Mean differences in covariates were evaluated by Student's t -tests. A stepwise regression analysis was performed to determine if GSI was an independent predictor of diastolic function. Results: Lower diastolic function and more adverse cardiovascular disease (CVD) risk factors were present in the S group ( n = 67) than the C group ( n = 545) ( p < 0.001). Covariates that were associated with diastolic dysfunction were higher GSI, male sex, higher body mass index (BMI), and systolic blood pressure (SBP) z -score ( R 2 = 0.18 to 0.25; p ≤ 0.05). Conclusion: Adverse diastolic function is seen in youth with increased arterial stiffness independent of CVD risk factors. Interventions to improve arterial stiffness prior to clinical onset of diastolic dysfunction are needed to prevent development of heart failure., Competing Interests: EU received grant funding for this project from NIH (NHLBI) R01 HL105591. The remaining authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2021 Madsen, Haley, Moore, Khoury and Urbina.)

Background Congenital heart disease practices and outcomes vary significantly across centers, including postoperative chest tube (CT) management, which may impact postoperative length of stay (LOS). We used collaborative learning methods to determine whether centers could adapt and safely implement best practices for CT management, resulting in reduced postoperative CT duration and LOS. Methods and Results Nine pediatric heart centers partnered together through 2 learning networks. Patients undergoing 1 of 9 benchmark congenital heart operations were included. Baseline data were collected from June 2017 to June 2018, and intervention-phase data were collected from July 2018 to December 2019. Collaborative learning methods included review of best practices from a model center, regular data feedback, and quality improvement coaching. Center teams adapted CT removal practices (eg, timing, volume criteria) from the model center to their local resources, practices, and setting. Postoperative CT duration in hours and LOS in days were analyzed using statistical process control methodology. Overall, 2309 patients were included. Patient characteristics did not differ between the study and intervention phases. Statistical process control analysis showed an aggregate 15.6% decrease in geometric mean CT duration (72.6 hours at baseline to 61.3 hours during intervention) and a 9.8% reduction in geometric mean LOS (9.2 days at baseline to 8.3 days during intervention). Adverse events did not increase when comparing the baseline and intervention phases: CT replacement (1.8% versus 2.0%, P =0.56) and readmission for pleural effusion (0.4% versus 0.5%, P =0.29). Conclusions We successfully lowered postoperative CT duration and observed an associated reduction in LOS across 9 centers using collaborative learning methodology.

Background: Hospital discharge delays can negatively affect patient flow and hospital charges. Our primary aim was to increase the percentage of acute care cardiology patients discharged within 2 hours of meeting standardized medically ready (MedR) discharge criteria. Secondary aims were to reduce length of stay (LOS) and lower hospital charges., Methods: A multidisciplinary team used quality improvement methods to implement and study MedR discharge criteria in our hospital electronic health record. The criteria were ordered on admission and modified on daily rounds. Bedside nurses documented the time when all MedR discharge criteria were met. A statistical process control chart measured interventions over time. Discharge before noon and 30-day readmissions were also tracked. Average LOS was examined, comparing the first 6 months of the intervention period to the last 6 months. Inpatient charges were reviewed for patients with >2 hours MedR discharge delay., Results: The mean percentage of patients discharged within 2 hours of meeting MedR discharge criteria increased from 20% to 78% over 22 months, with more patients discharged before noon (19%-32%). Median LOS decreased from 11 days (interquartile range: 6-21) to 10 days (interquartile range: 5-19) ( P = .047), whereas 30-day readmission remained stable at 16.3%. A total of 265 delayed MedR discharges beyond 2 hours occurred. The sum of inpatient charges from care provided after meeting MedR criteria was $332 038 (average $1253 per delayed discharge)., Conclusions: Discharge timeliness in pediatric acute care cardiology patients can be improved by standardizing medical discharge criteria, which may shorten LOS and decrease medical charges., Competing Interests: POTENTIAL CONFLICT OF INTEREST: The authors have indicated they have no potential conflicts of interest to disclose., (Copyright © 2021 by the American Academy of Pediatrics.)

We utilized the multicenter Pediatric Acute Care Cardiology Collaborative (PAC 3 ) 2017 and 2019 surveys to describe practice variation in therapy availability and changes over a 2-year period. A high acuity therapies (ATs) score was derived (1 point per positive response) from 44 survey questions and scores were compared to center surgical volume. Of 31 centers that completed the 2017 survey, 26 also completed the 2019 survey. Scores ranged from 11 to 34 in 2017 and 11 to 35 in 2019. AT scores in 2019 were not statistically different from 2017 scores (29/44, IQR 27-32.5 vs. 29.5/44, IQR 27-31, p = 0.9). In 2019, more centers reported initiation of continuous positive airway pressure (CPAP) and Bi-level positive airway pressure (BiPAP) in Acute Care Cardiology Unit (ACCU) (19/26 vs. 4/26, p < 0.001) and permitting continuous CPAP/BiPAP (22/26 vs. 14/26, p = 0.034) compared to 2017. Scores in both survey years were significantly higher in the highest surgical volume group compared to the lowest, 33 ± 1.5 versus 25 ± 8.5, p = 0.046 and 32 ± 1.7 versus 23 ± 5.5, p = 0.009, respectively. Variation in therapy within the ACCUs participating in PAC 3 presents an opportunity for shared learning across the collaborative. Experience with PAC 3 was associated with increasing available respiratory therapies from 2017 to 2019. Whether AT scores impact the quality and outcomes of pediatric acute cardiac care will be the subject of further investigation using a comprehensive registry launched in early 2019.

Introduction: The prevalence of congenital heart disease (CHD) in adults is rising necessitating a greater understanding of acquired diseases such as community-acquired pneumonia, which remains a leading cause of age-related mortality and morbidity in the general population. We hypothesise that the CHD population, given cardiopulmonary mechanics and altered immune function, bears a uniquely high risk for pneumonia-related hospitalisations and mortality., Methods: A countrywide cohort study was performed to calculate the relative risk and cumulative incidence of pneumonia hospitalisations and resultant 30-day mortality amongst the adult CHD population, matched 1:10 with non-CHD persons by gender, age, and adjusted for comorbidities. Cox proportional hazard regression quantified the impact of CHD severity and extracardiac defects., Results: The CHD cohort includes 17,162 adults. The majority demonstrate mild/moderate CHD complexity. The cumulative incidence of pneumonia hospitalisation was higher for adults with CHD (hazard ratio 1.90; 95% confidence interval: 1.74-2.06) than the comparison cohort. This risk was increased for those with extracardiac defects or a syndrome (hazard ratio: 4.34; 95% confidence interval: 3.39-5.54). Additionally, CHD individuals with severe/univentricular subtypes demonstrate a heightened risk compared to the non-CHD cohort (hazard ratio: 2.35; 95% confidence interval: 1.94-2.84), as well as compared to those with mild/moderate CHD (hazard ratio: 1.28; 95% confidence interval: 1.07-1.53). In addition, pneumonia hospitalisation mortality was elevated above the comparison population with a 30-day mortality rate ratio of 1.31 (95% confidence interval: 1.00-1.73)., Conclusion: Adults with CHD are at elevated risk of pneumonia hospitalisations and pneumonia-associated mortality. This risk is further elevated in those with severe CHD and extracardiac defects.

Among children with congenital heart disease (CHD), there is often neurodevelopmental and behavioral impairment with unclear implications regarding adult socioeconomic achievements. We aimed to compare economic self-sufficiency and educational attainment in CHD adults with sibling and general population controls. Using Danish population-based nationwide registries, this cohort study aimed to include all CHD subjects greater than 13 years born between 1963 and 1993. Comparison cohorts included: (1) sibling cohort and (2) general population cohort matched 10:1 on birth year and gender. We computed cumulative incidences of time to first full year of economic self-sufficiency, as well as educational attainment. We assessed the relative probability of self-sufficiency in all cohorts before 30 years of age, defined by Statistics Denmark federal standard. In total, we identified 7,019 CHD subjects, 6,257 full siblings, and 68,805 general population controls. The cumulative incidence of self-sufficiency by age 20 and 35 years for CHD subjects (49% and 84%, respectively) was lower than sibling (68% and 96%) and general population cohorts (67% and 95%). The relative probability of self-sufficiency for CHD subjects compared with siblings was 0.44 (95% confidence interval 0.39 to 0.49). By age 30, adults with CHD were less likely than their siblings to attain all levels of education. Among those achieving higher educational milestones, differences in self-sufficiency between cohorts were absent by age 35. In conclusion, CHD is associated with reduced adult economic self-sufficiency, and the relation between educational level attained and self-sufficiency may suggest that targeted interventions have the potential to improve adult self-sufficiency., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Background: CHD is the most common birth defect type, with one-fourth of patients requiring intervention in the first year of life. Caregiver understanding of CHD may vary. Health literacy may be one factor contributing to this variability., Methods: The study occurred at a large, free-standing children's hospital. Recruitment occurred at a free-of-charge CHD camp and during outpatient cardiology follow-up visits. The study team revised the CHD Guided Questions Tool from an eighth- to a sixth-grade reading level. Caregivers of children with CHD completed the "Newest Vital Sign" health literacy screen and demographic surveys. Health literacy was categorised as "high" (Newest Vital Sign score 4-6) or "low" (score 0-3). Caregivers were randomised to read either the original or revised Guided Questions Tool and completed a validated survey measuring understandability and actionability of the Guided Questions Tool. Understandability and actionability data analysis used two-sample t-testing, and within demographic group differences in these parameters were assessed via one-way analysis of variance., Results: Eighty-two caregivers participated who were largely well educated with a high income. The majority (79.3%) of participants scored "high" for health literacy. No differences in understanding (p = 0.43) or actionability (p = 0.11) of the original and revised Guided Questions Tool were noted. There were no socio-economic-based differences in understandability or actionability (p > 0.05). There was a trend towards improved understanding of the revised tool (p = 0.06)., Conclusions: This study demonstrated that readability of the Guided Questions Tool could be improved. Future work is needed to expand the study population and further understand health literacy's impact on the CHD community.

Paediatric chest pain is common, yet there remain significant barriers to educating and reassuring patients and their families. In July 2014, a blog titled 6 Questions to Ask When Your Child Complains of Chest Pain was posted to the Cincinnati Children's Hospital Medical Center website to help educate children and parents about paediatric chest pain. The enhanced interactions through internet-based resources create the opportunity to address chest pain in a way that both educate and provide reassurance. Through the reassurance, there is the potential to meaningfully reduce patient stress all while also decreasing medical costs and potential medical visits., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2020. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

BACKGROUND10: The Joint Commission identified inpatient alarm reduction as an opportunity to improve patient safety; enhance patient, family and nursing satisfaction; and optimise workflow. We used quality improvement (QI) methods to safely decrease non-actionable alarm notifications to bedside providers., Methods: In a paediatric tertiary care centre, we convened a multidisciplinary team to address alarm notifications in our acute care cardiology unit. Alarm notification was defined as any alert to bedside providers for each patient-triggered monitor alarm. Our aim was to decrease alarm notifications per monitored bed per day by 60%. Plan-Do-Study-Act testing cycles included updating notification technology, establishing alarm logic and modifying bedside workflow processes, including silencing the volume on all bedside monitors. Our secondary outcome measure was nursing satisfaction. Balancing safety measures included floor to intensive care unit transfers and patient acuity level., Results: At baseline, there was an average of 71 initial alarm notifications per monitored bed per day. Over a 3.5-year improvement period (2014-2017), the rate decreased by 68% to 22 initial alarm notifications per monitored bed per day. The proportion of initial to total alarm notifications remained stable, decreasing slightly from 51% to 40%. There was a significant improvement in subjective nursing satisfaction. At baseline, 32% of nurses agreed they were able to respond to alarms appropriately and quickly. Following interventions, agreement increased to 76% (p<0.001). We sustained these improvements over a year without a change in monitored balancing measures., Conclusion: We successfully reduced alarm notifications while preserving patient safety over a 4-year period in a complex paediatric patient population using technological advances and QI methodology. Continued efforts are needed to further optimise monitor use across paediatric hospital units., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2020. No commercial re-use. See rights and permissions. Published by BMJ.)

Background: Nearly every child undergoing congenital heart surgery has chest tubes placed intraoperatively. Center variation in removal practices and impact on outcomes has not been well described. This study evaluated variation in chest tube management practices and outcomes across centers., Methods: The study included patients undergoing any of 10 benchmark operations from June 2017 to May 2018 at participating Pediatric Acute Care Cardiology Collaborative (PAC 3 ) and Pediatric Cardiac Critical Care Consortium (PC 4 ) centers. Clinical data from PC 4 centers were merged with chest tube data from PAC 3 centers. Practices and outcomes were compared across centers in univariate and multivariable analysis., Results: The cohort included 1029 patients (N = 9 centers). Median chest tube duration varied significantly across centers for 9 of 10 benchmark operations (all P ≤ .03), with a "model" center noted to have the shortest duration for 9 of 10 operations (range, 27.9% to 87.4% shorter duration vs other centers across operations). This effect persisted in multivariable analysis (P < .0001). The model center had higher volumes of chest tube output before removal (median, 8.5 mL/kg/24 h [model] vs 2.2 mL/kg/24 h [other centers]; P < .001], but it did not have higher rates of chest tube reinsertion (model center 1.3% vs 2.1%; P = .59) or readmission for pleural effusion (model center 4.4% vs 3.0%; P = .31), and had the shortest length of stay for 7 of 10 operations., Conclusions: This study suggests significant center variation in chest tube removal practices and associated outcomes after congenital heart surgery. Best practices used at the model center have informed the design of an ongoing collaborative learning project aimed at reducing chest tube duration and length of stay., (Copyright © 2020 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Congenital heart disease (CHD) is associated with risk factors of central nervous system (CNS) infections including infective endocarditis, cardiac shunt physiology, and immune deficiencies. We aimed to investigate the risk of CNS infections in the CHD population compared to the general population. In this cohort study, we used Danish nationwide medical registries to identify individuals diagnosed with CHD at any age, born between 1977 and 2012. For each CHD individual, we matched 10 individuals on sex and birth year from the general population. Subjects were followed until first-time hospital diagnosis of CNS infection, death, emigration, or end of study. We computed cumulative incidences of CNS infections with death as a competing risk, as well as hazard ratios (HRs) with corresponding 95% confidence intervals (CIs) adjusted for birth year and sex. We identified 17,550 individuals with CHD (50% male). Among subjects with CHD, the cumulative incidence of CNS infection at age 30 years was 1.0% compared to 0.6% in the general population. The overall HR of CNS infections in CHD subjects relative to the general population was 2.1 (95% CI 1.6-2.7). The HR was 1.9 (95% CI 1.4-2.7) for mild to moderate CHD, 2.1 (95% CI 1.3-3.3) for severe CHD and 3.0 (95% CI 1.1-8.2) for univentricular physiology. The HR for CHD subjects without record of infective endocarditis was 2.0 (95% CI 1.5-2.5). The risk of CNS infections was increased among individuals with CHD compared to the general population, and the risk was not limited to individuals with infective endocarditis.

Objectives: To examine the strengths and opportunities for improvement of current home care education practices to inform the development of the Home Care for Heart Health intervention, and to develop a web-based intervention for parents and clinicians with complimentary print materials that could provide the right education at the right time to foster a safer transition from hospital to home., Methods: An inter-professional focus group of parents, clinicians, and designers was formed to co-create a home care education intervention for parents of children with congenital heart disease (CHD) and their care team. We used the Integrated New Product Development process model created by Jonathon Cagan and Craig Vogel at Carnegie Mellon University to develop the intervention. This process model is a way of thinking that combines horizontal and inter-disciplinary teams, stakeholder-centric focus, and a system of qualitative discovery and development evolving towards quantitative methods of refinement., Results: Our team developed the Home Care for Heart Health intervention. The evidenced-based intervention includes a quick reference guide for parents of children with CHD, an accompanying app, family-friendly pathways, and clinician education., Conclusion: Using an inter-professional approach, our team of clinicians, parents, and design experts were able to co-create a clinician-parent home care education intervention with broad application and lifelong relevance to the Congenital Heart Disease Community., Practice Implications: Our intervention has the potential to be used as a model for other home care education interventions for parents of children with chronic illnesses.

Background Congenital heart disease (CHD) is associated with risk factors for ischemic stroke including cardiac arrhythmias and heart failure. However, few long-term follow-up data exist on ischemic stroke risk and associated mortality in adults with CHD. Methods and Results Using Danish nationwide registries, we identified individuals aged ≥18 years diagnosed with CHD, at any age, from 1963 to 2017 and a sex and birth year-matched (1:10) general population comparison cohort. We computed risks, as well as sex and birth year-adjusted hazard ratios (aHRs) for ischemic stroke and 30-day post-stroke mortality in CHD adults compared with the general population. Analyses were stratified according to age <60 years (young) and ≥60 years (older). We identified 16 836 adults with CHD. The risk of ischemic stroke at age 60 years was 7.4% in the CHD cohort and 2.9% in the general population cohort. The adjusted hazard ratios for ischemic stroke compared with the general population was 3.8 (95% CI: 3.3-4.3) in young CHD adults and 1.6 (95% CI: 1.4-1.9) in older CHD adults. The adjusted hazard ratios for post-stroke mortality compared with the general population was 2.3 (95% CI: 1.2-4.4) in young CHD adults and 1.3 (95% CI: 0.9-1.9) in older CHD adults. Conclusions Both younger and older CHD adults have an increased risk of ischemic stroke and by 60 years of age 7.4% of CHD adults will have had an ischemic stroke. Post-stroke mortality was also increased in CHD adults compared with the general population.

Background: The Pediatric Acute Care Cardiology Collaborative (PAC 3 ) was established in 2014 to improve the quality, value, and experience of hospital-based cardiac acute care outside of the intensive care unit. An initial PAC 3 project was a comprehensive survey to understand unit structure, practices, and resource utilization across the collaborative. This report aims to describe the previously unknown degree of practice variation across member institutions., Methods: A 126-stem question survey was developed with a total of 412 possible response fields across nine domains including demographics, staffing, available resources and therapies, and standard care practices. Five supplemental questions addressed surgical case volume and number of cardiac acute care unit (CACU) admissions. Responses were recorded and stored in Research Electronic Data Capture (REDCap)., Results: Surveys were completed by 31 out of 34 centers (91%) with minimal incomplete fields. A majority (61%) of centers have a single dedicated CACU, which is contiguous or adjacent to the intensive care unit in 48%. A nurse staffing ratio of 3:1 is most common (71%) and most (84%) centers employed a resource nurse. Centralized wireless rhythm monitoring is used in 84% of centers with 54% staffed continuously. There was significant variation in the use of noninvasive respiratory support, vasoactive infusions, and ventricular assist devices across the collaborative. Approximately half of the surveyed centers had lesion-specific postoperative pathways and approximately two-thirds had protocols for single-ventricle patients., Conclusions: The PAC 3 hospital survey is the most comprehensive description of systems and care practices unique to CACUs to date. There exists considerable heterogeneity among unit composition and variation in care practices. These variations may allow for identification of best practices and improved quality of care for patients., (© 2019 Wiley Periodicals, Inc.)

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.

Background: More children with congenital heart disease (CHD) are surviving to adulthood, and CHD is associated with risk factors for dementia. We compared the risk of dementia in CHD adults to that of the general population., Methods: In this cohort study, we used medical registries and a medical record review covering all Danish hospitals to identify adults with CHD diagnosed between 1963 and 2012. These individuals with CHD were followed from January 1, 1981, 30 years of age, or date of first CHD registration (index date for matched members of the general population cohort) until hospital diagnosis of dementia, death, emigration, or end of study (December 31, 2012). For each individual with CHD, we identified 10 members of the general population utilizing the Danish Civil Registration System matched on sex and birth year. We computed cumulative incidences and hazard ratios (HRs) of dementia, adjusting for sex and birth year., Results: The cumulative incidence of dementia was 4% by 80 years of age in 10 632 adults with CHD (46% male). The overall HR comparing adults with CHD with the general population cohort was 1.6 (95% confidence interval [CI], 1.3-2.0). The HR among individuals with CHD without extracardiac defects was 1.4 (95% CI, 1.1-1.8). Adults with mild-to-moderate CHD had an HR of 1.5 (95% CI, 1.1-2.0), whereas the HR was 2.0 (95% CI, 1.2-3.3) for severe CHD, including univentricular hearts. The HR for early onset dementia (<65 years of age) was 2.6 (95% CI, 1.8-3.8), whereas the late-onset HR was 1.3 (95% CI, 1.0-1.8)., Conclusions: CHD was associated with an increased risk of dementia compared with the general population, in particular for early onset dementia. Further understanding of dementia risk in the population with CHD is a potential target for future investigation., (© 2018 American Heart Association, Inc.)

Background: The aim of this study was to examine the association between myocardial strain and arterial thickness and stiffness in young adults. Increased common carotid artery intima media thickness and peripheral arterial stiffness are known to precede coronary artery disease and cardiovascular (CV) events such as myocardial infarction and congestive heart failure. However, subclinical cardiac dysfunction can be detected in high-risk adults by myocardial strain echocardiography. The authors hypothesized that increased carotid artery intima media thickness would be associated with abnormal myocardial strain in young subjects who had obesity and type 2 diabetes mellitus., Methods: CV risk factors were collected in 338 young adults participating in a prospective, cross-sectional study. The CV parameters collected included intima-media thickness, peripheral arterial stiffness by brachial distensibility, and myocardial strain and strain rate. General linear models were constructed to determine if vascular structure and function measures were independently associated with myocardial strain and strain rate., Results: A linear relationship was found between global longitudinal strain obtained from the four-chamber view and global strain rate in systole and carotid intima-media thickness (four-chamber global longitudinal strain: β = 3.0, CV risk factor-adjusted R 2  = 0.34; global strain rate in systole: β = 0.0053, R 2  = 0.21; P ≤ .0001) and between four-chamber global longitudinal strain and lower brachial distensibility (β = -0.42, R 2  = 0.22; P < .001)., Conclusions: Adverse changes in vascular structure and function are simultaneously present with reduced myocardial systolic function., (Copyright © 2017 American Society of Echocardiography. Published by Elsevier Inc. All rights reserved.)

Cardiac surgery associated-acute kidney injury (CS-AKI) occurs in 30-50% of patients undergoing surgery for congenital heart disease. Here we determine if CS-AKI is associated with chronic kidney disease (CKD) in patients with congenital heart disease. Using Danish regional population-based registries, our cohort study included patients with congenital heart disease born between 1990-2010 with first cardiac surgery between 2005 and 2010 (under 15 years of age). Utilizing in- and out-patient laboratory serum creatinine data, we identified individuals fulfilling KDIGO stages of AKI within 5 days of cardiac surgery. A unique personal identifier enabled unambiguous data linkage and virtually complete follow-up. The cumulative incidences of CKD stages 2-5 according to presence of CS-AKI were computed utilizing serum creatinine values and Pottel's formula. Using Cox regression, the corresponding hazard ratios were computed, adjusting for sex, age at first cardiac surgery, calendar period of surgery, and congenital heart disease severity. Of 382 patients with congenital heart disease undergoing cardiac surgery, 127 experienced CS-AKI within 5 days of surgery. Median follow-up was 4.9 years. The five-year cumulative incidence of CKD for patients with CS-AKI was 12% (95% confidence interval 7%-20%), significantly higher than the 3% (1%-5%) for those without CS-AKI with a significant adjusted hazard ratio of 3.8 (1.4-10.4). Thus, CS-AKI in patients with congenital heart disease is common and is associated with an increased risk for CKD., (Copyright © 2017 International Society of Nephrology. Published by Elsevier Inc. All rights reserved.)

Background: Long-term survival for persons born with congenital heart disease (CHD) is improved, but limited knowledge exists of this growing population's acquired cardiovascular risk profile. This study's purpose was to assess CHD survivors' risk for type 2 diabetes mellitus (T2DM) with attention to the impact of cyanotic CHD., Methods and Results: This population-based cohort study included Danish subjects with CHD who were born between 1963 and 1980 and were alive at age 30 years. For each CHD case, we identified 10 individuals from the general population matched by sex and birth year, by using the Danish Civil Registration System. Complete follow-up was obtained through Danish public registries for death, emigration, and T2DM (diagnosis and prescriptions record). We computed cumulative incidences and hazard ratios of developing T2DM after age 30 for 5149 CHD subjects compared with the general population. After adjusting for CHD severity, as well as age, sex, preterm birth, and extracardiac defects, we analyzed the impact of cyanotic compared with acyanotic CHD. By age 45 years, the cumulative incidence of T2DM after age 30 was 4% among subjects with CHD. Subjects with CHD were more likely to develop T2DM than the general population (hazard raio 1.4, 95% CI 1.1-1.6). Subjects CHD who had cyanotic defects were more likely to develop T2DM than were subjects with acyanotic CHD (hazard ratio 1.9, 95% CI 1.1-3.3)., Conclusions: CHD survivors had an increased risk of developing T2DM after age 30. Patients with cyanotic CHD are at particular risk. Given the cardiovascular health burden of T2DM, attention to its development in CHD survivors seems warranted., (© 2016 The Authors. Published on behalf of the American Heart Association, Inc., by Wiley Blackwell.)

Sudden cardiac death in the young (SCDY) is the leading cause of death in young athletes during sport. Screening young athletes for high-risk cardiac defects is controversial. The purpose of this study was to assess the utility and feasibility of a comprehensive cardiac screening protocol in an adolescent population. Adolescent athletes were recruited from local schools and/or sports teams. Each subject underwent a history and/or physical examination, an electrocardiography (ECG), and a limited echocardiography (ECHO). The primary outcome measure was identification of cardiac abnormalities associated with an elevated risk for sudden death. We secondarily identified cardiac abnormalities not typically associated with a short-term risk of sudden death. A total of 659 adolescent athletes were evaluated; 64% men. Five subjects had cardiac findings associated with an elevated risk for sudden death: prolonged QTc >500 ms (n = 2) and type I Brugada pattern (n = 1), identified with ECG; dilated cardiomyopathy (n = 1) and significant aortic root dilation; and z-score = +5.5 (n = 1). History and physical examination alone identified 76 (11.5%) subjects with any cardiac findings. ECG identified 76 (11.5%) subjects in which a follow-up ECHO or cardiology visit was recommended. Left ventricular mass was normal by ECHO in all but 1 patient with LVH on ECG. ECHO identified 34 (5.1%) subjects in whom a follow-up ECHO or cardiology visit was recommended. In conclusion, physical examination alone was ineffective in identification of subjects at elevated risk for SCDY. Screening ECHO identified patients with underlying cardiac disease not associated with immediate risk for SCDY. Cost of comprehensive cardiac screening is high., (Copyright © 2014 Elsevier Inc. All rights reserved.)

Objective: To evaluate current preparticipation physical evaluation (PPE) clinical practice behaviors., Design: Telephone and Web-based survey study with attention to utilization of the Fourth Edition PPE Monograph., Setting: We contacted the Washington State American Academy of Pediatrics (AAP) and American Academy of Family Physicians (AAFP) chapters, all Washington State high school athletic directors (ADs), and every state high school athletic association., Interventions: Data collection and analysis regarding clinical practice., Main Outcome Measures: Awareness and utilization of the Fourth Edition PPE Monograph., Results: The response rate was 72% (559/776) from the AAP, 56% (554/990) from the AAFP, 75% (317/424) from the ADs, and 100% (50/50) from the state athletic associations. Few physicians (37%) and ADs (6%) reported an awareness of the PPE Monograph. Knowledge of the Monograph did not vary by physician type, practice/school location, or experience (P > 0.05). Reported obstacles to the delivery of the PPE included time with patient (56%) and the lack of a standard form (52%). Physician awareness of the Monograph reduced the perception of obstacles (P < 0.01). Athletic directors reported financial limitations of the family as the primary obstacle (62%). Adoption of a single statewide PPE form was well supported (96% of physicians and 67% of ADs). Nationally, only 46% of state athletic associations mandate a single form, and only 16% use a form consistent with the Fourth Edition PPE Monograph., Conclusions: The medical community is largely unaware of national screening guidelines. New directions for education and policy are necessary to improve this implementation gap.

Objective: The purpose of this study was to evaluate the association between fetal development of congenital heart defects (CHD) and maternal prepregnancy body mass index (BMI, kg/m(2)) in the largest population-based case-control study to date., Background: Mounting evidence implicates maternal obesity as a risk factor for birth defects. However, the association between maternal obesity and CHD in offspring has been less completely described., Methods: We conducted a study of CHD using linked birth-hospital discharge records for Washington State between 1992-2007. All infants with CHD (n = 14,142) were identified based on ICD9-CM discharge diagnosis codes. 141,420 controls, frequency matched on year of delivery, were selected at random from among infants without CHD. Maternal BMI was calculated from maternal prepregnancy data. Odds ratios (OR) and 95% confidence intervals (CI) for the association of CHD relative to maternal BMI were calculated, adjusted for gestational diabetes., Results: Infants with CHD were more likely to have an obese mother (OR 1.22, 95% CI 1.15-1.30). The strength of association increased with increasing BMI (BMI 30-34.9: OR 1.16, 95% CI 1.07-1.25; BMI 35-39.9: OR 1.25, 95% CI 1.13-1.39; BMI > = 40: OR 1.49, 95% CI 1.32-1.69). The association was greatest for left and right ventricular outflow tract defects (OR 1.27, 95% CI 1.02-1.59 and OR 1.43, 95% CI 1.20-1.69, respectively). Hypoplastic left heart syndrome was markedly associated (OR 1.86, 95% CI 1.13-3.05). There was no association with conotruncal defects (OR 1.04, 95% CI: 0.82-1.33)., Conclusions: We confirmed the association between CHD and maternal obesity and observed increasing risk with increasing obesity. Outflow tract defects appear uniquely associated. A greater risk of CHD among offspring is an important outcome of maternal obesity, and suggests a need for targeted medical management strategies., (© 2012 Wiley Periodicals, Inc.)

Objective: In the USA, the preparticipation physical evaluation (PPE) is the standard of care for screening eight million high-school athletes for their risk of sudden cardiac death (SCD). Our aim was to evaluate both physician and school compliance with national guidelines for SCD screening., Methods: We conducted a confidential survey of the Washington Chapter of the American Academy of Pediatrics (AAP), the Washington Academy of Family Physicians (WAFP) and Washington State high-school athletic directors. Responses were evaluated for compliance with the American Heart Association (AHA) guidelines for SCD screening., Results: We received a response rate of 72% (559/776) from the AAP, 56% (554/990) from the WAFP and 78% (317/409) from the athletic directors. Only 6% of all providers and 0% of schools were in compliance with AHA guidelines. In addition, 47% of the physicians and 6% of athletic directors reported awareness of the guidelines. There was no difference in compliance between physician specialties (p=0.20). Physician location, years of experience and exposure to SCD were not significantly associated with compliance. Provider knowledge of the guidelines, number of PPE/month and frequency of referrals to cardiology were all positively associated with improved overall compliance (p<0.05)., Conclusions: Despite the unaltered presence of the AHA SCD screening guidelines for the past 15 years, compliance with the recommendations is poor. Lack of compliance does not reflect clinical experience, but rather lack of knowledge of the guidelines themselves. New directions for provider education and policy requirements are needed to improve this implementation gap.