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The Fontan procedure is used to palliate complex forms of congenital heart disease. This results in adverse hepatic sequelae now known as Fontan-associated liver disease (FALD). Conventional laboratory measures of liver disease do not correlate well with FALD severity. Cytokeratin-18 (CK-18) is a measure of cell death and is sensitive in detecting other causes of liver disease. Our aim was to assess the use of a novel measure of liver disease, CK-18, in Fontan patients. This is a single-center, prospective, cross-sectional study of Fontan patients aged 8-21 years old. We performed ultrasound elastography, echocardiography, magnetic resonance imaging, and serum laboratory testing. Novel laboratory test CK-18 levels in Fontan subjects were compared to healthy age-matched controls. Thirteen Fontan patients were evaluated with a median age 15 years (10, 14), 4 Hypoplastic left heart syndrome, 11 were male, and 5 were symptomatic. Fontan patients had normal AST/ALT, but a significantly elevated liver stiffness by elastography (median 13.4 kPa). Hepatic stiffness by elastography was associated with diastolic-indexed (rho = 0.58, p = 0.04) ventricular volumes. Compared to 10 aged-matched controls, CK-18 was higher in the Fontan group-cleaved CK-18 protein (p < 0.01) and full CK-18 protein, (p = 0.02). CK-18 was positively associated with AST and ALT. Elevated CK-18 levels were found in Fontan patients compared to controls suggesting hepatic cell death even in these relatively healthy Fontan patients. CK-18 was elevated prior to changes in traditional testing. CK-18 may be a useful sensitive marker of liver disease in FALD.

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Entrustable professional activities (EPAs) are "observable essential tasks expected to be performed by a physician for safe patient care in practice." Six Pediatric Cardiology (PC) EPAs and their level of supervision (LOS) scales were developed by medical educators in PC using a modified Delphi process and reviewed by the Subspecialty Pediatrics Investigator Network (SPIN). However, their general use in assessment for PC fellows for graduation requirements has yet to be studied. The objective of this study was to determine the minimum LOS required for PC fellows to graduate and compare it with the minimum LOS expected for safe and effective practice for the six PC EPAs, from the perspective of the PC Fellowship Program Directors(FPD). All Fellowship Program Directors(FPD) of ACGME-accredited PC fellowships were surveyed through SPIN between April 2017 and August 2017. For each of the PC EPAs, the FPDs were asked to indicate the minimum LOS expected for graduation and whether they would allow a fellow to graduate if this level was not achieved and the minimum LOS expected for a practicing pediatric cardiologist to provide safe and effective patient care. The minimum LOS was defined as the LOS for which no more than 20% of FPDs would want a lower level. The survey response rate was 80% (47/59). The majority of the FPDs did not require a minimum LOS of five corresponding to unsupervised practice in any of the six PC EPAs at graduation. For EPAs related to imaging, arrhythmia management, and management of cardiac problems, the minimum LOS for graduation was 3, corresponding to being "trusted to perform a task with indirect supervision for most simple and a few complex cases." For the EPAs related to interventional cardiology, heart failure pulmonary hypertension, and cardiac intensive care, the minimum LOS for graduation was 2, corresponding to being "trusted to perform a task only with direct supervision and coaching." The minimum LOS considered necessary for safe and effective practice for all but one EPA was 3. For the EPA related to the management of cardiac problems, the minimum LOS for safe practice was 4, corresponding to being "trusted to execute tasks independently except for few complex and critical cases." Most PC FPDs reported they would not require fellows to achieve the highest entrustment level for any of the six PC EPAs for graduation. It is crucial that educational programs evolve to address these essential activities during training better and that stakeholders ensure that graduating PC fellows have adequate resources and infrastructure to continue professional development as early career pediatric cardiologists.

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BACKGROUND: During the COVID-19 pandemic, Kentucky prohibited elective medical procedures from 3/18/2020-4/27/2020. We sought to determine if cessation of elective procedures in Kentucky during the COVID-19 pandemic resulted in a decrease in the proportion of rarely appropriate outpatient transthoracic echocardiograms interpreted at the open echocardiography lab at Norton Children's Hospital. METHODS: A retrospective chart review was conducted comparing proportions of rarely appropriate outpatient paediatric transthoracic echocardiograms performed pre-COVID (3/21/2019-4/28/2019) and during COVID (3/19/2020-4/27/2020). Transthoracic echocardiogram indication was determined by chart review and echocardiogram reports. Indication appropriateness was evaluated using paediatric appropriate use criteria for initial outpatient transthoracic echocardiogram or CHD follow-up as applicable. RESULTS: Of transthoracic echocardiograms pre-COVID, 100 (37.7%) were rarely appropriate versus 18 (20.2%) during COVID. Pre-COVID, paediatric cardiologists tended to order fewer rarely appropriate transthoracic echocardiograms than paediatricians (35.9% versus 46.4%), although this difference was not statistically significant. Cardiologists ordered the majority of outpatient transthoracic echocardiograms during COVID (77/89, 86.5%), limiting the ability to compare transthoracic echocardiogram indications by provider type. There was no significant difference in diagnostic yield of initial outpatient transthoracic echocardiograms with (13.0%) abnormal studies pre-COVID versus 7 (15.5%) during COVID. CONCLUSION: While elective procedures were prohibited in Kentucky during the COVID-19 pandemic, a decrease in the proportion of rarely appropriate outpatient paediatric transthoracic echocardiograms was observed. There was no significant difference in diagnostic yield of initial outpatient transthoracic echocardiograms between time periods, suggesting that clinically significant echocardiogram findings were still detected despite more prudent utilisation of echocardiography during this time.

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OBJECTIVE: This study sought to determine the impact of cardiac point-of-care ultrasound (cPOCUS) in a pediatric emergency department (ED) on cardiology subspecialty utilization for subjects with chest pain or syncope. Diagnostic yield of cPOCUS and transthoracic echocardiograms (TTEs) for these subjects was also examined. METHODS: A retrospective chart review of subjects presenting to a tertiary pediatric ED with chest pain or syncope 1 year before (2015, pre-cPOCUS group) and 1 year after (2017, cPOCUS group) introduction of cPOCUS was conducted. Subjects aged 2 to 18 years evaluated for these symptoms were included. Those with known heart defects, prior abnormal TTE, or asthma exacerbation at presentation were excluded. In both groups, cardiology subspecialty utilization was assessed by determining whether cardiology referrals, cardiology consultations, or follow-up TTEs were completed. Results of TTEs were reviewed and classified as incidental (no follow-up needed), minor (follow-up needed, but intervention unlikely), moderate (nonurgent intervention needed), and severe (hospitalization/urgent intervention needed). Cardiac point-of-care ultrasound results were compared with any follow-up TTEs. Data were analyzed using χ 2 or Student t test as appropriate. RESULTS: A total of 1230 subjects were analyzed: 595 pre-cPOCUS and 635 cPOCUS group. There was no significant difference in TTEs (42 vs 46), cardiology consultations (36 vs 37), or cardiology referrals (47 vs 37) between groups. Of 67 cPOCUS scans performed, 63 were normal, 3 showed small pericardial effusion, and 2 demonstrated left ventricular dysfunction. Of 88 TTEs in both groups (0.7% subjects), 76 were normal, 5 had incidental, 6 had minor, and 1 had a severe finding present on cPOCUS (0.08% subjects; 95% confidence interval, 0%-0.45%). CONCLUSIONS: The introduction of cPOCUS did not increase cardiology subspecialty utilization in subjects presenting to the pediatric ED with chest pain or syncope. Cardiac point-of-care ultrasound may be useful in evaluating global biventricular systolic function and effusion in this population.

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Most children with congenital heart disease (CHD) survive to adulthood, owing largely to significant advances in the diagnosis and management of CHD over the past few decades. Primary care providers are essential partners in the recognition and management of these patients in our current medical environment. This article reviews the role of the primary care physician in detecting fetuses, infants, and children with possible CHD. Furthermore, this article discusses common primary care issues arising for patients with CHD, including growth and development, mental illness, dental care, and the transition to adult primary care.

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OBJECTIVE: The 2016 American Board of Pediatrics (ABP) content outline is comprehensive, including more than 50 cardiology-specific objectives within eight content areas. This study complements the quantitative analysis of a Kentucky-wide survey of trainees, pediatricians, and pediatric cardiologists asking them to identify "most important" cardiology content by analyzing their open-ended comments about "what should be added" and "why?" within these eight categories. DESIGN, METHODS, OUTCOME MEASURES: This cross-sectional study used an original, online survey instrument based on the 2016 ABP cardiology-specific objectives. We began an initial analysis of the qualitative data using Pandit's version of Glaser and Strauss Grounded theory (constant comparison). However, upon finding an abundance of comments focused on Diagnosis, we proceeded with a secondary analysis that further categorized Diagnosis comments into three themes aligned with Bloom's taxonomy. Additional comments focused on Management and clustered into Emergent/Acute Care (Resuscitation); Short-term Care (Inpatient); and Longitudinal Care (Outpatient). RESULTS: Of the 136 respondents, 23 (17%) were residents, 15 (11%) fellows, 85 (62%) pediatricians, and 13 (10%) pediatric cardiologists with 80% of attendings having faculty/gratis faculty status. The open-ended questions "what needs to be added" and "why" generated 93 comments; 60 of which focused on Diagnosis; further classified as Recognize (16), Differentiate (12), and Evaluate (32). Management comments were related to acuity and care setting, grouped as Emergent/Acute Care (Resuscitation) [10]; Short-term Care (Inpatient) [6]; and Longitudinal Care (Outpatient) [17]. CONCLUSIONS: The 93 comments analyzed for this article showed a distinct preference for all respondents, trainees, pediatricians, and cardiologists alike, to value the addition of diagnostic skills with emphasis in the "evaluate" skill set as important cardiology curricular content beyond that included in the 2016 ABP cardiology-specific objectives. Responses could be used to provide practical guidance for curriculum design and reform.

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OBJECTIVE: Although pediatrician-reported relevance of Canadian cardiology-specific objectives has been studied, similar data are not available for the 2016 American Board of Pediatrics (ABP) cardiology-specific objectives. This study asked Kentucky trainees, pediatricians, and pediatric cardiologists to identify "most important" content within these objectives. DESIGN, METHODS, OUTCOME MEASURES: This cross-sectional study used an original, online survey instrument based on the 2016 ABP cardiology-specific objectives. We collected quantitative data (numerical indications of importance) and qualitative data (open-ended replies regarding missing content and difficulty in teaching and learning). Respondents indicated the top two choices of most important items within eight content areas. Descriptive statistics (frequencies and percentages) and chi-square analysis were calculated. Content within categories was organized using naturally occurring "clusters" and "gaps" in scores. Common themes among open-ended qualitative responses were identified using Pandit's version of Glaser and Strauss Grounded theory (constant comparison). RESULTS: Of the 136 respondents, 23 (17%) were residents, 15 (11%) fellows, 85 (62%) pediatricians, and 13 (10%) pediatric cardiologists. Of attendings, 80% reported faculty/gratis faculty status. Naturally occurring clusters in respondent-designated importance resulted in ≤3 "most selected" objectives per content area. Objectives in "most selected" content pertained to initial diagnosis (recognition of abnormality/disease) (n = 16), possible emergent/urgent intervention required (n = 14), building a differential (n = 8), and planning a workup (n = 4). Conversely, themes for "least selected" content included comanagement with subspecialist (n = 15), knowledge useful in patient-family communication (n = 9), knowledge that can be referenced (as needed) (n = 7), and longitudinal/follow-up concerns (n = 5). CONCLUSIONS: This study demonstrated the utility of an online survey methodology to identify pediatric cardiology content perceived most important. Learners and faculty generally provided concordant responses regarding most important content within the cardiology-specific ABP objectives. Medical educators could apply this methodology to inform curriculum revision.

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An evaluation of increased response effort to dispose of items was conducted to improve recycling at a university. Signs prompting individuals to recycle and notifying them of the location of trash and recycling receptacles were posted in each phase. During the intervention, trashcans were removed from the classrooms, and one large trashcan was available in the hallway next to the recycling receptacles. Results showed that correct recycling increased, and trash left in classrooms increased initially during the second intervention phase before returning to baseline levels.

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Sight-word instruction can be a useful supplement to phonics-based methods under some circumstances. Nonetheless, few studies have evaluated the conditions under which pictures may be used successfully to teach sight-word reading. In this study, we extended prior research by examining two potential strategies for reducing the effects of overshadowing when using picture prompts. Five children with developmental disabilities and two typically developing children participated. In the first experiment, the therapist embedded sight words within pictures but gradually faded in the pictures as needed using a least-to-most prompting hierarchy. In the second experiment, the therapist embedded text-to-picture matching within the sight-word reading sessions. Results suggested that these strategies reduced the interference typically observed with picture prompts and enhanced performance during teaching sessions for the majority of participants. Text-to-picture matching also accelerated mastery of the sight words relative to a condition under which the therapist presented text without pictures.

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OBJECTIVE: To assess health-related quality of life (HRQoL) of adolescents with repaired tetralogy of Fallot (TOF) and whether impairments in HRQoL domains are associated with neurocognitive and medical factors. STUDY DESIGN: Parents of subjects with TOF and healthy referents 13-16 years of age completed the Child Health Questionnaire-Parent Form 50, generating psychosocial (PsS) and physical (PhS) health summary scores. Adolescents completed the Child Health Questionnaire-Child Form 87 and concurrent in-person neurocognitive testing. We analyzed relationships of PsS and PhS scores with neurocognitive performance and medical factors. RESULTS: Compared with referents (n = 85), adolescents with TOF without a genetic diagnosis (n = 66) had lower PsS (50.9 ± 9.4 vs 57.2 ± 4.2, P < .001) and PhS scores (49.4 ± 9.5 vs 55.8 ± 4.9; P < .001). Compared with a normative sample, these adolescents with TOF had similar PsS scores (P = .52) but significantly lower PhS scores (P = .01). Within adolescents with TOF without genetic disorders, lower PsS scores were highly associated with worse neurocognitive measures, particularly the parent-reported Behavior Rating Inventory of Executive Function composite (r = -0.66, P < .001) and Parent Conners' attention deficit-hyperactivity disorder Index T score (r = -0.54, P < .001), whereas associations of PhS scores with neurocognitive measures were weaker. CONCLUSIONS: Psychosocial health status in adolescents with TOF without genetic disorders was worse than in healthy referents without risk factors for brain injury but similar to a normative sample; physical health status was worse in these adolescents than in either comparison group. Within these subjects with TOF, worse psychosocial health status was most highly associated with concurrent executive dysfunction and attention deficit-hyperactivity disorder. Optimizing HRQoL constitutes another indication for attention to neurodevelopment in children with congenital heart disease.

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