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Problem: West Virginia perennially leads the US in child obesity and adult heart disease. We had screened over 100,000 school children for cardiometabolic risk factors (CMRF) in the Coronary Artery Risk Detection in Appalachian Communities (CARDIAC) program over 20 years until the 2017 economic downturn. 60,000 had lipid panels. WVU Health System includes encounters with over 200,000 WV children. We hypothesize that CMRF screening has decreased without the statewide CARDIAC Program. Methods: We queried our instance of Epic Electronic Health Record that includes much of northern WV to ascertain BMI, Total Cholesterol (TC) , Low Density Lipoprotein Cholesterol (LDL), triglyceride level (TG) and hemoglobin A1C. We also searched for use of cholesterol-lowering medicines. Results: 137,566 children under 18 years had a BMI measure and we have reported a 5%ile increase in the COVID era. Only 8498 have had a total cholesterol check (avg ± SD 159+34 mg/dl), 9964 had triglyceride (109 ± 74 mg/dl) and 4874 had LDL (92.04 ± 29.8 mg/dl). 2609 children had average HA1C 6.03 ± 1.7%. 607 children had LDL ≥ 130 mg/dl including 174 with LDL ≥ 160 mg/dl. 33 were given lipid-lowering medication and had LDL change from 248 ± 86 mg/dl to 181 ± 42 mg/dl at follow-up. Average decrease was 67 ± 79 mg/dl (p < 0.0001). Conclusion: Few children are being screened for TC, LDL and TG absent the statewide CARDIAC lipid screening program in WV. Lipid-lowering medicines are sparsely used in children with significant risk of Familial Hypercholesterolemia (FH). Family history is not available for the cohort. None in FH range were below the 130 mg/dl LDL target value after treatment. In this pediatric population with usual incidence of FH and high prevalence of dyslipidemia of obesity with high BMI and TG levels, little lipid screening is occurring without the statewide school-based program. Child health care providers need to intervene to prevent even greater WV coronary disease in the future. Policy-makers can impact the future health of WV school children by promoting and funding screening for cardiometabolic risk factors now.

Background The United States, and especially West Virginia, have a tremendous burden of coronary artery disease (CAD). Undiagnosed familial hypercholesterolemia (FH) is an important factor for CAD in the U.S. Identification of a CAD phenotype is an initial step to find families with FH. Objective We hypothesized that a CAD phenotype detection algorithm that uses discrete data elements from electronic health records (EHRs) can be validated from EHR information housed in a data repository. Methods We developed an algorithm to detect a CAD phenotype which searched through discrete data elements, such as diagnosis, problem lists, medical history, billing, and procedure (International Classification of Diseases [ICD]-9/10 and Current Procedural Terminology [CPT]) codes. The algorithm was applied to two cohorts of 500 patients, each with varying characteristics. The second (younger) cohort consisted of parents from a school child screening program. We then determined which patients had CAD by systematic, blinded review of EHRs. Following this, we revised the algorithm by refining the acceptable diagnoses and procedures. We ran the second algorithm on the same cohorts and determined the accuracy of the modification. Results CAD phenotype Algorithm I was 89.6% accurate, 94.6% sensitive, and 85.6% specific for group 1. After revising the algorithm (denoted CAD Algorithm II) and applying it to the same groups 1 and 2, sensitivity 98.2%, specificity 87.8%, and accuracy 92.4; accuracy 93% for group 2. Group 1 F1 score was 92.4%. Specific ICD-10 and CPT codes such as “coronary angiography through a vein graft” were more useful than generic terms. Conclusion We have created an algorithm, CAD Algorithm II, that detects CAD on a large scale with high accuracy and sensitivity (recall). It has proven useful among varied patient populations. Use of this algorithm can extend to monitor a registry of patients in an EHR and/or to identify a group such as those with likely FH.

Introduction: The CARDIAC project surveyed cardiometabolic risk and risk for familial hypercholesterolemia (FH) through a school-based program, demonstrating the importance of universal blood cholesterol screening in children. At most, 10% of FH is diagnosed in the United States. Hypothesis: We hypothesize a disconnect between identification of risk factors and the follow-up care received by those children and young adults. Methods: Between 1998 and 2016, 60,404 5th grade children had their LDL-C levels assessed through venipuncture lipid panels. Of the 638 children who had an LDL-C ≥ 160mg/dl, indicative of probable FH, 395 were identified through EPIC EHR, phone calls, and mail surveys. WVU IRB approved verbal consent by participants. Information on their current status was obtained including medical care for their cholesterol, lipid-lowering medications (LLM), other medications, and family history of cardiac-related events. Results: Out of the 395 children identified, 174 were male and 221 were female. We had follow-up lipid panels for 58. Eight participants on a LLM had a follow-up LDL-C that was 57.5 +/- 50.1 mg/dl lower than the 5th grade level. Fifty subjects not on a LLM had an LDL-C that was 27.2 +//- 48.0 mg/dl lower than baseline. No follow-up LDL-C data was available on 33 participants who received a LLM. While only 41 children had ever taken a LLM, 56 participants were taking oral contraceptive, and 63 a psychotropic medicine. Only 1 participant was enrolled in the FH Registry. No subject was found with major adverse coronary event. Conclusion: Follow-up after screening demonstrated improved lipid levels in several subjects without known medication. Many subjects were receiving oral contraceptive agents and psychotropic medicines associated with dyslipidemia, usually without follow-up lipid panels. Roughly one-third of participants had unknown mid-term course. Epic EHR for WVU Health System found 393 subjects. The inability to document ongoing medical care in addition to demonstrated lack of care received by those who had an LDL greater than 160mg/dl shows that there is insufficient follow-up care for young people with probable FH. In conclusion, the data points to areas to improve, including better education for patients with this cardiometabolic risk factor. Education of health care professionals, telemedicine, patient-driven advocacy groups, EHRs that cover large catchments, and patient communication portals promise to better focus ongoing care for persons with abnormal lipid profiles.

Objective: Patients with successfully repaired coarctation of the aorta (CoA) need continued follow-up due to risks of restenosis and abnormal arterial compliance, causing early onset hypertension/coronary artery disease. We hypothesize that subtle hemodynamic and structural abnormalities can be predicted in the clinic by an abnormal arm-leg blood pressure (BP) gradient (i.e. arm > leg systolic BP). Design: Uncomplicated CoA patients repaired between 1990 and 2007 with follow-up clinic visits documenting right arm and leg systolic BPs, along with a recent echocardiogram, were studied. Data included the Doppler peak instantaneous (PeakV) and mean velocities (MeanV) in the descending aorta along with measures of the proximal transverse arch (TA) diameter and left ventricular wall (LVPWd) thickness. Measurements were indexed by Z-scores. Patients were grouped by higher systolic BP in the leg (Group 1) or arm (Group 2). Results: Eighty-one patients met the criteria with 52 in Group 1 (median 12.5 years follow-up) and 29 in Group 2 (median 12 years follow-up). Group 2 group had significantly increased arm systolic BP Z-scores (p

We provide guidance for conducting clinical trials with Indigenous children in the United States. We drew on extant literature and our experience to describe 3 best practices for the ethical and effective conduct of clinical trials with Indigenous children. Case examples of pediatric research conducted with American Indian, Alaska Native, and Native Hawaiian communities are provided to illustrate these practices. Ethical and effective clinical trials with Indigenous children require early and sustained community engagement, building capacity for Indigenous research, and supporting community oversight and ownership of research. Effective engagement requires equity, trust, shared interests, and mutual benefit among partners over time. Capacity building should prioritize developing Indigenous researchers. Supporting community oversight and ownership of research means that investigators should plan for data-sharing agreements, return or destruction of data, and multiple regulatory approvals. Indigenous children must be included in clinical trials to reduce health disparities and improve health outcomes in these pediatric populations. Establishment of the Environmental Influences on Child Health Outcomes Institutional Development Award States Pediatric Clinical Trials Network (ECHO ISPCTN) in 2016 creates a unique and timely opportunity to increase Indigenous children’s participation in state-of-the-art clinical trials.

Introduction: Research capacity building is a critical component of professional development for pediatrician scientists, yet this process has been elusive in the literature. The ECHO IDeA States Pediatric Clinical Trials Network (ISPCTN) seeks to implement pediatric trials across medically underserved and rural populations. A key component of achieving this objective is building pediatric research capacity, including enhancement of infrastructure and faculty development. This article presents findings from a site assessment inventory completed during the initial year of the ISPCTN.Methods: An assessment inventory was developed for surveying ISPCTN sites. The inventory captured site-level activities designed to increase clinical trial research capacity for pediatrician scientists and team members. The inventory findings were utilized by the ISPCTN Data Coordinating and Operations Center to construct training modules covering 3 broad domains: Faculty/coordinator development; Infrastructure; Trials/Research concept development.Results: Key lessons learned reveal substantial participation in the training modules, the importance of an inventory to guide the development of trainings, and recognizing local barriers to clinical trials research.Conclusions: Research networks that seek to implement successfully completed trials need to build capacity across and within the sites engaged. Our findings indicate that building research capacity is a multi-faceted endeavor, but likely necessary for sustainability of a unique network addressing high impact pediatric health problems. The ISPCTN emphasis on building and enhancing site capacity, including pediatrician scientists and team members, is critical to successful trial implementation/completion and the production of findings that enhance the lives of children and families.

Modern machine learning techniques (such as deep learning) offer immense opportunities in the field of human biological aging research. Aging is a complex process, experienced by all living organisms. While traditional machine learning and data mining approaches are still popular in aging research, they typically need feature engineering or feature extraction for robust performance. Explicit feature engineering represents a major challenge, as it requires significant domain knowledge. The latest advances in deep learning provide a paradigm shift in eliciting meaningful knowledge from complex data without performing explicit feature engineering. In this article, we review the recent literature on applying deep learning in biological age estimation. We consider the current data modalities that have been used to study aging and the deep learning architectures that have been applied. We identify four broad classes of measures to quantify the performance of algorithms for biological age estimation and based on these evaluate the current approaches. The paper concludes with a brief discussion on possible future directions in biological aging research using deep learning. This study has significant potentials for improving our understanding of the health status of individuals, for instance, based on their physical activities, blood samples and body shapes. Thus, the results of the study could have implications in different health care settings, from palliative care to public health.

BACKGROUND: The Coronary Artery Risk Detection in Appalachian Communities (CARDIAC) Project is a state-wide risk factor screening program that operated in West Virginia for 19 years and screened more than 100,000 5th graders for obesity, hypertension, and dyslipidemia. OBJECTIVES: We investigated siblings in the CARDIAC Project to assess whether cardiometabolic risk factors (CMRFs) correlate in siblings. METHODS: We identified 12,053 children from 5752 families with lipid panel, blood pressure, and anthropometric data. A linkage application (LinkPlus from the U.S. Centers for Disease Control and Prevention) matched siblings based on parent names, addresses, telephone numbers, and school to generate a linkage probability curve. Graphical and statistical analyses demonstrate the relationships between CMRFs in siblings. RESULTS: Siblings showed moderate intraclass correlation coefficient of 0.375 for low-density lipoprotein cholesterol (LDL-C), 0.34 for high-density lipoprotein cholesterol (HDL-C), and 0.22 for triglyceride levels. The body mass index (BMI) intraclass correlation coefficient (0.383) is slightly better (2%) than LDL-C or HDL-C, but the standardized beta values from linear regression suggest a 3-fold impact of sibling LDL-C over the child’s own BMI. The odds ratio of a second sibling having LDL-C < 110 mg/dL with a first sibling at that level is 3.444:1 (Confidence Limit 3.031–3.915, P < .05). The odds ratio of a sibling showing an LDL-C ≥ 160 mg/dL, given a first sibling with that degree of elevated LDL-C is 29.6:1 (95% Confidence Limit: 15.54–56.36). The individual LDL-C level correlated more strongly with sibling LDL-C than with the individual’s own BMI. Seventy-eight children with LDL-C > 160 mg/dL and negative family history would have been missed, which represents more than half of those with LDL-C > 160 mg/dL (78 vs 67 or 54%). CONCLUSIONS: Sibling HDL-C levels, LDL-C levels, and BMIs correlate within a family. Triglyceride and blood pressure levels are less well correlated. The identified CMRF relationships strengthen the main findings of the overall CARDIAC Project: an elevated BMI is not predictive of elevated LDL-C and family history of coronary artery disease poorly predicts cholesterol abnormality at screening. Family history does not adequately identify children who should be screened for cholesterol abnormality. Elevated LDL-C (>160 mg/dL) in a child strongly suggests that additional siblings and parents be screened if universal screening is not practiced.

BACKGROUND The Coronary Artery Risk Detection in Appalachian Communities (CARDIAC) Project is a chronic disease risk factor surveillance, intervention, and research initiative aimed at combating the unacceptably high prevalence of heart disease, diabetes, and other chronic illnesses in West Virginia. OBJECTIVES AND METHODS The school-based public health project identifies health risk factors in children, educates families, informs primary care physicians, and provides resources to schools to help improve population health, beginning with children. RESULTS AND CONCLUSION Details regarding methodology, results, and conclusions derived from this unique public health initiative that has screened over 200,000 children are the subject of this 18- year review.

Short stature is associated with increased LDL-cholesterol levels and coronary artery disease in adults. We investigated the relationship of stature to LDL levels in children in the West Virginia Coronary Artery Risk Detection in Appalachian Communities (CARDIAC) Project to determine whether the genetically determined inverse relationship observed in adults would be evident in fifth graders. A cross-sectional survey of schoolchildren was assessed for cardiovascular risk factors. Data collected at school screenings over 18 years in WV schools were analyzed for 63,152 fifth-graders to determine relationship of LDL to stature with consideration of age, gender, and BMI. The first (shortest) quartile showed an LDL level of 93.6 mg/dl compared with an LDL level of 89.7 mg/dl for the fourth (tallest) quartile. Each incremental increase of 1 SD of height lowered LDL by 0.049 mg/dl (P < 0.0001). Multivariate analysis showed LDL to vary inversely as a function of the first (lowest) quartile of height after controlling for gender, median age, BMI percentile for age and gender, and year of screening. The odds ratio for LDL ≥ 130 mg/dl for shortest versus tallest quartile is 1.266 (95% CL 1.162–1.380). The odds ratio for LDL ≥ 160 mg/dl is 1.456 (95% CL 1.163–1.822). The relationship between short stature and LDL, noted in adults, is confirmed in childhood.

Introductoin The prevalence of childhood cardiovascular disease (CVD) risk factors often increases in more rural geographic regions in the USA. However, research on the topic often has conflicting results. Researchers note differences in definitions of rurality and other factors that would lead to differences in inference, including appropriate use of statistical clustering analysis, representative data, and inclusion of individual-level covariates. The present study's objective was to examine CVD risk factors during childhood by geographic distribution in the US Appalachian region as a first step towards understanding the health disparities in this area. Methods Rurality and CVD risk factors (including blood pressure, body-mass index (BMI), and cholesterol) were examined in a large, representative sample of fifth-grade students (N=73 014) from an Appalachian state in the USA. A six-category Rural-Urban Continuum Codes classification system was used to define rurality regions. Mixed modeling analysis was used to appropriately cluster individuals within 725 unique zip codes in each of these six regions, and allowed for including several individual-level socioeconomic factors as covariates. Results Rural areas had better outcomes for certain CVD risk factors (lowest low-density lipoprotein cholesterol (LDL-C), and blood pressure (BP) and highest high-density lipoprotein cholesterol (HDL-C)) whereas mid-sized metro and town areas presented with the worst CVD risk factors (highest BMI% above ideal, mean diastolic BP, LDL-C, total cholesterol, triglyceride levels and lowest HDL-C) outcomes in children and adolescence in this Appalachian state. Conclusions Counter to the study hypothesis, mid-sized metro areas presented with the worst CVD risk factors outcomes in children and adolescence in the Appalachian state. This data contradicts previous literature suggesting a straightforward link between rurality and cardiovascular risk factors. Future research should include a longitudinal design and explore some of the mechanisms between cardiovascular risk factors and rurality.

BACKGROUND: Few resources exist for prospective, longitudinal analysis of the relationships between early life environment and later obesity in large diverse samples of children in the United States (US). In 2016, the National Institutes of Health launched the Environmental influences on Child Health Outcomes (ECHO) program to investigate influences of environmental exposures on child health and development. We describe demographics and overweight and obesity prevalence in ECHO, and ECHO’s potential as a resource for understanding how early life environmental factors affect obesity risk. METHODS: In this cross-sectional study of 70 extant US and Puerto Rico cohorts, 2003–2017, we examined age, race/ethnicity, and sex in children with body mass index (BMI) data, including 28,507 full-term post-birth to

Introduction: Coronary Artery Risk Detection in Appalachian Communities is a school-based cross-sectional screening program to detect significant cardiometabolic disease risk factors (CMRFs) in WV children. Fifth graders have undergone school screening for family history, anthropometrics and lipid panel values since 1999 including screening for Acanthosis Nigricans (AN Pos or Neg). Our hypothesis is that presence of AN can predict need to screen for CMRFs. Methods: Parental consent and child assent were obtained before screening day using an IRB-approved protocol. Height and weight were used to calculate BMI and BMI%ile. Systolic blood pressure over 95%ile, triglyceride over 150 mg/dl, HDL under 40 mg/dl and BMI over 95%ile were tabulated and correlated. Glucose and HOMA IR were only checked for AN Pos subjects and thus not considered in analysis. Results: 102,930 5 th graders participated in CARDIAC up to 2017. 101,101 had BMI determination, 100,000 blood pressures, 67,710 lipid panels were checked. 27.9% of children (28715) were obese, defined as BMI over 95%ile and 18.4% (18,931 subjects) were overweight. 10.8% of AN pos children vs. 1.9% of all children exhibited 4 CMRFs (Obese BMI, Htn, high TG, low HDL, OR 7.31, 95% CL 6.4-8.35). Conclusion: AN correlated to CMRF clustering but many CMRF pos subjects were AN Neg (79.5% or 1236/1555 of those with 4 CMRFs). Multifactoral screening of lipids, BMI, blood pressure are indicated to identify risk for cardiovascular disease.

BACKGROUND: In West Virginia (WV), 47% of fifth-grade children are either overweight or obese. There is no clear consensus regarding the definition of insulin resistance in children, and directly measuring insulin on the population level is costly. Two proposed measures examined further in this study include triglyceride (TRIG)/high-density lipoprotein cholesterol (HDL-C) ratio and TRIG/low-density lipoprotein (LDL-C) ratio. The purpose of this study is to examine the relationship between TRIG/HDL-C ratio, TRIG/LDL-C ratio and insulin resistance in fifth-graders with acanthosis nigricans (AN). METHODS: Between 2007 and 2016, 52,545 fifth-grade students in WV were assessed for AN. Fasting glucose and insulin levels were collected only for a sub-group of students who were AN-positive and was used to determine insulin resistance using the Homeostatic Model for Insulin Resistance (HOMA-IR) equation. Statistical analysis included t tests and logistic regression with receiver operating characteristic curves. RESULTS: Of the students assessed for AN, 4.5% (n = 2360) tested positive. The prevalence of insulin resistance was 79% (n = 814) among 1030 with AN and complete HOMA-IR. TRIG/HDL-C ratio and TRIG/LDL-C ratio were significantly associated with insulin resistance (TRIG/HDL-C:Est. = 0.36, P < 0.0001, AUC = 0.68; TRIG/LDL-C: Est. = 0.87, P < 0.0001, AUC = 0.69). Multivariate analysis showed that increased body mass index (Est. = 0.05, P < 0.0001), gender (Est. = 0.49, p < 0.0001) and TRIG/HDL-C ratio (Est. = 0.21, P < 0.0001) were significantly associated with insulin resistance. CONCLUSIONS: TRIG/HDL-C is a better surrogate marker of insulin resistance in AN-positive children compared to TRIG/LDL-C ratio; so, on a population-level, cholesterol rather than insulin may be obtained for preliminary testing of early insulin resistance in children.

Introduction: Electronic Health Records (EHRs) benefit record keeping, information collation, error prevention, and charge capture. They provide a large database of clinical information that can be used for research. Sorting vast amounts of data manually is inefficient, hence, an effectual, validated method is required to uncover information from large sets of data and generate knowledge. The U.S., and especially West Virginia, has a tremendous burden of cardiovascular disease (CVD). Undiagnosed Familial Hypercholesterolemia (FH) is an important factor for CVD in the U.S. FH results in elevated levels of LDL from childhood and early atherosclerotic disease. We are interested in better screening processes for FH. One method is to detect adults with coronary artery disease (CAD) and determine if their lipid levels are indicative of FH. Relatives and children can then be screened for FH and treated. Efficient identification of a CAD phenotype from EHRs is an important initial step in this screening process. Hypothesis: We hypothesized that a CAD phenotype detection algorithm that uses discrete data elements from EHRs can be validated as a precursor to detection of FH. Methods: We developed an algorithm to detect a CAD phenotype, which searched through discrete data elements, such as diagnoses lists (ICD-10) and procedure (CPT) codes. Direct inspection of EHR discrete data avoided the need for artificial intelligence, such as natural language processing. The algorithm was applied to a cohort of 1,000 patients with varying characteristics. We then determined which patients had CAD by systematically going through EHRs. Following this, we revised the algorithm by refining the constraints under which it operated. We ran the algorithm again on the same 1,000 patients, and determined the accuracy of the modified algorithm. Results: Manual validation of the 1,000 patients resulted in 413 with CAD and 587 without. The original algorithm distinguished 488 CAD positive patients and 512 CAD negative patients. This was 89% accurate, 96% sensitive, and 85% specific. After revising the algorithm and applying it to the same cohort, it determined that there were 474 CAD patients and 526 without CAD. This was 93% accurate, 99% sensitive, and 89% specific. Conclusion: EHR usage has created a large pool of minable clinical data. However, without an efficient method to obtain inferences from it, the information cannot be effectually utilized. We have created an algorithm that detects CAD on a large scale with high accuracy. It has proven to be useful among a varied patient population. Since the constraints that are used, such as ICD codes and CPT codes, are universal, it can be utilized across many hospital systems; although, local validation is prudent. Using this algorithm can select a population with a propensity for FH, thereby allowing us to screen and manage patients with undiagnosed FH or other familial dyslipidemias.

Introduction: West Virginia exhibits pervasive cardiovascular disease (CVD) that may relate to a combination of ancestry and shared environment in families, including vulnerabilities related to diet, physical activity and tobacco use. Coronary Artery Risk Detection in Appalachian Communities (CARDIAC) is a school-based child risk factor screening program that has evaluated over 90,000 WV fifth graders in the past 20 years. Reverse Cascade screening for Familial Hypercholesterolemia (FH) has been difficult with the CARDIAC population. The WVU CTSI Integrated Data Repository (IDR) includes over 2 million records. Hypothesis: Linkage of child CARDIAC data to parent IDR data will allow new information discovery to inform management of CVD. Methods: We used direct demographic data linkage via Oracle with Soundex conversion of names, in the IDR, to find parents of the CARDIAC participants. Data was analyzed in the VMWare SSL environment. Results: 4759 children have a parent(s) identified. 959 mothers and 524 fathers have an LDL level from IDR. Race, BMI and gender was recorded from CARDIAC. 6.8 % of children, 40% of mothers and 44.8% of fathers have an abnormal LDL level >130 mg/dl in IDR. Positive predictive value of the abnormal child lipid level (≥130 mg/dl) is 17% for some parent (56/325) to be abnormal. 4 parents had LDL >190 mg/dl with child > 160, indicating likely FH in the pair (2.7% of pairs or 1 in 371 pairs). Conclusion: Formation of a virtual cohort of CARDIAC children and parents allows Virtual Reverse Cascade Screening to find FH. This project highlights the importance of familial tendency to hyperlipidemia that can aid detection of early lipid abnormality and cardiovascular risk in children and their young parents to promote wellness and potentially avoid early coronary artery disease. We are constructing a virtual longitudinal cohort to study CVD in WV as a part of a Learning Health System in which data management is at the forefront of healthcare improvement.

Objective As the adult congenital heart disease population increases, poor transition from pediatric to adult care can lead to suboptimal quality of care and an increase in individual and institutional costs. In 2008, the American College of Cardiology and American Heart Association updated the adult congenital heart disease practice guidelines and in 2011, the American Heart Association recommended transition guidelines to standardize and encourage appropriate timing of transition to adult cardiac services. The objective of this study was to evaluate if patient age or complexity of congenital heart disease influences pediatric cardiologists’ decision to transfer care to adult providers and to evaluate the compliance of different types of cardiology providers with current adult congenital heart disease treatment guidelines. Design A single-center retrospective review of 991 adult congenital heart disease patients identified by ICD-9 code from 2010 to 2012. Setting Academic and community outpatient cardiology clinics. Patients Nine hundred ninety-one patients who are 18 years and older with congenital heart disease. Intervention None. Outcomes measures The compliance with health maintenance and transfer of care recommendations in the outpatient setting. Results For patients seen by pediatric cardiologists, only 20% had transfer of care discussions documented, most often in younger simple patients. Significant differences in compliance with preventative health guidelines were found between cardiology provider types. Conclusion Even though a significant number of adults with congenital heart disease are lost to appropriate follow-up in their third and fourth decades of life, pediatric cardiologists discussed transfer of care with moderate and complex congenital heart disease patients less frequently. Appropriate transfer of adults with congenital heart disease to an adult congenital cardiologist provides an opportunity to reinforce the importance of regular follow-up in adulthood and may improve outcomes as adult congenital cardiologists followed the adult congenital heart disease guidelines more consistently than pediatric or adult cardiologists.

Introduction: The Coronary Artery Risk Detection in Appalachian Communities (CARDIAC) Project gathers anthropometric, BP and lipid data from fifth graders in West Virginia in the past 18 y. 60,403 children had LDL cholesterol and we found 5259 sets of siblings by direct match on mothers first and last name. The suggestion that more sibships could be identified prompted evaluation of Link Plus software from Centers for Disease Control (CDC) to improve matching. Methods: LinkPlus generates potential matches via a probabilistic algorithm that allows relative weighting of multiple factors such as first and last name. For our purposes the deduplication rather than matching algorithm was run using mother’s first and last name using the NYSIIS (New York State Identification and Intelligence System) phonetic schema to avoid creating multiple many-to-many relationships that were difficult to analyze. Additional variables considered included county, street address, telephone, fathers first and last name, school. Subject last name was used as a blocking variable. Results: 7602 matched siblings were generated by the program that determined a probability score ranging from 61.3 to cut off at 15; few matches were observed below this level. The figure demonstrates exponential decay beginning at a probability score of 26 with 95% accuracy at 25.5. 6827 pairs were included at that level including 6824 matched pairs and only 3 false positive pairs. Partial matches (n = 61) likely are half sibs including exact match of telephone and/or street but only one parent matching. Child surname was not used in the algorithm. Typographical errors were accounted by Link Plus. Lipid correlations were similar to those found with excel but more robust. Conclusion: The Link Plus record matching program from CDC is able to successfully determine sibships with increased sensitivity compared with a direct match from a sorted excel file. The program was able to identify likely sibs and half-sibs plus avoid non-match due to minor typo errors in the analyzed fields.

Familial hypercholesterolemia (FH) is an important, treatable cause of elevated LDL cholesterol that can be remediated to lower risk of premature coronary artery disease. Strategies for identification include universal screening, cascade screening of relatives of persons found with either early cardiovascular disease (CVD) or LDL-C consistent with FH, and big data approaches to identify persons with high cholesterol and premature CVD. Universal screening of children is advocated to find FH at an early age with still asymptomatic parents and negative family history.

A system for collection, distribution, and long distant, asynchronous interpretation of cardiac auscultation has been developed and field-tested in rural China. We initiated a proof-of-concept test as a critical component of design of a system to allow rural physicians with little experience in evaluation of congenital heart disease (CHD) to obtain assistance in diagnosis and management of children with significant heart disease. The project tested the hypothesis that acceptable screening of heart murmurs could be accomplished using a digital stethoscope and internet cloud transmittal to deliver phonocardiograms to an experienced observer. Of the 7993 children who underwent school-based screening in the Menghai District of Yunnan Province, Peoples Republic of China, 149 had a murmur noted by a screener. They had digital heart sounds and phonocardiograms collected with the HeartLink tele auscultation system, and underwent echocardiography by a cardiology resident from the First Affiliated Hospital of Kunming Medical University. The digital phonocardiograms, stored on a cloud server, were later remotely reviewed by a board-certified American pediatric cardiologist. Fourteen of these subjects were found to have CHD confirmed by echocardiogram. Using the HeartLink system, the pediatric cardiologist identified 11 of the 14 subjects with pathological murmurs, and missed three subjects with atrial septal defects, which were incorrectly identified as venous hum or Still's murmur. In addition, ten subjects were recorded as having pathological murmurs, when no CHD was confirmed by echocardiography during the field study. The overall test accuracy was 91% with 78.5% sensitivity and 92.6% specificity. This proof-of-concept study demonstrated the feasibility of differentiating pathologic murmurs due to CHD from normal functional heart murmurs with the HeartLink system. This field study is an initial step to develop a cost-effective CHD screening strategy in low-resource settings with a shortage of trained medical professionals and pediatric heart programs.

* Abbreviations: AAP — : American Academy of Pediatrics CSHCN — : children with special health care needs EHR — : electronic health record EIF — : emergency information form PHR — : personal health record In the current issue of Pediatrics , an innovative article by Abraham et al1, “Impact of emergency information forms for children with medical complexity: a simulation study” presents an excellent example of the use of emergency scenarios to study otherwise difficult-to-observe events in emergency and critical care; specifically, emergencies of medically complex children. The emergency information form (EIF) organizes emergency-focused medical information for children with special health care needs (CSHCN). Before the advent of electronic health records (EHRs) in the 1990s, the EIF began as sets of wallet cards promoted by state Emergency Medical Services for Children Programs in New Mexico and Ohio that listed medications and diagnoses. This was carried forward by Sacchetti and Gerardi who created a concise 1-page paper summary with a unique added focus of an advice section that listed a child’s special problem, emergencies likely to arise from this problem, and recommended treatments.2 In the initial joint American Academy of Pediatrics (AAP) and American College of Emergency Physician Committee on Pediatric Emergency Medicine policy statement, “Emergency Preparedness for CSHCN”, it is recommended that primary and specialty caregivers join with the … Address correspondence to Lee. A. Pyles, MD, MS, Department of Pediatrics, West Virginia University School of Medicine, 1 Medical Center Dr, Box 9214, Morgantown, WV 26506. E-mail: lpyles{at}hsc.wvu.edu

Introduction: Coronary Artery Risk Detection in Appalachian Communities (CARDIAC) is a project that has assessed childhood risk factors for cardiovascular disease (CVD) for nearly twenty years. We have screened approximately 95,000 children from WV that now include 60,403 with lipid panels. Eight-thousand-five-hundred-fifty-seven persons have one or more siblings who have participated in the project. To begin to consider genetic primordial antecedents of CVD in WV schoolchildren, we linked sibships for analysis. Methods: Linkage software designed to deduplicate EHRs proved poorly adaptable to the problem of family matching. Successive excel spreadsheets were used to directly match either mother or father’s first and last name with care taken to avoid similar names by requiring additional matching parameters. A group of children were identified with younger siblings subsequently enrolled in CARDIAC and the two older children were matched for analysis when 3 siblings were found. Body Mass Index z-score was calculated for each child. Fasting lipid levels were obtained by venipuncture and measured by a commercial laboratory (LabCorp). Results: Children (5259)with at least one sibling with LDL levels available for both were found along with 1384 third siblings. The mean LDL level for the first child was 90.5 mg/dl and 92.1 mg/dl for the second child. The mean BMI z-score was +0.83 for the group of 95,000 children, +0.73 for the first sibling and +0.72 for the second sibling. Sibling BMI z-scores were strongly correlated. Multivariate analysis demonstrated that the LDL level for the second child correlated strongly with both the older sibling’s LDL level and with the second child’s BMI z-score. This model showed R2 = 0.15 and the following prediction equation: LDLsibling2 = 56.3 + 0.377*LDL-proband + 2.24*BMI-z-score-sibling2 p = 4.33 x 10 -175 for LDL and p = 4.06 x 10 -15 for BMI z-score Six-hundred-sixty-eight children had an LDL level greater than 130 mg/dl including 88 sets of siblings. 12 pairs of siblings each had an LDL level > 160 mg/dl. Conclusion: the analysis demonstrates a strong correlation between sibling LDL-c levels that is not as well explained by the correlation of BMI z-score as by LDL-c. This analysis along with our recent study of LDL and height will help to characterize CVD risk in this population to focus future investigations in children and families.

Advances in palliation of congenital heart disease have resulted in improved survival to adulthood. Many of these patients ultimately develop end-stage heart failure requiring left ventricular assist device implantation (LVAD). However, morphologic differences in the systemic ventricle of these patients require careful attention to cannula placement. We report on the evolution of our surgical technique for implanting LVADs in 3 patients with transposition of the great arteries and congenitally corrected transposition of the great arteries. Applying standard LV cannulation techniques to the systemic ventricle led us too anteriorly in our first patient, creating obstruction by the moderator band. Subsequent use of epicardial and transesophageal echocardiography allowed for intraoperative localization of the intracardiac muscular structures to identify the optimal cannulation site. The acute angle of the inflow cannula on the DeBakey LVAD (MicroMed Technology, Houston, TX) required flipping the device 180°. The HeartMate II device (Thoratec, Pleasanton, CA) could be shifted towards the midline. One patient underwent successful transplant and 2 are home waiting for a donor organ. We conclude from our experience that LVAD surgery can be safely performed in patients with congenital heart disease when implanted under echocardiographic guidance.

Extracorporeal membrane oxygenation (ECMO) is routinely used to support cardiopulmonary failure in infants and children. Suboptimal outcomes for primary cardiac support suggest a need for investigation of the impact of ECMO on the heart. Twenty-four newborn lambs received a brief period of ECMO support to investigate the hypothesis that ECMO produces cardiac dysfunction in newborn lamb. Dorset newborn lambs, 4–7 days of age, were exposed to ECMO for 5 min at a 100 ml/kg flow rate and quickly weaned off. Measurements included echocardiographic mean left ventricular (LV) velocity of circumferential fiber shortening corrected for heart rate (mVCFc), LV shortening fraction, and peak systolic wall stress plus hemodynamic measurement of LV maximum rate of pressure change with time (LV dp/dt max), maximum rate of pressure change divided by developed pressure (LV dp/dtP), right atrial pressure, pulmonary capillary wedge pressure, mean pulmonary artery pressure, LV peak and end-diastolic pressure, and aortic pressure. These measures were also obtained after an exposure to 5 min of ECMO and immediate disconnect for 5 min, followed by ECMO administration for 1 h again, followed by discontinuation of ECMO. LV mVCFc is decreased after exposure to 5 min of ECMO support despite a decrease in LV peak systolic wall stress that provides afterload reduction. LV mVCFc is inversely related to peak systolic wall stress at a significance level of p < 0.0001. The time period after initiation of ECMO is a significant factor in the model (p = 0.0097). Time [baseline] was different from the other time points with p = 0.0010. Average mVCFc at baseline is 1.27 ± 0.35 and decreases to 1.01 ± 0.42 after 5 min of ECMO that is then withdrawn. Peak systolic wall stress decreases from 36.0 ± 13.1 at baseline to 29.8 ± 12.1 after 5 min of ECMO. LV dp/dt max decreases from 1,769 ± 453 mmHg/s at baseline to 1,311 ± 513 mmHg/s after exposure to 5 min of ECMO (p = 0.0005). Baseline LV dp/dt max is different from each point after start of ECMO. Diastolic LVdp/dt min increased from −1,340 ± 477 mmHg/s to −908 ± 393 mmHg/s at 5 min. Echocardiographic mVCFc, when considered in isolation or as a function of LV peak systolic wall stress, shows diminished LV function after ECMO. Hemodynamic measurement of LV dp/dt max and LV dp/dt min confirms the observation. Separation of the humoral from mechanical effect of ECMO with the short exposure to the extracorporeal circuit shows that an immediate decrement of LV function occurs at initiation of ECMO, a finding that has not been stressed with previous studies of extracorporeal support. This implies a potentially outcome-limiting deleterious effect for the patient who requires ECMO support for the heart rather than the lungs. We should continue to strive to understand and ameliorate this deleterious effect of the extracorporeal circulation circuit.

The pulmonary atresia and intact ventricular septum (PAIVS) spectrum usually includes very hypoplastic right ventricles (RVs) and tricuspid valves (TVs) and often RV-coronary artery connections (RV-CACs). These abnormalities are often thought irreversible and typically only 30% reach a 2-ventricle repair (2VR). Our hypothesis, however, has been PAIVS is a developmental defect and catch-up growth is possible in all. To answer this question, we attempted to obtain up to 10year echo data on RV and TV growth and function in the 24 survivors (of 28 patients) from 1989 to 1999. To maximize the validity of the growth results obtained, we used only paired studies and required biplane echos to accurately estimate RV volumes, TV size, and function. Measurements were indexed to determine the z score (standard deviation of the mean from predicted). All patients had an RV outflow patch and ASD reduction. We have found catch-up growth required complete relief of RV obstruction and a mildly restrictive (3–5mmHg) ASD to encourage TV flow. A central shunt was placed in 38%. RV-CACs (2.7±1.6/pt) were uneventfully ligated off bypass in 9/28 (32%). For the 24 survivors, 19 adequate paired biplane studies were obtained from here and elsewhere and for 12 allowed a 10year follow-up. The RV volume z scores grew from −5.1±2.5 to 0.9±1.9 about 10years later. The TVs, even with increased flow early, grew more slowly than the RVs. Nevertheless, the very small TVs in 10 pts went from z scores of −4.6±0.4 to −0.04±−1.5 within about 5–10years. Valvotomies were done in 3. RV function was high normal (EF=74.5±0.1%) presumably because of pulmonary regurgitation in the 8 pts who had adequate studies. We conclude: (1) Catch-up growth appears reliable in PAIVS pts when RV obstruction is relieved and TV flow is increased (2) RV function was good and resembled well-repaired TOF (3) These results encourage pursuing 2VRs in PAIVS patients.

Pulmonary atresia and intact ventricular septum (PAIVS), more than most congenital cardiac defects, requires detailed assessment for initial decision making and to guide intraoperative procedures. Among the important questions to answer are the degree of tricuspid valve (TV) and right ventricular (RV) hypoplasia and whether or not significant connections exist between the coronary arteries and the right ventricle (RV-CACs). When the TV and RV hypoplasia are severe and/or significant RV-CACs exist, these lesions are thought to preclude a two-ventricle repair (2VR) at most institutions. Our approach, however, is that these lesions can be defined and successfully treated making an adequate 2VR probable. We have developed and successfully employed a combined echocardiographic and angiographic approach for neonates at the severe end of the PAIVS spectrum and particularly those with RV-CACs that has guided and allowed 2VRs in these infants. Our approach includes preoperative, intraoperative and postoperative echocardiography including transthoracic, transesophageal (TEE) and cardiac surface imaging. The echocardiographic studies are used to define the TV and RV abnormalities and determine if RV-CACs are present. Significant RV-CACs are indicated by bidirectional flow in the RV-CAC with associated coronary flow reversal and dilated major coronary arteries with abrupt caliber changes at the site of the connection. When significant RV-CACs are present, cardiac catheterization with complementary right ventricular and aortic root angiography from similar projections aids localization of the connections as well as the associated coronary stenoses and interruptions which seem to result from hypertensive, hypoxic perfusion. The combined use of these techniques allows preoperative localization of significant RV-CACs while the use of intraoperative transesophageal and epicardial surface echocardiography aids in their ligation. Postoperatively, identification of any regional wall motion abnormality by TEE, should prompt a search for previously undetected or residual coronary artery connections. This detailed diagnostic approach to the evaluation and treatment of PAIVS will even allow infants at the severe end of the spectrum to be on a 2VR track.

Pediatric Cardiac Care Consortium is a registry of cardiac catheterizations, surgical operations, and autopsies performed for infants, children, and adults with congenital heart disease. Four examples of use of PCCC data to evaluate variability in morphology, management, and outcomes for the procedures are described. Consideration is given to the following clinical problems: (1) the experience with surgical heart block in operative closure of perimembranous VSD, (2) the transition away from atrial baffle operations to the arterial switch operation for simple transposition of the great arteries, (3) the experience of planned 3 stage palliation of hypoplastic left heart syndrome, and (4) the identification of a high risk combination of cardiovascular anomalies in Williams syndrome. Analysis of registry outcomes allows ongoing quality improvement at a cardiac center to consider not only its own experience but that of the overall group. The PCCC data can be used to personalize management of rare congenital cardiac anomalies and combinations of anomalies. The PCCC registry allows longitudinal consideration of issues such as staged repairs and incidence of unplanned reoperation. In future years, the PCCC can facilitate investigations into the etiology of congenital heart disease.

Williams syndrome is a multifaceted disorder that includes a spectrum of cardiovascular anomalies. Due to its rare occurrence, outcome data for operations and cardiac catheterization are limited. We reviewed data from 242 individuals from the Pediatric Cardiac Care Consortium (PCCC) with Williams syndrome and associated cardiovascular lesions, and their frequency, and assessed their effects on mortality. In the PCCC, from 1984 to 1999 there were approximately 100,000 entries for cardiac procedures, involving more than 62,000 patients. The diagnosis of Williams syndrome was based on clinical features and determined by each site. Most patients were diagnosed with the availability of the FISH probe for region 7q11.23. Using a spreadsheet application, Microsoft Excel, the selected patients were analyzed for various types of cardiac anomalies. The most common cardiovascular lesions and the mortality rate in patients with Williams syndrome were examined. A complete tabulation of all cardiovascular lesions was assembled. There were 292 catheterizations and 143 operations reported to the PCCC. One hundred six patients had both an operation and a catheterization. The three main cardiovascular anomalies were supravalvular aortic stenosis (SVAS; 169), pulmonary artery stenosis (PAS; 130), and coarctation or aortic arch hypoplasia (Arch; 32). One hundred five patients had a single lesion, 70 with SVAS, 29 with PAS, and 6 with an arch anomaly. Ninety-two had more than one lesion: 80 with SVAS and PAS, 7 with PAS and Arch, and 5 with SVAS and Arch. Seventy individuals have only SVAS, 29 PAS, and 6 Arch alone. There was a total of 15 deaths. The mortality rate was highest in the group with the combination of SVAS and PAS (7 surgical and 5 catheter; 12 of 80 patients [15%]; p = 0.0001, chi(2)). In conclusion, our data represent the largest collection of individuals with Williams syndrome who underwent cardiac catheterization and/or operation. The data suggest that children with Williams syndrome and bilateral outflow tract obstruction have statistically and clinically significantly higher mortality associated with catheterization or operation.

Objective At the severe end of the spectrum of infants with pulmonary atresia and intact ventricular septum, the likelihood of significant right ventricle to coronary artery connections increases. Our purpose is to present the first series of right ventricle to coronary artery connections ligated off bypass before right ventricular decompression and to evaluate the consequences of this approach. Methods From 1988 to 2007, 19 patients with pulmonary atresia and intact ventricular septum had a total of 69 right ventricle to coronary artery connections identified preoperatively, and 10 more were located intraoperatively. Of these, 71 were judged large enough to warrant off-pump direct ligation. Preoperative diagnosis was by transthoracic echocardiography and angiography. Transesophageal and surface echocardiography were used for intraoperative location. Direct visualization and echocardiographic assessment for regional wall motion abnormalities determined the effects of ligation. Right ventricular decompression was done in all patients. Results After ligation, coronary flow converted from moderately or largely retrograde to antegrade pefusion. Ligation produced no visual myocardial consequences or immediate local wall motion abnormalities. For 3 patients, however, apical-septal wall motion abnormalities appeared from 2 hours to 3 days postoperatively. Serial studies were done to assess the later effects in the 16 of 19 30-day survivors. No evidence for myocardial injury was found, and all continued on a 2-ventricle repair course. Conclusion The location and ligation of right ventricle to coronary artery connections can be reliably accomplished off bypass. Coronary flow became antegrade, improving myocardial oxygenation. No myocardial damage was observed. Inapparent right ventricle to coronary artery connections occasionally enlarge secondarily after right ventricular decompression, making early follow-up evaluation necessary after ligation. Despite the initial presence of significant right ventricle to coronary artery connections, 2-ventricle repairs are possible with long-term benefits.

Introduction: Auscultation (Ausc) is used around the world to screen for congenital heart disease (CHD). We describe initial field testing of a cloud-based Ausc information management system in rural China school children with limited access to cardiac care . Our goal is to develop an economical system to facilitate access to cardiac care for underserved children in low- and middle-income countries. Hypothesis: Web/cloud based distant transmission of auscultation exam can facilitate screening for congenital heart disease. Methods: A team of cardiology staff from a tertiary hospital conducted field screening for CHD) in rural school children in Yunnan Province, People’s Republic of China. Each child with a murmur with traditional Ausc underwent echo screening and digital Ausc (Thinklabs, USA). Heart sounds were collected from 5 positions (URSB, ULSB, LLSB, apex and mid-LSB), and uploaded to a server using a tablet application (HeartLink System, USA). Blinded Ausc 3-5 months later was compared with echo finding at on-site screening. Results: 8000 children were screened with standard Ausc over 4 days by a team from Kunming Medical University and First University Hospital. Heart sounds were recorded for 149 subjects. The best vibratory quality of an innocent Still murmur was heard at LLSB in 52,at mid-LSB in 51, apex in 10 and URSB/ULSB in 4. Digital Ausc found pathologic murmurs in 11 of 14 subjects with echo-diagnosed CHD. Three missed were ASD called venous hum in 2 of 3; likely related to respiratory artifact at the URSB/ULSB. Accuracy was 136/149 or 91% with 10 false positive. Positive predictive value 52% and negative predictive value 98%. The graphical phonocardiogram facilitates visual analysis of S2 splitting and the duration and timing of the cardiac murmur. Conclusions: The cloud-based digital Ausc system is able to collect, transmit, organize and display phonocardiograms for remote CHD screening and consultation. Difficulty with respiratory contamination of the signal is noted. VSD and Still murmur were successfully differentiated. The visual phonocardiographic display can aid S2 analysis for splitting. The pilot study demonstrates the potential use of this system to extend pediatric cardiology expertise to underserved rural areas around the world.

Introduction: The Coronary Artery Risk Detection in Appalachian Communities (CARDIAC) Project has screened West Virginia 5th graders since 1998 to facilitate primordial prevention of coronary heart disease (CHD) in WV. LDL-c levels above 175 mg/dl in children suggest Familial Hyperlipidemia (FH) in the child’s family and a level above 160 mg/dl with history of CHD in relatives can also establish a diagnosis. Hypothesis: Based on previous adult literature, the association of lower height with higher LDL level observed in adults begins in childhood and is prominent in children with LDL in FH range. Methods: Fifth graders are screened yearly in WV schools with parental consent for Body Mass Index and lipid panel. Lipids were analyzed with respect to either short stature < 2 SD for height or comparing 1st (shortest) and 4th quartiles of the population. Statistical analysis for age- and gender-adjusted height percentiles was performed in SAS. Results: 59,386 children had lipid and height data. Mean LDL-c for 1st vs. 4th quartile of height was 94.08 mg/dl (95% Confidence Interval-CI 93.66-94.51) vs. 90.03 mg/dl (CI 89.65-90.42). First quartile of height students had average 4.05 mg/dl higher LDL-c (95% CI 3.48 -4.62 mg/dl). 4398 children had an LDL level above 130 g/dl, 632 above 160 mg/dl and 247 above 175 mg/dl. The Chi square analysis of short stature (height 130 g/dl was also significant (p=0.013) with increased odds of LDL-c above 130 g/dl compared to non-short stature (OR= 1.37, CL 1.07-1.75). Table 1 shows odds ratio for varying levels of elevated LDL-c for the first (shortest) vs. 4th (tallest) quartile of students. Conclusions: Shorter stature is associated with higher LDL-c level in WV 5th graders generally and in those children with increased risk for genetic dyslipidemia. The trend to increasing odds ratio in strata of higher LDL-c supports a recent report of association of single nucleotide polymorphisms selecting for lower genetic height and higher LDL-c.

Introduction: Congenital heart defects (CHD) may include hypoplastic valves, ventricles and vessels which complicate repairs. Our hypotheses were that hypoplastic structures are developmental rather than primarily genetic in origin and the correct biomechanical signal would induce catch up growth. Our corollary hypothesis was that the growth signal is provided by flow, not pressure. First stage operations, therefore, were designed to increase flow and induce growth. Once growth was sufficient, a second operation would often complete the repair. We report our results using flow induced growth of hypoplastic structures to facilitate repair in 3 types of CHD. Methods: Three groups of CHD patients with associated hypoplastic structures: unbalanced atrioventricular canal defects (UAVC); pulmonary atresia with intact ventricular septum (PAIVS); and coarctation of the aorta (CoA) were reviewed. Operations increased flow through the hypoplastic structures, such as creating restrictive septal defects to increase flow through the hypoplastic AV valves and ventricles (UAVC, PAIVS) or relieving obstruction (CoA) to enhance aortic valve and arch flow. Results: On follow up, virtually all (85/90) hypoplastic structures reached normal size and the rest had nearly comparable growth (Table). Conclusions: 1) Operations designed to increase flow through hypoplastic structures reliably induced catch up growth of UAVC, PAIVS and CoA lesions. 2) The growth response supported underdevelopment as the cause of hypoplasia and flow as the growth signal to reverse it. 3) Growth induction allowed two ventricle repairs in UAVC and PAIVS patients and avoided extended arch repairs in CoA; all with decreased operative risk. 4) Following catch up growth of the hypoplastic lesions, the outlook improved to that of patients with balanced AVCs, normal sized RVs in pulmonary atresia and well repaired CoA. 5) Once normal size was reached growth continued, producing a durable result.

Introduction: Patients with successfully repaired coarctation (CoA) need long term follow up due to the risk of abnormal arterial elasticity and complications of hypertension. We hypothesize that even subtle hemodynamic abnormalities are marked by lack of a blood pressure standing wave effect to the lower extremities (arm ≥ leg systolic BP: ARM Group) and is associated with elevated aortic flow velocities, increased left ventricular posterior wall thickness (LVPWd), higher arm BP and a greater requirement for interventions compared to subjects with the normal standing wave effect (leg > arm SBP: LEG Group). Methods: CoAs repaired between 1990 and 2008 with at least two documented right arm and leg SBPs at clinic visits along with M-mode, 2D and Doppler echocardiography were selected. The largest recorded peak instantaneous (PeakV) and mean velocities (MeanV) in the descending aorta were reviewed, as were measures of the proximal transverse arch (TA) and LVPWd. Measurements were indexed by Z-score. Patient records were also reviewed for reintervention and antihypertensive medical therapy. Results: Eighty patients met our criteria with 29 ARM (median age 12 years) and 51 LEG (median 13 years) Group members. Compared to LEG, ARM had significantly increased arm SBP Z-score (p -4 ), MeanV (p -6 ), and LVPWd (p Conclusions: Higher leg SBP after CoA repair is a marker for salutary hemodynamic and echo parameters as well as freedom from interventions post repair. Follow-up tailored by recognizing good outcome in LEG Group could be accomplished with less expense and obtrusive testing.

As the adult congenital heart disease population increases, poor transition from pediatric to adult care can lead to suboptimal quality of care and an increase in individual and institutional costs. In 2008, the American College of Cardiology and American Heart Association updated the adult congenital heart disease practice guidelines and in 2011, the American Heart Association recommended transition guidelines to standardize and encourage appropriate timing of transition to adult cardiac services. The objective of this study was to evaluate if patient age or complexity of congenital heart disease influences pediatric cardiologists' decision to transfer care to adult providers and to evaluate the compliance of different types of cardiology providers with current adult congenital heart disease treatment guidelines.A single-center retrospective review of 991 adult congenital heart disease patients identified by ICD-9 code from 2010 to 2012.Academic and community outpatient cardiology clinics.Nine hundred ninety-one patients who are 18 years and older with congenital heart disease.None.The compliance with health maintenance and transfer of care recommendations in the outpatient setting.For patients seen by pediatric cardiologists, only 20% had transfer of care discussions documented, most often in younger simple patients. Significant differences in compliance with preventative health guidelines were found between cardiology provider types.Even though a significant number of adults with congenital heart disease are lost to appropriate follow-up in their third and fourth decades of life, pediatric cardiologists discussed transfer of care with moderate and complex congenital heart disease patients less frequently. Appropriate transfer of adults with congenital heart disease to an adult congenital cardiologist provides an opportunity to reinforce the importance of regular follow-up in adulthood and may improve outcomes as adult congenital cardiologists followed the adult congenital heart disease guidelines more consistently than pediatric or adult cardiologists.

Children with special health care needs require special advanced planning for their unique emergencies. A Web site has been developed to allow secure Internet access to a database of Emergency Information Forms developed using the American College of Emergency Physicians/American Academy of Pediatrics format. The content and organization of the Web site, found at http://www.memscis.org, are described. A tour of the site is available. A set of XML data elements has been defined. Additional disaster preparedness elements have been added to the American College of Emergency Physicians/American Academy of Pediatrics Emergency Information Forms. The organization, security, and relationship of the site to electronic health records are described.

Pediatric patients frequently seek medical treatment in the emergency department (ED) unaccompanied by a legal guardian. Current state and federal laws and medical ethics recommendations support the ED treatment of minors with an identified emergency medical condition, regardless of consent issues. Financial reimbursement should not limit the minor patient’s access to emergency medical care or result in a breach of patient confidentiality. Every clinic, office practice, and ED should develop policies and guidelines regarding consent for the treatment of minors. The physician should document all discussions of consent and attempt to seek consent for treatment from the family or legal guardian and assent from the pediatric patient. Appropriate medical care for the pediatric patient with an urgent or emergent condition should never be withheld or delayed because of problems with obtaining consent. This statement has been endorsed by the American College of Surgeons, the Society of Pediatric Nurses, the Society of Critical Care Medicine, the American College of Emergency Physicians, the Emergency Nurses Association, and the National Association of EMS Physicians.

A current debate on the surgical management of patients with tetralogy of Fallot (TOF) focuses largely on primary repair vs initial shunt in younger and/or smaller patients. To characterize practice patterns throughout the United States with respect to the choice of initial surgical procedure in patients with TOF, we analyzed data from a multicenter database. This retrospective study focused on 938 patients from 12 institutions who underwent their initial operation during the 10-year period 1986 through 1995. Overall, the percentage of shunts decreased from 35.1% (1986-1990) to 22.0% (1991-1995) (p < 0.0001). The percentage of primary repairs increased accordingly. However, there was marked interinstitutional variability. For the group of patients aged 3 months or less the overall in-hospital mortality was significantly higher than that for older patients for both shunts and repairs. Multiple logistic regression analysis indicated that age, weight, date of surgery, and the interactions between date of surgery and institutional volume and between age and institutional volume were significant predictors of the initial surgical management of TOF. With this model only part of the observed variance could be explained. Other unidentified variables, including "institutional preference," may be significant factors influencing the choice of initial surgical procedure.

Objectives: Congenital heart disease may include hypoplastic heart valves, ventricles or great arteries. Infants with coarctation of the aorta (CoA) often have a hypoplastic transverse aortic arch (TAA) which can greatly complicate surgical repairs. Although these defects are often considered to be genetic in origin, our hypothesis was that they are problems of development which are potentially reversible. We tested the corollary hypothesis that hypoplastic TAAs retain normal developmental potential and the increased aortic flow after CoA repair provides the biomechanical signal for catch-up growth. Methods: Infants (N = 19) with TAA hypoplasia who underwent surgical CoA repair were studied for TAA growth by echocardiography done prior to and at intervals up to 13 years later. The TAA diameters were indexed using nomograms and calculated as standard deviations from expected size (SDE). Normal range is ± 2 SDE and < −2 indicates hypoplasia. Results: 1) TAA growth was rapid and significant within 3 months. 2) The initial average TAA SDE = −3.7 (range: −7.6 to −2.1) (0/19 normal); 3 months = −1.5 (-4.1 to 0.9) (12/17); 1 year = −1.0 (-4.2 to 1.3) (15/19); 5 to 13 years = −0.6 (-2.1 to 0.9) (16/17 normal). Conclusions: 1) Hypoplastic aortic arches grew rapidly to normal size with increased flow following CoA repair. 2) The results suggest the cause of hypoplasia was underdevelopment from low flow and, when increased, flow provided the biomechanical signal to induce catch up growth. 3) Infants were a relevant model for demonstrating the aortic growth signal. 4) Growth induction by increased blood flow could be used to reverse aortic underdevelopment in other selected patients.

The articles in this issue reveal the complexity of the PAIVS spectrum and the methods of treatment that are currently being used. Despite the complexity, the articles provide greater understanding of the developmental events leading to these lesions and the methods that could be used to reverse them. Taken together, the evidence certainly favors embarking on a 2VR track whenever possible. The number of completed biventricular repairs will be increased and also the adverse later consequences of a hypertensive RV and persisting significant RV to coronary artery connections will be eliminated when they are not. The algorithm, therefore, is heavily weighted to 2VRs.

The care of critically ill children has become more complex and demanding. This document establishes recommendations for developing regionalized integration of the care of these children into the emergency medical services system. These recommendations were developed by pediatricians with expertise in pediatric critical care, transport, and emergency medicine from the Committee on Pediatric Emergency Medicine, and the Pediatric Section of the Society of Critical Care Medicine Task Force on Regionalization of Pediatric Critical Care. The document was developed from existing guidelines from a number of professional organizations including the American Academy of Pediatrics and the Society of Critical Care Medicine, a thorough review of the literature, and expert consensus.

In a study to compare the clinical diagnostic skills of academic general pediatricians and academic pediatric cardiologists in the evaluation of heart murmurs, a total of 128 patients (aged 1 month to 18 years) newly referred to a university pediatric cardiology clinic were evaluated by one of three general pediatricians and one of four pediatric cardiologists. The murmurs were clinically classified as innocent, pathologic, or possibly pathologic. The classification was revised after the review of electrocardiogram (EKG) and chest radiograph (CXR), if indicated. The definitive diagnosis was ascertained by echocardiography (94 normal, 34 abnormal). The general pediatricians identified as many pathologic heart murmurs as the pediatric cardiologists (27/34 vs. 29/34), with no difference in sensitivity, 79% vs. 85% (p=0.53). The similarity in sensitivity could be because the general pediatricians were more cautious in the classification of heart murmurs and had classified more innocent heart murmurs as pathologic than the pediatric cardiologists (13/39 vs. 3/23), 41% vs. 13% (p=0.02). The pediatric cardiologists correctly identified more innocent murmurs than general pediatricians (52/94 vs.72/94), with a better specificity, 55% vs. 76% (p=0.001); however, the accuracy of prediction of innocence was similar for both groups (52/59 vs. 72/77), 88% vs. 93% (p=0.36). The revision of diagnosis with review of EKG and CXR was more often misleading than helpful for either group. Academic general pediatricians would identify most of the pathologic murmurs and are no more likely than an academic pediatric cardiologist to misclassify a pathologic heart murmur as innocent. Clin Pediatr. 1999;38:511-518

We describe the use of two in vitro tests to characterize plasma antioxidant capacity at the time of cardiac bypass in operations for congenital heart disease in 30 patients aged 3 days to 16 years (average 4.4 +/- 0.9 years [standard error]). Bypass and crossclamp time, circuit volume, and type of operation were recorded for each patient. First, a test of plasma radical antioxidant power measured chain breaking (secondary) antioxidant capacity of plasma to prevent oxidation of linoleic acid in vitro. Second, overall ability of plasma to prevent lipid peroxidation was assessed by a classic test of plasma inhibition of malondialdehyde formation in a beef brain homogenate. Plasma total radical antioxidant power level at baseline was 0.74 +/- 0.03 mumol/ml plasma, which decreased to 0.15 +/- 0.05 mumol/ml plasma after bypass (p0.001) and 0.26 +/- 0.08 mumol/ml plasma with recovery (n = 18, p0.001). Analysis of variance of postbypass total radical antioxidant power value showed age (p = 0.0002, r = 0.63) and bypass time (p = 0.009, r = 0.4677) to be significant factors. Pump prime volume in milliliters per kilogram and preoperative hemoglobin value were not significant factors. Beef brain malondialdehyde formation in vitro was limited 92% +/- 3% by normal plasma before operation versus 53% +/- 5% after operation (p0.001) and 51% +/- 5% at recovery after arrival in the pediatric intensive care unit (p0.001). Analysis of variance of the changes from before to after operation showed age p = 0.0015, r = 0.55) and bypass time (p = 0.033, r = 0.39) to be significant factors. Thus antioxidant capacity of plasma is significantly diminished after cardiopulmonary bypass in children. Young patient age and long duration of cardiopulmonary bypass are identified as factors that correlate positively with depletion of antioxidant capacity with bypass.

Objective: The purpose of this paper is to review the current state of health information technology (HIT) training programs and identify limitations in workforce expectations and student/trainee level of preparedness. A framework is proposed to build a more effective training program, differentiate HIT and health informatics, and emphasize the critical role of interprofessional collaboration for informatics-related curriculum. We define interprofessionalism as the multi-sector collaborations among academia, industry (Health Care Organizations), and vendors to produce competent informaticians.Methods: Critical review of published HIT and health informatics curricular competencies was conducted, including those published by the Office of the National Coordinator (ONC) for HIT, the American Medical Informatics Association (AMIA), the International Medical Informatics Association (IMIA), and the Council on Accreditation for Health Informatics and Information Management. A review of literature related to HIT and health informatics education and training was also completed.Results: The paper presents a framework for promoting health informatics training with an interprofessional foundation. The core components of the curricular competencies include understanding the healthcare system, biomedical data, computer programming, data analytics, usability, and technology infrastructure. To effectively deliver the content, programs require collaboration between academic institutions, healthcare organizations, and industry vendors.Conclusions: HIT and health informatics-related training programs, in their current form, are not meeting industry needs. The proposed framework addresses the current limitations by providing unique pathways for content delivery by promoting interprofessional collaboration and partnerships between academia and industry.