Validation of remote anthropometric measurements in a rural randomized pediatric clinical trial in primary care settings.

Rural children are more at risk for childhood obesity but may have difficulty participating in pediatric weight management clinical trials if in-person visits are required. Remote assessment of height and weight observed via videoconferencing may provide a solution by improving the accuracy of self-reported data. This study aims to validate a low-cost, scalable video-assisted protocol for remote height and weight measurements in children and caregivers. Families were provided with low-cost digital scales and tape measures and a standardized protocol for remote measurements. Thirty-three caregiver and child (6-11 years old) dyads completed remote (at home) height and weight measurements while being observed by research staff via videoconferencing, as well as in-person measurements with research staff. We compared the overall and absolute mean differences in child and caregiver weight, height, body mass index (BMI), and child BMI adjusted Z-score (BMIaz) between remote and in-person measurements using paired samples t-tests and one sample t-tests, respectively. Bland-Altman plots were used to estimate the limits of agreement (LOA) and assess systematic bias. Simple regression models were used to examine associations between measurement discrepancies and sociodemographic factors and number of days between measurements. Overall mean differences in child and caregiver weight, height, BMI, and child BMIaz were not significantly different between remote and in-person measurements. LOAs were - 2.1 and 1.7 kg for child weight, - 5.2 and 4.0 cm for child height, - 1.5 and 1.7 kg/m2 for child BMI, - 0.4 and 0.5 SD for child BMIaz, - 3.0 and 2.8 kg for caregiver weight, - 2.9 and 3.9 cm for caregiver height, and - 2.1 and 1.6 kg/m2 for caregiver BMI. Absolute mean differences were significantly different between the two approaches for all measurements. Child and caregiver age were each significantly associated with differences between remote and in-person caregiver height measurements; there were no significant associations with other measurement discrepancies. Remotely observed weight and height measurements using non-research grade equipment may be a feasible and valid approach for pediatric clinical trials in rural communities. However, researchers should carefully evaluate their measurement precision requirements and intervention effect size to determine whether remote height and weight measurements suit their studies.Trial registration: ClinicalTrials.gov NCT04142034 (29/10/2019).

Active vs Traditional Methods of Recruiting Children for a Clinical Trial in Rural Primary Care Clinics: A Cluster-Randomized Clinical Trial.

Importance: To our knowledge, there are no published randomized clinical trials of recruitment strategies. Rigorously evaluated successful recruitment strategies for children are needed. Objective: To evaluate the feasibility of 2 recruitment methods for enrolling rural children through primary care clinics to assess whether either or both methods are sufficiently effective for enrolling participants into a clinical trial of a behavioral telehealth intervention for children with overweight or obesity. Design, Setting, and Participants: This cluster-randomized clinical trial of 2 recruitment methods was conducted at 4 primary care clinics in 4 separate states. Each clinic used both recruitment methods in random order. Clinic eligibility criteria included at least 40% pediatric patients with Medicaid coverage and at least 100 potential participants. Eligibility criteria for children included a rural home address, age 6 to 11 years, and body mass index at or above the 85th percentile. Recruitment began February 3, 2020, and randomization of participants occurred on August 17, 2020. Data were analyzed from October 3, 2021, to April 21, 2022. Interventions: Two recruitment methods were assessed: the active method, for which a list of potential participants seen within the past year at each clinic was generated through the electronic health record and consecutively approached by research staff based on visit date to the clinic, and the traditional method, for which recruitment included posters, flyers, social media, and press release. Clinics were randomized to the order in which the 2 methods were implemented in 4-week periods, followed by a 4-week catch-up period using the method found most effective in previous periods. Main Outcomes and Measures: For each recruitment method, the number and proportion of randomized children among those who were approached was calculated. Results: A total of 104 participants were randomized (58 girls [55.8%]; mean age, 9.3 [95% CI, 9.0-9.6] years). Using the active method, 535 child-parent dyads were approached and 99 (18.5% [95% CI, 15.3%-22.1%]) were randomized. Using the traditional method, 23 caregivers expressed interest, and 5 (21.7% [95% CI, 7.5%-43.7%]) were randomized. All sites reached full enrollment using the active method and no sites achieved full enrollment using the traditional method. Mean time to full enrollment was 26.3 (range, 21.0-31.0) days. Conclusions and Relevance: This study supports the use of the active approach with local primary care clinics to recruit children with overweight and obesity from rural communities into clinical trials. Trial Registration: ClinicalTrials.gov Identifier: NCT04142034.

Conducting a pediatric randomized clinical trial during a pandemic: A shift to virtual procedures.

Background/Objective: Prior to the COVID-19 pandemic, our research group initiated a pediatric practice-based randomized trial for the treatment of childhood obesity in rural communities. Approximately 6 weeks into the originally planned 10-week enrollment period, the trial was forced to pause all study activity due to the COVID-19 pandemic. This pause necessitated a substantial revision in recruitment, enrollment, and other study methods in order to complete the trial using virtual procedures. This descriptive paper outlines methods used to recruit, enroll, and manage clinical trial participants with technology to obtain informed consent, obtain height and weight measurements by video, and maintain participant engagement throughout the duration of the trial. Methods: The study team reviewed the IRB records, protocol team meeting minutes and records, and surveyed the site teams to document the impact of the COVID-19 shift to virtual procedures on the study. The IRB approved study changes allowed for flexibility between clinical sites given variations in site resources, which was key to success of the implementation. Results: All study sites faced a variety of logistical challenges unique to their location yet successfully recruited the required number of patients for the trial. Ultimately, virtual procedures enhanced our ability to establish relationships with participants who were previously beyond our reach, but presented several challenges and required additional resources. Conclusion: Lessons learned from this study can assist other study groups in navigating challenges, especially when recruiting and implementing studies with rural and underserved populations or during challenging events like the pandemic.

Capacity Building for a New Multicenter Network Within the ECHO IDeA States Pediatric Clinical Trials Network.

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.

Best Practices for Conducting Clinical Trials With Indigenous Children in the United States.

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.

Coronary Artery Disease Phenotype Detection in an Academic Hospital System Setting.

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.

Deep learning for biological age estimation.

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.

Cardiometabolic risk factors in siblings from a statewide screening program.

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.

Understanding childhood obesity in the US: the NIH environmental influences on child health outcomes (ECHO) program.

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 <2 years and 38,332 aged 2-18 years. Main outcomes included high BMI for age <2 years, and at 2-18 years overweight (BMI 85th to <95th percentile), obesity (BMI ≥ 95th percentile), and severe obesity (BMI ≥ 120% of 95th percentile). RESULTS: The study population had diverse race/ethnicity and maternal demographics. Each outcome was more common with increasing age and varied with race/ethnicity. High BMI prevalence (95% CI) was 4.7% (3.5, 6.0) <1 year, and 10.6% (7.4, 13.7) for 1 to <2 years; overweight prevalence increased from 13.9% (12.4, 15.9) at 2-3 years to 19.9% (11.7, 28.2) at 12 to <18 years. ECHO has the statistical power to detect relative risks for 'high' BMI ranging from 1.2 to 2.2 for a wide range of exposure prevalences (1-50%) within each age group. CONCLUSIONS: ECHO is a powerful resource for understanding influences of chemical, biological, social, natural, and built environments on onset and trajectories of obesity in US children. The large sample size of ECHO cohorts adopting a standardized protocol for new data collection of varied exposures along with longitudinal assessments will allow refined analyses to identify drivers of childhood obesity.

Early testing of insulin resistance: a tale of two lipid ratios in a group of 5th graders screened by the Coronary Artery Risk Detection in Appalachian Communities Project (CARDIAC Project).

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.

LDL cholesterol level in fifth-grade schoolchildren associates with stature.

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.

Examination of cardiovascular risk factors and rurality in Appalachian children.

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.

Initial Field Test of a Cloud-Based Cardiac Auscultation System to Determine Murmur Etiology in Rural China.

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.

The Coronary Artery Risk Detection in Appalachian Communities (CARDIAC) Project: An 18 Year Review.

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.

Compliance with Adult Congenital Heart Disease Guidelines: Are We Following the Recommendations?

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.

Two-ventricle repairs in the unbalanced atrioventricular canal defect spectrum with midterm follow-up.

OBJECTIVES: Unbalanced atrioventricular (AV) canal defects include a hypoplastic ventricle (HV) and AV valve (HAVV) precluding complete 2-ventricle repairs (2VRs). Catch-up growth would solve this problem and was induced by increasing HAVV flow. The objectives were to assess reliability of HV and HAVV growth and provide 5- to 15-year 2VR follow-up. METHODS: From 1990 to 2005, 23 consecutive infants (13 females and 10 males) with echo-diagnosed unbalanced AV canal defects (n = 20) or subsets (n = 3) underwent 2VRs. HV volumes (18 left and 5 right) and HAVV sizes estimated from biplane echoes and z values (standard deviation from expected) were determined. Hypoplasia was defined by a z value of less than -2.0. Three operative approaches were used: (1) Staged repairs (n = 9) had complete AVV repairs with partial atrial septal defect and ventricular septal defect closures, which increased HAVV flow and maintained stability. The septal defects were closed later. (2) An asymmetric valve partition (n = 8) was used to increase HAVV size. (3) For moderate hypoplasia, HAVV flow was increased and ASDs/VSDs were left for stability (n = 6). Follow-up at 5 to 19 years was done locally. RESULTS: Staged repairs began at 20 to 328 days (average, 129 days) and were completed 5 to 145 days later (average, 101 days). Midterm survival was 87% (20/23) after 1 central nervous system bleed after trial weaning from extracorporeal membrane oxygenation and 2 later deaths from hyperkalemia. Reoperations for AVV regurgitation (n = 3), AVV stenosis (n = 1), and mitral valve replacement (n = 1) were satisfactory. On follow-up, all hypoplastic structures (HV and HAVV) had grown to normal size. Two patients "doing well" were lost to follow-up. Survivors have satisfactory 2VRs, with 15 of 18 taking no cardiac failure medications. CONCLUSIONS: Reliable HV/HAVV catch-up growth was induced, and all midterm 2VRs were satisfactory.

"Vanishing" pulmonary valve stenosis.

OBJECTIVE: Both spontaneous resolution and progression of mild pulmonary valve stenosis (PS) have been reported. We reviewed characteristics of the pulmonary valve (PV) to determine factors that could influence resolution of mild PS. METHODS: Fifteen asymptomatic pediatric patients with spontaneous resolution of isolated mild PS were retrospectively reviewed. RESULTS: There was no correlation between the PV gradient, clinical presentation, age at diagnosis, or PV morphology. The PV annulus was small at initial presentation, which normalized at follow up. When corrected for the body surface area (z-score), the PV annulus was normal in all patients, including at initial evaluation. CONCLUSIONS: Based on our observation, neither age at diagnosis, nor PV-morphology-influenced resolution of mild PS. The variable clinical presentation makes it difficult to categorize and observe mild PS by auscultation alone. The PV annulus z-score could be a useful adjunct to determine the course and serial observation of mild PS.

Mitral and tricuspid valve repair and growth in unbalanced atrial ventricular canal defects.

OBJECTIVE: Congenital mitral and tricuspid valve abnormalities in unbalanced atrioventricular canal defects are complex. We designed procedures to both repair and induce growth of hypoplastic atrioventricular valves and ventricles to achieve 2-ventricle repairs. Midterm data were assessed for reliability of catch-up growth, resulting quality of atrioventricular valves, and adequacy of 2-ventricle repairs. METHODS: The 24 consecutive infants (14 female and 10 male) with unbalanced atrioventricular canal defects had significant hypoplasia of 1 atrioventricular valve and/or ventricle (an echocardiography-derived z value of ≤-3.0 standard errors of the mean below expected). Operative approaches included the following: (1) Staged repair was performed, with complete valve repair, partial closure of the atrial septal, and ventricular septal defects, and (usually) pulmonary artery banding. After adequate growth, repair was completed. A vestigial mitral valve (4-7 mm) in 3 patients led to partitioning the large tricuspid valve, creating a second mitral valve. (2) Repair with a shift in atrioventricular valve partitioning was performed to increase hypoplastic atrioventricular valve size. (3) Repair with snared atrial septal defects and ventricular septal defect was performed to allow intracardiac shunting. The hypoplastic atrioventricular valves and hypoplastic ventricles were reassessed on local follow-up (5-15 years). RESULTS: The initial z scores were -2.8 to -7.4 for hypoplastic atrioventricular valves and -1.0 to -7.5 for hypoplastic ventricles. Follow-up z scores were -0.6 to -2.7 for hypoplastic atrioventricular valves and -2.0 to +1.8 for hypoplastic ventricles. Another 11 patients were also judged to be within normal limits. Three reoperations were for mitral valve regurgitation, and 1 reoperation was for mitral valve replacement. One patient died of central nervous system bleed just before extracorporeal membrane oxygenation weaning, and 2 patients died of late potassium overdose, for an 88% survival. Survivors are well with 2-ventricle repairs, and 15 of 19 patients are not taking cardiac medications. CONCLUSIONS: Increasing atrioventricular valve flow reliably induced growth. Valve repair and growth achieved a 2-ventricle repair in all patients.

Parent and emergency physician comfort with a system of on-line emergency-focused medical summaries for infants with significant cardiac disease.

Surveys were developed and administered to assess parental comfort with emergency care for children with special health care needs (CSHCN) with cardiac disease and the impact of a web-based database of emergency-focused clinical summaries (emergency information forms-EIF) called Midwest Emergency Medical Services for Children Information System (MEMSCIS) on parental attitudes regarding emergency care of their CSHCN. We hypothesized that MEMSCIS would improve the parent and provider outlook regarding emergencies of young children with heart disease in a randomized controlled trial. Children under age 2 were enrolled in MEMSCIS by study nurses associated with pediatric cardiac centers in a metropolitan area. Parents were surveyed at enrollment and 1 year on a 5-Point Likert Scale. Validity and reliability of the survey were evaluated. Study nurses formulated the emergency-focused summaries with cardiologists. One-hundred-seventy parent subjects, 94 study and 76 control, were surveyed at baseline and 1 year. Parents felt that hospital personnel were well-prepared for emergencies of their children and this improved from baseline 4.07 ± 1.03 to 1 year 4.24 ± 1.04 in study parents who had an EIF for their child and participated in the program (p = 0.0114) but not control parents. Parents perceived an improved comfort level by pre-hospital (p = 0.0256) and hospital (p = 0.0031) emergency personnel related to the MEMSCIS program. The MEMSCIS Program with its emergency-focused web-based clinical summary improved comfort levels for study parents. We speculate that the program facilitated normalization for parents even if the EIF was not used in an emergency during the study. The MEMSCIS program helps to prepare the family and the emergency system for care of CSHCN outside of the medical home.