Patient Driven EKG Device Performance in Adults with Fontan Palliation.

The aim of this study was to evaluate the accuracy of the KardiaMobile (KM) device in adults with a Fontan palliation, and to assess the KM function as a screening tool for atrial arrhythmias. While patient driven electrocardiogram (EKG) devices are becoming a validated way to evaluate cardiac arrhythmias, their role for patients with congenital heart disease is less clear. Patients with single ventricle Fontan palliation have a high prevalence of atrial arrhythmias and represent a unique cohort that could benefit from early detection of atrial arrhythmias. This single center prospective study enrolled adult patients with Fontan palliation to use the KM heart rhythm monitoring device for both symptomatic episodes and asymptomatic weekly screening over a 1-year period. Accuracy was assessed by comparing the automatic KM interpretation (KM-auto) to an electrophysiologist overread (KM-EP) and traditional EKG. Fifty patients were enrolled and 510 follow-up transmissions were received. The sensitivity and specificity of enrollment KM-auto compared to EKG was 65% and 100%, respectively. The sensitivity and specificity of enrollment KM-auto compared to the KM-EP was 75% and 96%, respectively. In the adult Fontan palliation, the accuracy of the KM device to detect a normal rhythm was reliable and best with a physician overread. Abnormal or uninterpretable KM-auto device interpretations, symptomatic transmissions, and any transmissions with a high heart rate compared to a patient's normal baseline should warrant further review.

Feasibility and Acceptability of a Prospective Syphilis Sexual Network Study for Sexual Minority Men.

BACKGROUND: In preparation for a prospective syphilis network study of sexual minority men, we conducted a mixed-methods formative study with the following objectives: a) assess acceptability of respondent driven sampling (RDS), b) assess acceptability of study procedures, c) social network seed selection, and d) pilot an ecological momentary assessment (EMA) study to assess social networking, sexual and substance use behavior. METHODS: We conducted in-depth interviews with eight providers serving sexual minority men and five focus group discussions with 34 sexual minority men, prioritizing four target populations: young Black sexual minority men, 2) on PrEP, 3) living with HIV, 4) not engaged in care. The 4-week EMA pilot was conducted with 40 sexual minority men. EMA survey responses were analyzed to evaluate how different compensation levels influenced response rates. Brief exit surveys were used to assess EMA app acceptability. RESULTS: Primary themes identified through qualitative data collection: 1) importance of developing trust and maintaining confidentiality during proposed recruitment activities, 2) importance of compensating participants appropriately for study activities, and 3) cultural considerations for increasing visibility and participation of young Black sexual minority men. All EMA participants reported being "completely comfortable" reporting sexual behavior through the app. Most (78%) preferred the app to in-person interviews. Several participants identified technical issues with the app, including not receiving push notifications and spontaneous closure. CONCLUSIONS: This mixed-methods formative study allowed for adjustments to and tailoring of the planned network study, including recruitment protocols, compensation type and amount, and EMA survey wording and response items.

Update of How Pediatric Cardiologists Noninvasively Evaluate Patients with Hypoplastic Left Heart Syndrome: 2013 vs. 2023.

Noninvasive functional assessment of systemic right ventricles (RV) in hypoplastic left heart syndrome (HLHS) is challenging. This study aimed to compare pediatric cardiologists' current noninvasive imaging practices to those reported in 2013. A web-based survey was distributed to pediatric cardiologists via various listservs which queried timing of echocardiograms during HLHS palliative stages and measurements of RV function. Demographics of 156 participants who completed the 2023 survey were similar to survey participants in 2013 (n = 222). Respondents were mostly male (62%), echocardiographers (48%), in university-based practice (67%) in North America (95%). Echocardiograms were predominantly obtained monthly during interstage I (41%), every 6 months during interstage II (56%), and every year post-Fontan (68%), which is consistent from 2013. Routine cardiac magnetic resonance imaging (cMRI) significantly increased in interstage II (8.2%, 17%) and post-Fontan (24%, 56%) populations, respectively. Qualitative assessment (41%), ejection fraction (EF) by 3D (20%), fractional area change (16%), and RV strain/strain rate (13%) were preferred methods for systolic assessment, whereas a plurality of respondents (41%) did not believe RV diastolic measurements were valid. The largest gap between currently obtained and desired measurements existed for EF by 3D (46.5% points) and RV strain/strain rate (44.5% points). No differences existed between imagers compared to non-imagers. Variability in evaluating HLHS patients continues among pediatric cardiologists compared to 10 years ago. Qualitative assessment remains the primary RV systolic functional evaluation. Providers do not rely on quantitative RV diastolic function assessments in HLHS patients. Use of cMRI is increasing for RV functional analysis.

Surfactant treatment at birth in a contemporary cohort of preterm infants with bronchopulmonary dysplasia.

OBJECTIVE: Initial surfactant studies demonstrated improvements in survival and need for respiratory support. However, as the use of non-invasive respiratory support has increased the use of surfactant has decreased. We examined in a contemporary cohort of BPD patients if surfactant use was associated with BPD severity. STUDY DESIGN: An observational study using data from the BPD Collaborative Registry. RESULTS: 971 infants with BPD met entry criteria, 864 (89%) had received surfactant in the first 72 h of life (SURF) and the remainder had not (no surfactant). There was an association between SURF and BPD grade, with a greater likelihood of grade 3 BPD in infants who received surfactant in the DR or who had 2 or more doses. CONCLUSIONS: We speculate that the use of surfactant in the DR and use of multiple doses reflect the impact of perinatal factors beyond immaturity alone that increase the risk for grade 3 BPD.

A pilot study of Keto Prescribed+: A healthy thinking and eating educational program for African American women.

ABSTRACT: African American (AA) women have the highest prevalence of obesity in addition to health disparities in preventable diet-related diseases (i.e., diabetes, hypertension), which places them at increased risk for cardiovascular disease. The purpose of this pilot study was to assess the feasibility, acceptability, and preliminary effectiveness of the Keto Prescribed+ (KetoRx+) program on associated physical and psychosocial outcomes among this population. The KetoRx+ program is a healthy eating and thinking educational intervention. The program combined online and in-person community group sessions over 8 weeks. The Keto Prescribed+ was found to be feasible and acceptable with comments on ways to increase acceptability from participants completing program (n = 10). Physical outcomes changed showed an average decrease in weight of 10lbs (SD = 5), baseline average 226lbs. Waist-to-hip ratio and systolic blood pressure also trended down. Psychosocial outcomes showed improvement trends. The KetoRx+ program is feasible and acceptable for overweight or obese AA women. Preliminary efficacy was established for most physical and psychosocial outcomes. However, more research is needed to identify specific program components contributing to healthy lifestyle behavior change and to establish program efficacy and effectiveness. Culturally adapted community-based biopsychosocial interventions using ketogenic nutrition therapy may help improve cardiovascular health of adult AA women.

Use of Initial Endotracheal Versus Intravenous Epinephrine During Neonatal Cardiopulmonary Resuscitation in the Delivery Room: Review of a National Database.

OBJECTIVE: To assess whether initial epinephrine administration by endotracheal tube (ET) in newly born infants receiving chest compressions and epinephrine in the delivery room (DR) is associated with lower rates of return of spontaneous circulation (ROSC) than newborns receiving initial intravenous (IV) epinephrine. STUDY DESIGN: We conducted a retrospective review of neonates receiving chest compressions and epinephrine in the DR from the AHA Get With The Guidelines-Resuscitation registry from October 2013 through July 2020. Neonates were classified according to initial route of epinephrine (ET vs IV). The primary outcome of interest was ROSC in the DR. RESULTS: In total, 408 infants met inclusion criteria; of these, 281 (68.9%) received initial ET epinephrine and 127 (31.1%) received initial IV epinephrine. The initial ET epinephrine group included those infants who also received subsequent IV epinephrine when ET epinephrine failed to achieve ROSC. Comparing initial ET with initial IV epinephrine, ROSC was achieved in 70.1% vs 58.3% (adjusted risk difference 10.02; 95% CI 0.05-19.99). ROSC was achieved in 58.3% with IV epinephrine alone, and 47.0% with ET epinephrine alone, with 40.0% receiving subsequent IV epinephrine. CONCLUSIONS: This study suggests that initial use of ET epinephrine is reasonable during DR resuscitation, as there were greater rates of ROSC compared with initial IV epinephrine administration. However, administration of IV epinephrine should not be delayed in those infants not responding to initial ET epinephrine, as almost one-half of infants who received initial ET epinephrine subsequently received IV epinephrine before achieving ROSC.

Feasibility and utility of in-home body weight support harness system use in young children treated for spinal muscular atrophy: A single-arm prospective cohort study.

PURPOSE: This single-arm prospective cohort study aimed to evaluate the feasibility and utility of in-home body weight support harness system (BWSS) use in children treated for spinal muscular atrophy (SMA). METHODS: Individuals with 2 or 3 copies of SMN2 who received pharmacotherapeutic treatment, had head control, and weight <50lbs were enrolled. Families were provided a BWSS and documented use. Motor outcome assessments were completed at baseline, month 3 and month 6. Families provided feedback in an end of study survey. RESULTS: All 32 participants (2.9 (SD 1.9) yrs), improved or remained stable on all outcomes. Average reported frequency of use was 4.1(2.3) hrs/week. Controlling for other covariates, frequency of use explained over 70% of the variability in change scores. Family feedback was overwhelmingly positive. CONCLUSION: Use of in-home BWSS is a safe, feasible and useful option to increase exercise dosage after treatment in SMA and may help optimize motor abilities. TRIAL REGISTRATION: Study registered with: Clinicaltrials.gov Clinicaltrials.gov identifier: NCT05715749.

Transpyloric feeding is associated with adverse in-hospital outcomes in infants with severe bronchopulmonary dysplasia.

OBJECTIVE: To estimate the association of transpyloric feeding (TPF) with the composite outcome of tracheostomy or death for patients with severe bronchopulmonary dysplasia (sBPD). STUDY DESIGN: Retrospective multi-center cohort study of preterm infants <32 weeks with sBPD receiving enteral feedings. We compared infants who received TPF at 36, 44, or 50 weeks post-menstrual age to those who did not receive TPF at any of those timepoints. Odds ratios were adjusted for gestational age, small for gestational age, male sex, and invasive ventilation and FiO2 at 36 weeks. RESULTS: Among 1039 patients, 129 (12%) received TPF. TPF was associated with an increased odds of tracheostomy or death (aOR 3.5, 95% CI 2.0-6.1) and prolonged length of stay or death (aOR 3.1, 95% CI 1.9-5.2). CONCLUSIONS: Use of TPF in sBPD after 36 weeks was infrequent and associated with worse in-hospital outcomes, even after adjusting for respiratory severity at 36 weeks.

Association of the respiratory severity score with bronchopulmonary dysplasia-associated pulmonary hypertension in infants born extremely preterm.

OBJECTIVE: To test the hypothesis that elevations in the respiratory severity score (RSS) are associated with increased probability of bronchopulmonary dysplasia-associated pulmonary hypertension (BPD-PH). STUDY DESIGN: Retrospective cohort study of infants born extremely preterm admitted to a BPD center between 2010 and 2018. Echocardiograms obtained ≥ 36 weeks' post-menstrual age (PMA) were independently adjudicated by two blinded cardiologists to determine the presence/absence of BPD-PH. Multivariable logistic regression estimated the association between RSS and BPD-PH. RESULT: BPD-PH was observed in 68/223 (36%) of subjects. The median RSS at time of echocardiography was 3.04 (Range 0-18.3). A one-point increase in the RSS was associated with BPD-PH, aOR 1.3 (95% CI 1.2-1.4), after adjustment for gestational age and PMA at time of echocardiography. CONCLUSION: Elevations in the RSS were associated with a greater probability of BPD-PH. Prospective studies are needed to determine the validity and performance of RSS as a clinical susceptibility/risk biomarker for BPD-PH.

Exploring the Association of Male Sex With Adverse Outcomes in Severe Bronchopulmonary Dysplasia: A Retrospective, Multicenter Cohort Study.

BACKGROUND: Bronchopulmonary dysplasia (BPD) is a significant contributor to morbidity and death in infants who are born premature. Male sex is an independent risk factor for the development of BPD. However, whether male sex is associated with adverse outcomes that occur after formal diagnosis of severe BPD prior to hospital discharge remains unclear. RESEARCH QUESTION: Is male sex associated with a higher risk of adverse outcomes in infants with established severe BPD? STUDY DESIGN AND METHODS: A retrospective, multicenter cohort study of infants enrolled in the BPD Collaborative Registry from January 1, 2015, to June 29, 2022, was performed. Demographics, clinical characteristics, and outcomes were stratified by sex (ie, male vs female). Regression modeling was used to estimate the association of sex with the primary composite outcome of death or tracheostomy at hospital discharge. RESULTS: We identified 1,156 infants with severe BPD, defined at 36 weeks postmenstrual age by the National Institutes of Health 2001 consensus definition. The cohort was predominantly male (59% male infants, 41% female infants). However, rates of mechanical ventilation at 36 weeks postmenstrual age (ie, type 2 severe BPD) did not differ by sex. Overall mortality rates within the cohort were low (male infants, 5.3%; female infants, 3.6%). The OR of death or tracheostomy for male-to-female infants was 1.0 (95% CI, 0.7-1.5). INTERPRETATION: Our results lead us to speculate that, although sex is an important variable that contributes to the development and pathogenesis of severe BPD, it does not appear to be associated with adverse outcomes in this cohort of infants with established disease. The surprising results raise important questions surrounding the temporal role of biological sex in the development of severe BPD and its progression during the neonatal ICU stay. As we explore the phenotypes and endotypes of BPD, it is imperative to consider how sex modulates the disease from birth through hospital discharge.

Evaluating Listening Effort in Unilateral, Bimodal, and Bilateral Cochlear Implant Users.

OBJECTIVE: Evaluate listening effort (LE) in unilateral, bilateral, and bimodal cochlear implant (CI) users. Establish an easy-to-implement task of LE that could be useful for clinical decision making. STUDY DESIGN: Prospective cohort study. SETTING: Tertiary neurotology center. METHODS: The Sentence Final Word Identification and Recall Task, an established measure of LE, was modified to include challenging listening conditions (multitalker babble, gender, and emotional variation; test), in addition to single-talker sentences (control). Participants listened to lists of sentences in each condition and recalled the last word of each sentence. LE was quantified by percentage of words correctly recalled and was compared across conditions, across CI groups, and within subjects (best aided vs monaural). RESULTS: A total of 24 adults between the ages of 37 and 82 years enrolled, including 4 unilateral CI users (CI), 10 bilateral CI users (CICI), and 10 bimodal CI users (CIHA). Task condition impacted LE (P < .001), but hearing configuration and listener group did not (P = .90). Working memory capacity and contralateral hearing contributed to individual performance. CONCLUSION: This study adds to the growing body of literature on LE in challenging listening conditions for CI users and demonstrates feasibility of a simple behavioral task that could be implemented clinically to assess LE. This study also highlights the potential benefits of bimodal hearing and individual hearing and cognitive factors in understanding individual differences in performance, which will be evaluated through further research.

Associations of Cancer Patients' Demographic and Clinical Characteristics With Cannabis-Related Interest and Behaviors.

Background: Cannabis interest and use is increasing in the United States, yet research on its use among cancer patients is limited. Methods: Individuals with cancer completed an anonymous cross-sectional questionnaire. Multivariable logistic regressions estimated odds ratios (OR) between patients' demographic and clinical characteristics with cannabis-related interest, current use, and provider recommendation. Results: Participants (n = 943) were, on average, 61.7 years old. Older patients were less likely to use cannabis products (OR = 0.42, confidence interval [95% CI]: 0.26-0.69) and less likely to be interested in cannabis (OR = 0.60, 95% CI: 0.44-0.84) than younger patients. Those with higher education were less likely to be using cannabis (OR = 0.41, 95% CI: 0.25-0.67) and less likely to have received a provider recommendation of cannabis use than the least educated (OR = 0.38, 95% CI: 0.19-0.76). Cancer spread and type were significant correlates of provider recommendation of cannabis use. Conclusions: Additional research is warranted to better understand cancer patients' motivations for cannabis use and interest.

Marijuana and Cannabidiol Use Prevalence and Symptom Management Among Patients with Cancer.

Symptoms such as pain, nausea, and anxiety are common in individuals with cancer. Treatment of these issues is often challenging. Cannabis products may be helpful in reducing the severity of these symptoms. While some studies include data on the prevalence of cannabis use among patients with cancer, detailed data remain limited, and none have reported the prevalence of cannabidiol (CBD) use in this population. Adult patients with cancer attending eight clinics at a large, NCI-designated Comprehensive Cancer Center completed a detailed, cannabis-focused questionnaire between 2021 and 2022. Eligible participants were diagnosed with invasive cancer and treated in the past 12 months. Summary statistics were calculated to describe the sample regarding cannabis use. Approximately 15% (n = 142) of consented patients (n = 934) reported current cannabis use (defined as use within the past 12 months). Among which, 75% reported cannabis use in the past week. Among current cannabis users, 39% (n = 56; 6% overall) used CBD products. Current users reported using cannabis a median of 4.5 (interquartile range: 0.6­7.0) days/week, 2.0 (1.0­3.0) times per use/day, and for 3 years (0.8­30.0). Use patterns varied by route of administration. Patients reported moderate to high relief of symptoms with cannabis use. This study is the most detailed to date in terms of cannabis measurement and provides information about the current state of cannabis use in active cancer. Future studies should include complete assessments of cannabis product use, multiple recruitment sites, and diverse patient populations. SIGNIFICANCE: Clinicians should be aware that patients are using cannabis products and perceive symptom relief with its use.

Utility of the NEONATE Score at an Institution that Routinely Performs the Hybrid Procedure for Hypoplastic Left Heart Syndrome.

NEONATE score > 17 has been proposed as a risk factor for interstage mortality/cardiac transplant (IM/T) for patients with single ventricle physiology. Hybrid procedure is assigned 6 points, the highest possible score for that surgical variable. Most centers reserve the hybrid procedure for high-risk patients. Goal of this study was to evaluate the NEONATE score at a center that routinely performs the hybrid procedure. Retrospective chart review of patients undergoing the hybrid procedure was performed (2008-2021). Demographics and variables used for the NEONATE score were collected. Maximization of Youden's J Statistic used to determine cohort-specific optimal threshold for patients undergoing comprehensive Stage II procedure (H-CSII) versus those with IM/T (H-IM/T). Total of 120 patients met inclusion criteria (H-CSII = 105, H-IM/T = 15). Gestational age was median 39 weeks (IQR 38, 39) and birth weight was 3.18 kg (2.91, 3.57). No patient was discharged with opiates or required post-operative extracorporeal circulatory support. Optimal threshold, as selected by maximizing Youden's J Statistic, was 22. Score > 22 had a positive predictive value of 0.33 (95% CI 0.12-0.62), negative predictive values of 0.90 (95% CI 0.83-0.95), and accuracy of 0.83 (95% CI 0.75-0.90) for IM/T. At a center that routinely performs the hybrid procedure, value of > 22 had the highest accuracy. This suggests that the hybrid procedure is not necessarily intrinsically a risk-factor for IM/T, but rather patient selection for the hybrid procedure may play a larger role at centers that do not routinely perform this procedure.

The Risk of Developmental Dysplasia of the Hip in Premature Infants with Breech Presentation at Birth.

OBJECTIVE: This article evaluates the risk (defined here as incidence per 1,000 infants) of developmental dysplasia of the hip (DDH) in premature infants undergoing hip ultrasound (HUS) for breech presentation only compared with other indications. STUDY DESIGN: Retrospective study of infants born between January 1, 2009 and December 31, 2018 at <37 weeks' gestation with a HUS obtained in the first year of life, at Nationwide Children's Hospital, the only available facility for pediatric radiology and orthopaedic services in central Ohio. We calculated risk differences and odds ratios of DDH by the indication of HUS. RESULTS: From 2,397 infants reviewed, 89% underwent HUS for breech presentation only. The local incidence of DDH for infants undergoing HUS for breech-only indication was 15 per 1,000 compared with 155 per 1,000 for infants undergoing HUS for other indications. The odds ratio of DDH diagnosis was 12.1 (95% confidence interval: 7.5, 19.6) for infants undergoing HUS for an indication other than breech presentation only relative to infants undergoing HUS for the indication of breech presentation only. CONCLUSION: The risk of DDH diagnosis in premature infants undergoing HUS for breech presentation is much lower than those undergoing HUS for clinical concerns and other risk factors. Screening these infants with physical examination may be sufficient. KEY POINTS: · Breech presentation is the most common indication for hip ultrasound in premature infants.. · The risk of DDH is lower in premature infants with breech presentation compared with premature infants with other clinical concerns.. · The risk of DDH is higher in infants born at 33 to 36 weeks of gestation compared with those born at an earlier gestation..

Association of the Respiratory Severity Score with Bronchopulmonary Dysplasia-Associated Pulmonary Hypertension in Infants Born Extremely Preterm.

Objective: To test the hypothesis that elevations in the respiratory severity score (RSS) are associated with increased risk of bronchopulmonary dysplasia-associated pulmonary hypertension (BPD-PH). Study Design: Retrospective cohort study of infants born extremely preterm admitted to a BPD center between 2010-2018. Echocardiograms obtained ≥36 weeks' post-menstrual age (PMA) were independently adjudicated by two blinded cardiologists to determine the presence/absence of BPD-PH. Multivariable logistic regression estimated the association between RSS with BPD-PH. Result: BPD-PH was observed in 68/223 (36%) of subjects. The median RSS at time of echocardiography was 3.04 (Range 0-18.3). A one-point increase in RSS was associated with BPD-PH, aOR 1.3 (95% CI 1.2-1.4), after adjustment for gestational age and PMA at time of echocardiography. Conclusion: Elevations in the RSS were associated with a greater risk of BPD-PH. Prospective studies are needed to determine the validity and performance of RSS as a clinical susceptibility/risk biomarker for BPD-PH.

Bayley trajectories predict school readiness better than single assessments in formerly very preterm preschoolers.

BACKGROUND: Development of children born very preterm (VPT) is evaluated using the Bayley Scales of Infant Development. Early Bayley scores may not predict later outcomes. We studied whether VPT Bayley trajectories in the early years predicted school readiness better than single assessments. METHODS: We prospectively evaluated 53 VPT at 4-5 years using standardized measures of school readiness, including the domains of cognition, early mathematical and literacy abilities, and motor skills. Predictors were Bayley-III scores obtained 1-5 times/child between 6 and 35 months. Linear mixed models (LMM) with random effects extracted estimated random effect for slope (change in Bayley score/1 year) and fixed+random effect sum for the intercept (initial Bayley score) for each participant, to then evaluate 4-5-year outcomes prediction. RESULTS: Variability of individual trajectories prevailed across developmental domains. For the initial LMM, adding Bayley change to models with only initial score improved model fits for several Bayley-III domains. Models containing estimates for initial Bayley scores and Bayley change explained significantly more variability in school readiness scores (21-63%) than either variable alone. CONCLUSION: Neurodevelopmental follow-up of VPT is more relevant to school readiness when children are assessed multiple times in the first 3 years. Neonatal intervention research could use early trajectories rather than single timepoints as outcomes. IMPACT: This study is the first to examine individual Bayley scores and trajectories to predict school readiness of formerly preterm children at 4-5 years. Modeling demonstrated extreme variability of individual trajectories compared to the group's average trajectories. Models containing initial Bayley scores and Bayley change over time explained more variability in preschool readiness than either variable alone. Using the Bayley to predict future school readiness is enhanced by administration across multiple follow-up visits and inclusion of change across the first 3 years. Follow-up care models and clinical trial design for neonatal interventions may benefit from a trajectory-based approach to outcomes evaluation.