Fetal Echocardiographic Evaluation of Tricuspid Valve and Right Ventricular Function Including Global Longitudinal Strain in Hypoplastic Left Heart Syndrome and Association with Postnatal Outcomes.

Despite significant advancements in the care of patients with hypoplastic left heart syndrome (HLHS) morbidity and mortality remain high. Postnatal right ventricular dysfunction and tricuspid regurgitation (TR) are associated with worse outcomes in HLHS. We aim to determine if right ventricle functional parameters and TR on fetal echocardiogram are associated with postnatal outcomes in HLHS patients. Retrospective review was performed on all fetuses with HLHS from 2014 to 2022 at our institution. Initial and follow up fetal echocardiogram measurements of right ventricular myocardial performance index (MPI), fractional area change (FAC) and global longitudinal strain (GLS) were retrospectively measured. The presence and severity of TR was recorded from the fetal echocardiogram reports. Postnatal outcomes including transplant-free survival, hospital length of stay > 30 days after initial palliation and need for bidirectional Glenn at < 4 months were reviewed. Forty-three subjects met inclusion criteria. Mean gestational age at presentation was 26.1 ± 5.9 weeks. Nine subjects died and 3 required heart transplantation. Initial fetal echocardiogram MPI was significantly lower (better) (0.36 ± 0.06 vs 0.44 ± 0.11; p = < 0.001) and FAC was significantly higher (better) (45 ± 6% vs 40 ± 8%; p = 0.035) in transplant-free survivors. Fetal right ventricular GLS and presence of mild TR were not associated with postnatal outcome. In fetuses with HLHS, abnormal MPI and right ventricular FAC are associated with decreased transplant-free survival. There was no observed association between GLS and postnatal outcomes. To our knowledge this is the first study examining fetal right ventricular function and GLS in HLHS patients and its link to postnatal outcomes.

Injection Medialization in Infants with Vocal Fold Immobility Improves Dysphagia.

BACKGROUND: Unilateral vocal fold immobility (VFI) is a known cause of morbidity amongst children following congenital heart surgery. Injection medialization (IM) provides medial distraction and improves glottic closure. Limited objective data is available for the effect of IM in young children (<2 years-old) with VFI. METHODS: Retrospective case series of infants <2 who underwent IM for VFI after congenital cardiac surgery. Primary outcome was objective reduced risk of aspiration based on Dysphagia and Outcome Severity Scores (DOSS) on Video swallow study (VFSS) performed prior to and within 4 weeks following IM. Secondary analysis included perioperative complications and number of children who were able to avoid NG or G tube placement. RESULTS: 17 children <2 years of age had unilateral VFI after congenital cardiac surgery and underwent IM. The median age at time of initial cardiac surgery was 6 days (IQR 3-7). There was no intraoperative or postoperative stridor or associated complications. All 17 patients had preoperative aspiration noted on VFSS. Average swallowing outcomes on VFSS improved after IM with an increase in DOSS score (preop score 3 (IQR 2-4) to postop score 6.5 (IQR 5-7) [P = 0.001]). At 2 months following IM, of the patients who had improvement in swallowing function, 50% (n = 6) were able to feed completely orally, 25% (n = 3) were fed orally with an NG wean, and 3 (25%) had a G tube placed. CONCLUSION: Initial results suggest that IM is safe and improves early objective swallowing outcomes in children <2 years old with VFI after congenital cardiac surgery. LEVEL OF EVIDENCE: IV Laryngoscope, 2024.

Utility of apical four-chamber longitudinal strain in the assessment of childhood cancer survivors: A multicenter study.

BACKGROUND: A previous multicenter study showed that longitudinal changes in standard cardiac functional parameters were associated with the development of cardiomyopathy in childhood cancer survivors (CCS). Evaluation of the relationship between global longitudinal strain (GLS) changes and cardiomyopathy risk was limited, largely due to lack of quality apical 2- and 3-chamber views in addition to 4-chamber view. We sought to determine whether apical 4-chamber longitudinal strain (A4LS) alone can serve as a suitable surrogate for GLS in this population. METHODS: A4LS and GLS were measured in echocardiograms with acceptable apical 2-, 3-, and 4-chamber views. Correlation was evaluated using Pearson and Spearman coefficients, and agreement was evaluated with Bland-Altman plots. The ability of A4LS to identify normal and abnormal values compared to GLS as the reference was evaluated. RESULTS: Among a total of 632 reviewed echocardiograms, we identified 130 echocardiograms from 56 patients with adequate views (38% female; mean age at cancer diagnosis 8.3 years; mean follow-up 9.4 years). Correlation coefficients between A4LS and GLS were .89 (Pearson) and .85 (Spearman), with Bland-Altman plot of GLS-A4LS showing a mean difference of -.71 ± 1.8. Compared with GLS as the gold standard, A4LS had a sensitivity of 86% (95% CI 79%-93%) and specificity of 82% (69%-95%) when using normal range cutoffs and 90% (82%-97%) and 70% (58%-81%) when using ±2 standard deviations. CONCLUSION: A4LS performs well when compared with GLS in this population. Given the more recent adoption of apical 2- and 3-chamber views in most pediatric echocardiography laboratories, A4LS is a reasonable stand-alone measurement in retrospective analyses of older study cohorts and echocardiogram biorepositories.

Consensus-Based Development of a Pediatric Echocardiography Complexity Score: Design, Rationale, and Results of a Quality Improvement Collaborative.

BACKGROUND: The complexity of congenital heart disease has been primarily stratified on the basis of surgical technical difficulty, specific diagnoses, and associated outcomes. We report on the refinement and validation of a pediatric echocardiography complexity (PEC) score. METHODS AND RESULTS: The American College of Cardiology Quality Network assembled a panel from 12 centers to refine a previously published PEC score developed in a single institution. The panel refined complexity categories and included study modifiers to account for complexity related to performance of the echocardiogram. Each center submitted data using the PEC scoring tool on 15 consecutive inpatient and outpatient echocardiograms. Univariate and multivariate analyses were performed to assess for independent predictors of longer study duration. Among the 174 echocardiograms analyzed, 68.9% had underlying congenital heart disease; 44.8% were outpatient; 34.5% were performed in an intensive care setting; 61.5% were follow-up; 46.6% were initial or preoperative; and 9.8% were sedated. All studies had an assigned PEC score. In univariate analysis, longer study duration was associated with several patient and study variables (age <2 years, PEC 4 or 5, initial study, preoperative study, junior or trainee scanner, and need for additional imaging). In multivariable analysis, a higher PEC score of 4 or 5 was independently associated with longer study duration after controlling for study variables and center variation. CONCLUSIONS: The PEC scoring tool is feasible and applicable in a variety of clinical settings and can be used for correlation with diagnostic errors, allocation of resources, and assessment of physician and sonographer effort in performing, interpreting, and training in pediatric echocardiography.

The American Association for Thoracic Surgery (AATS) 2023 Expert Consensus Document: Recommendation for the care of children with trisomy 13 or trisomy 18 and a congenital heart defect.

OBJECTIVES: Recommendations for surgical repair of a congenital heart defect in children with trisomy 13 or trisomy 18 remain controversial, are subject to biases, and are largely unsupported with limited empirical data. This has created significant distrust and uncertainty among parents and could potentially lead to suboptimal care for patients. A working group, representing several clinical specialties involved with the care of these children, developed recommendations to assist in the decision-making process for congenital heart defect care in this population. The goal of these recommendations is to provide families and their health care teams with a framework for clinical decision making based on the literature and expert opinions. METHODS: This project was performed under the auspices of the AATS Congenital Heart Surgery Evidence-Based Medicine Taskforce. A Patient/Population, Intervention, Comparison/Control, Outcome process was used to generate preliminary statements and recommendations to address various aspects related to cardiac surgery in children with trisomy 13 or trisomy 18. Delphi methodology was then used iteratively to generate consensus among the group using a structured communication process. RESULTS: Nine recommendations were developed from a set of initial statements that arose from the Patient/Population, Intervention, Comparison/Control, Outcome process methodology following the groups' review of more than 500 articles. These recommendations were adjudicated by this group of experts using a modified Delphi process in a reproducible fashion and make up the current publication. The Class (strength) of recommendations was usually Class IIa (moderate benefit), and the overall level (quality) of evidence was level C-limited data. CONCLUSIONS: This is the first set of recommendations collated by an expert multidisciplinary group to address specific issues around indications for surgical intervention in children with trisomy 13 or trisomy 18 with congenital heart defect. Based on our analysis of recent data, we recommend that decisions should not be based solely on the presence of trisomy but, instead, should be made on a case-by-case basis, considering both the severity of the baby's heart disease as well as the presence of other anomalies. These recommendations offer a framework to assist parents and clinicians in surgical decision making for children who have trisomy 13 or trisomy 18 with congenital heart defect.

Ultrasound Screening After Cardiac Surgery Shows Vocal Fold Impairment and Predicts Aspiration.

INTRODUCTION: Vocal fold motion impairment (VFMI) is a known consequence after high-risk cardiac surgery. We implemented a universal laryngeal ultrasound (LUS) screening protocol for VFMI after the Norwood and aortic arch surgery. We hypothesized that LUS would accurately identify VFMI and predict postoperative aspiration. METHODS: We implemented a screening algorithm with LUS for patients undergoing high-risk cardiac surgery at a tertiary care pediatric hospital. Positively screened patients underwent flexible nasolaryngoscopy (FNL). Patients with an abnormal FNL underwent a video-fluoroscopic swallow study (VFSS). Patient demographics, length of stay, and swallowing outcomes were assessed. Two-tailed chi square and Wilcoxon rank sum tests were used to assess for differences. RESULTS: Sixty-seven patients underwent either Norwood or arch reconstruction over a 16-month period and underwent universal LUS. The average birth weight was 3.24 kg (SD 0.57). Of the 67 patients, VFMI was identified by LUS and 100% confirmed on FNL in 58.21% (n = 39/67) of patients. Aspiration and penetration on VFSS were higher in the group with VFMI as compared with those without VFMI (53.8% vs. 21.4%, p = 0.008). There was no difference in length of stay between patients who did not have a diagnosis of VFMI and those found to have VFMI (41.0 days vs 45.3 days p = 0.73). CONCLUSIONS: Universal LUS screening for patients following high-risk cardiac surgery may lead to earlier identification of postoperative VFMI and aspiration. Recognition of VFMI through this universal screening program could lead to earlier interventions and possibly improved swallowing outcomes. LEVEL OF EVIDENCE: 3 Laryngoscope, 134:1939-1944, 2024.

Enhanced congenital cardiac surgery knowledge with multidisciplinary collaborative education series for paediatric cardiology fellows and nurse practitioners.

INTRODUCTION: Paediatric cardiologists and nurse practitioners lack structured education tools focused on basic cardiac surgery principles. However, non-surgical specialties caring for surgical patients require this knowledge for comprehensive clinical care. We created a cardiac surgical educational curriculum focused on improving knowledge and attitudes towards communication for non-surgical trainees and advanced practice providers. METHODS: Over one academic year, six paediatric cardiology fellows and seven paediatric cardiac surgery nurse practitioners at Seattle Children's Hospital participated in this study. With surgical supervision, six lectures were prepared by each fellow and delivered monthly. Sessions were hybrid and recorded for later viewing. Pre- and post-intervention survey of attitudes regarding surgical topics and pre- and post- test-based knowledge assessments were administered. RESULTS: Participants positively rated the usefulness of the lecture series (4.2/5) and would recommend it to a colleague (4.5/5). Self-reported confidence discussing surgical concepts with patients increased from 2.3 to 3.4 among paediatric cardiology fellows (p < 0.001) and from 2.8 to 3.9 among nurse practitioners (p < 0.001), out of 5. In both groups, knowledge assessment scores improved from 54 to 79% post-intervention (p < 0.001). CONCLUSIONS: After a six-part educational series taught by paediatric cardiology fellows, both paediatric cardiology fellows and paediatric cardiac surgery nurse practitioners demonstrated improved knowledge and reported increased comfort counselling families on basic cardiac surgery topics. Structured, active-learning lessons taught by fellows for non-surgical audiences can improve attitudes and build clinically relevant knowledge. Creating an effective level-appropriate multidisciplinary curriculum accessible to various types of medical providers could enhance comprehensive care of complex congenital cardiac surgery patients.

Affective reactivity to daily stressors and immune cell gene expression in the MIDUS study.

Affective reactivity to stress is a person-level measurement of how well an individual copes with daily stressors. A common method of measuring affective reactivity entails the estimation of within-person differences of either positive or negative affect on days with and without stressors present. Individuals more reactive to common stressors, as evidenced by affective reactivity measurements, have been shown to have increased levels of circulating pro-inflammatory markers. While affective reactivity has previously been associated with inflammatory markers, the upstream mechanistic links underlying these associations are unknown. Using data from the Midlife in the United States (MIDUS) Refresher study (N = 195; 52% female; 84% white), we quantified daily stress processes over 10 days and determined individuals' positive and negative affective reactivities to stressors. We then examined affective reactivity association with peripheral blood mononuclear cell (PBMC) gene expression of the immune-related conserved transcriptional response to adversity. Results indicated that individuals with a greater decrease in positive affect to daily stressors exhibited heightened PBMC JUNB expression after Bonferroni corrections (p-adjusted < 0.05). JUNB encodes a protein that acts as a transcription factor which regulates many aspects of the immune response, including inflammation and cell proliferation. Due to its critical role in the activation of macrophages and maintenance of CD4+ T-cells during inflammation, JUNB may serve as a potential upstream mechanistic target for future studies of the connection between affective reactivity and inflammatory processes. Overall, our findings provide evidence that affective reactivity to stress is associated with levels of immune cell gene expression.

Improving Timeliness of Vocal Fold Mechanical Injury Screening Following Norwood or Arch Reconstruction: A Quality Improvement Initiative at a Single Center.

Vocal fold (VF) immobility is a common complication after pediatric cardiothoracic surgeries involving the aortic arch and conotruncal region. Nasolaryngoscopy is considered the standard for diagnosis but is invasive and requires expertise and special resources. VF ultrasound (VF US) is an efficient, non-invasive alternative for VF evaluation in the post-cardiac surgical setting. Our aim was to improve screening rates for vocal fold motion impairment (VFMI) by implementing VF US in a group of pre-identified high-risk patients after index cardiac surgeries using Quality Improvement (QI) methodology. The QI project included formation of a widely representative stakeholder team, collaborative development of a screening protocol for the cohort of patients in our tertiary center. Baseline data were derived by retrospective review of screening and incidence of VFMI in a similar post-surgical cohort in 2 years prior to this intervention. We implemented an US screening algorithm with multidisciplinary care coordination. We evaluated feeding practices and length of stay (LOS) related to our screening interventions and documented follow up practices. Screening for VFMI by ultrasound increased from 59 to 92% after implementation of the VF screening protocol. Additionally, time between extubation and VF US decreased from 7.7 to 2.3 days. The positive predictive value of VF US was 96%. Patients with VFMI had a longer LOS and greater dependence on tube feeds at discharge after index surgery. We successfully implemented an ultrasound-based screening protocol for VFMI and demonstrated improved screening, timeliness and high positive predictive value of ultrasound.

Patent foramen ovale in children: Unique pediatric challenges and lessons learned from adult literature.

A patent foramen ovale (PFO) is a frequent incidental finding during echocardiography in otherwise healthy children. In most healthy children with a diagnosis of isolated incidental PFO, no further follow-up or intervention is necessary. In some children, PFO is associated with certain clinical syndromes such as cryptogenic stroke, decompression sickness, migraine, and platypnea-orthodeoxia syndrome. This review discusses PFO anatomy, diagnostic imaging, PFO-associated clinical situations, management options, and the role of PFO in certain congenital heart disease. This review also highlights the current deficiency of pediatric data guiding management of these uncommon but important PFO-associated conditions. Future multicenter randomized controlled studies are necessary to guide the management of these unique and challenging PFO-associated conditions.

A longitudinal analysis of the relationship between housing insecurity and physical health among midlife and aging adults in the United States.

Rationale: A large body of work demonstrates the impact of housing instability on health by exploring the effects of evictions and homelessness on psychological wellbeing of young adults and children. However, limited research leverages national longitudinal data to examine whether and how experiences of a range of housing insecurity events, rather than just eviction or homelessness, affect physical health among midlife and older adults. Objective: The current study examines (1) prevalence of housing insecurity among midlife and older adults by age and race, (2) linkages between housing insecurity experiences and facets of physical health, and (3) age and race moderations on these effects. Method: This study employs regression models to examine whether experiences of housing insecurity affect self-rated physical health and chronic physical conditions among midlife and older adults (N = 2598) leveraging two waves of the National Study of Midlife in the United States (MIDUS). Results: Models revealed that housing insecurity experiences predicted poorer self-rated physical health and additional chronic conditions, even when controlling for previous physical health. Moderation analyses indicated that housing insecurity has a stronger relationship with chronic conditions among midlife adults as compared to older adults, and among Black adults as compared to white adults. These results suggest that experiences of housing insecurity leave adults vulnerable to compromised physical health, and that housing insecurity experiences may be particularly detrimental to the health of midlife Black adults. Conclusions: This research adds to the extant literature by introducing a comprehensive measure of housing insecurity experiences, and contributes to a life course perspective regarding how housing insecurity can affect physical health. This research has implications for policy that addresses housing insecurity as a public health concern, especially in the aftermath of the 2008 recession and the economic and housing crisis caused by the COVID-19 pandemic.

Estimating filling pressures in paediatric heart transplant recipients using echocardiographic parameters and B-type natriuretic peptide.

BACKGROUND: Longitudinal evaluation of allograft diastolic function in paediatric heart transplant recipients is important for early detection of acute rejection, cardiac allograft vasculopathy, and graft dysfunction. Mean diastolic right atrial and pulmonary capillary wedge pressures obtained at catheterisation are the reference standards for assessment. Echocardiography is non-invasive and more suitable for serial surveillance, but individual parameters have lacked accuracy. This study aimed to identify covariates of post-transplant mean right atrial and pulmonary capillary wedge pressures, including B-type natriuretic peptide and certain echocardiographic parameters. METHODS: A retrospective review of 143 scheduled cardiac catheterisations and echocardiograms from 56 paediatric recipients transplanted from 2007 to 2011 was performed. Samples with rejection were excluded. Univariate and multivariate linear regression models using backward selection were applied to a database consisting of B-type natriuretic peptide, haemodynamic, and echocardiographic data. RESULTS: Ln B-type natriuretic peptide, heart rate z-score, left ventricular end-diastolic dimension z-score, mitral E/e', and percent interventricular septal thickening in systole were independently associated with mean right atrial pressure. Ln B-type natriuretic peptide, heart rate z-score, left ventricular end-diastolic dimension z-score, left ventricular mass (observed/predicted), and mitral E/e' were independently associated with mean pulmonary capillary wedge pressure. Covariates of B-type natriuretic peptide included mean pulmonary artery and pulmonary capillary wedge pressures, height, haemoglobin, fractional shortening, percent interventricular septal thickening in systole, and pulmonary vascular resistance index. CONCLUSIONS: B-type natriuretic peptide and echocardiographic indices of diastolic function were independently related to post-transplant mean right atrial and pulmonary capillary wedge pressures in paediatric heart transplant recipients without rejection.

mRNA Coronavirus Disease 2019 Vaccine-Associated Myopericarditis in Adolescents: A Survey Study.

In this survey study of institutions across the US, marked variability in evaluation, treatment, and follow-up of adolescents 12 through 18 years of age with mRNA coronavirus disease 2019 (COVID-19) vaccine-associated myopericarditis was noted. Only one adolescent with life-threatening complications was reported, with no deaths at any of the participating institutions.

Echocardiographic assessment of right ventricular volume in repaired tetralogy of Fallot: a novel approach to an older technique.

BACKGROUND: In repaired tetralogy of Fallot (rTOF), right ventricular (RV) enlargement leads to poor outcomes. However, evaluating the RV has limitations; cardiac magnetic resonance (CMR) and 3D echocardiography have barriers including cost and accessibility. Traditional echocardiography is limited given the complex geometry and anterior location of the RV. We propose a novel echocardiographic evaluation of RV volume using 2 separate views. METHODS: This is a retrospective study of rTOF patients with echocardiogram, CMR, and exercise tests. By echocardiogram, we collected RV length in parasternal long axis (PLAX), area in 4-chamber (4C) view, and measurements per standard guidelines. RV end-diastolic and end-systolic volume (RVEDV and RVESV) were calculated as 5/9 (4C area * PLAX length). RESULTS: Forty-five patients with 66 sets of CMR, echocardiogram, and exercise tests were included (mean age 13.3 ± 3.2 years). The echocardiographic RVEDV and RVESV showed strong correlation with CMR parameters (r = 0.81 and 0.72; p≤ 0.0001), and moderate correlation with peak oxygen pulse (0.63 and 0.49; p≤0.0001). Guideline measurements had no significant correlation. Echocardiographic RVEDV and RVESV were higher in those requiring subsequent pulmonary valve replacement. Indexed echocardiographic RVEDV of 93 ml/m2 had 92% sensitivity and 50% specificity (area under curve 0.75 (p = 0.001)) in predicting CMR RV/LV EDV ratio > 2, which is an early indicator for pulmonary valve replacement. CONCLUSIONS: This novel technique correlates strongly with CMR, better than traditional parameters. While echocardiogram will not replace CMR, this method would be useful in predicting the RV volume, progression of dilation, and timing of CMR.

Health Care Policy and Congenital Heart Disease: 2020 Focus on Our 2030 Future.

The congenital heart care community faces a myriad of public health issues that act as barriers toward optimum patient outcomes. In this article, we attempt to define advocacy and policy initiatives meant to spotlight and potentially address these challenges. Issues are organized into the following 3 key facets of our community: patient population, health care delivery, and workforce. We discuss the social determinants of health and health care disparities that affect patients in the community that require the attention of policy makers. Furthermore, we highlight the many needs of the growing adults with congenital heart disease and those with comorbidities, highlighting concerns regarding the inequities in access to cardiac care and the need for multidisciplinary care. We also recognize the problems of transparency in outcomes reporting and the promising application of telehealth. Finally, we highlight the training of providers, measures of productivity, diversity in the workforce, and the importance of patient-family centered organizations in advocating for patients. Although all of these issues remain relevant to many subspecialties in medicine, this article attempts to illustrate the unique needs of this population and highlight ways in which to work together to address important opportunities for change in the cardiac care community and beyond. This article provides a framework for policy and advocacy efforts for the next decade.

Impact of Socioeconomic Status, Race and Ethnicity, and Geography on Prenatal Detection of Hypoplastic Left Heart Syndrome and Transposition of the Great Arteries.

BACKGROUND: Prenatal detection (PND) has benefits for infants with hypoplastic left heart syndrome (HLHS) and transposition of the great arteries (TGA), but associations between sociodemographic and geographic factors with PND have not been sufficiently explored. This study evaluated whether socioeconomic quartile (SEQ), public insurance, race and ethnicity, rural residence, and distance of residence (distance and driving time from a cardiac surgical center) are associated with the PND or timing of PND, with a secondary aim to analyze differences between the United States and Canada. METHODS: In this retrospective cohort study, fetuses and infants <2 months of age with HLHS or TGA admitted between 2012 and 2016 to participating Fetal Heart Society Research Collaborative institutions in the United States and Canada were included. SEQ, rural residence, and distance of residence were derived using maternal census tract from the maternal address at first visit. Subjects were assigned a SEQ z score using the neighborhood summary score or Canadian Chan index and separated into quartiles. Insurance type and self-reported race and ethnicity were obtained from medical charts. We evaluated associations among SEQ, insurance type, race and ethnicity, rural residence, and distance of residence with PND of HLHS and TGA (aggregate and individually) using bivariate analysis with adjusted associations for confounding variables and cluster analysis for centers. RESULTS: Data on 1862 subjects (HLHS: n=1171, 92% PND; TGA: n=691, 58% PND) were submitted by 21 centers (19 in the United States). In the United States, lower SEQ was associated with lower PND in HLHS and TGA, with the strongest association in the lower SEQ of pregnancies with fetal TGA (quartile 1, 0.78 [95% CI, 0.64-0.85], quartile 2, 0.77 [95% CI, 0.64-0.93], quartile 3, 0.83 [95% CI, 0.69-1.00], quartile 4, reference). Hispanic ethnicity (relative risk, 0.85 [95% CI, 0.72-0.99]) and rural residence (relative risk, 0.78 [95% CI, 0.64-0.95]) were also associated with lower PND in TGA. Lower SEQ was associated with later PND overall; in the United States, rural residence and public insurance were also associated with later PND. CONCLUSIONS: We demonstrate that lower SEQ, Hispanic ethnicity, and rural residence are associated with decreased PND for TGA, with lower SEQ also being associated with decreased PND for HLHS. Future work to increase PND should be considered in these specific populations.

Impact of nutritional status on prevalence of left ventricular hypertrophy in children undergoing liver transplant.

OBJECTIVE: We sought to (1) determine the prevalence of cardiac changes in patients with ESLD awaiting OLT (2) determine relationship between nutritional indices and cardiac changes. METHODS: Retrospective review of transthoracic ECHO, clinical and nutritional information of pediatric patients evaluated for OLT. ECHO was analyzed for LVH, defined as LVMI > 95 g/m2.7 and/or RWT > 0.42. These findings were correlated with age, ESLD etiology, growth and nutritional parameters as well as pre- and post-OLT. RESULTS: Sixty-five patients were included, all had normal left ventricular systolic function. Nine patients (14%) had LVMI > 95 g/m2.7 , five patients (8%) had RWT > 0.42, none met both criteria. None had thickened interventricular septal wall. Fourteen patients (20%) had significant left ventricular dilation. Nutritional deprivation was modestly present-weight under third percentile in 22%, length under third percentile in 24%, and both weight and length under third percentile in 17%. There were 12 patients (17%) with MUAC below two standard deviations for age; of these one had an elevated LVMI and another had an RWT > 0.42. CONCLUSIONS: In this contemporary cross-sectional evaluation, a smaller proportion of patients with ESLD had LVH in contrast to prior studies. Despite a comparable disease burden, our cohort had better nutritional status. Though there was a trend between nutritional and LVH indices, this correlation may be better assessed prospectively in a larger cohort.

Height Versus Body Surface Area to Normalize Cardiovascular Measurements in Children Using the Pediatric Heart Network Echocardiographic Z-Score Database.

Normalizing cardiovascular measurements for body size allows for comparison among children of different ages and for distinguishing pathologic changes from normal physiologic growth. Because of growing interest to use height for normalization, the aim of this study was to develop height-based normalization models and compare them to body surface area (BSA)-based normalization for aortic and left ventricular (LV) measurements. The study population consisted of healthy, non-obese children between 2 and 18 years of age enrolled in the Pediatric Heart Network Echo Z-Score Project. The echocardiographic study parameters included proximal aortic diameters at 3 locations, LV end-diastolic volume, and LV mass. Using the statistical methodology described in the original project, Z-scores based on height and BSA were determined for the study parameters and tested for any clinically significant relationships with age, sex, race, ethnicity, and body mass index (BMI). Normalization models based on height versus BSA were compared among underweight, normal weight, and overweight (but not obese) children in the study population. Z-scores based on height and BSA were calculated for the 5 study parameters and revealed no clinically significant relationships with age, sex, race, and ethnicity. Normalization based on height resulted in lower Z-scores in the underweight group compared to the overweight group, whereas normalization based on BSA resulted in higher Z-scores in the underweight group compared to the overweight group. In other words, increasing BMI had an opposite effect on height-based Z-scores compared to BSA-based Z-scores. Allometric normalization based on height and BSA for aortic and LV sizes is feasible. However, height-based normalization results in higher cardiovascular Z-scores in heavier children, and BSA-based normalization results in higher cardiovascular Z-scores in lighter children. Further studies are needed to assess the performance of these approaches in obese children with or without cardiac disease.

Validation of Prenatal Aortic Arch Angle Measurements in the Diagnosis of Neonatal Coarctation of the Aorta.

Prenatal prediction of coarctation of the aorta (CoA) is challenging. Methods identifying prenatal CoA have high sensitivity with significant false positives. We previously derived prenatal aortic arch angles for identifying CoA with high sensitivity and specificity and aim to validate these angles and compare them with a model utilizing ascending aorta (AAo) and isthmus (Aoi) measures. Retrospective case/cohort study of fetuses with prenatal suspicion for CoA. 35 fetuses were included. Measurements included: ascending-descending aortic angle (AAo.DAo), transverse-descending aortic angle (TAo.DAo); diameters and z-scores of Aoi from sagittal (Aoi-sag), three-vessel (Aoi-3VV) view and AAo. Discriminant functions for the 5 variables were compared using histograms and positive/negative predictive values (PPV/NPV). CoA was confirmed in 28/35 neonates. The PPV and NPV for angle measures were 100% and 77%. The AAo + Aoi-3VV model PPV and NPV were 92% and 80% and Aoi-sag + Aoi-3VV model were 82% and 71%. A linear discriminant model utilizing the 3 most predictive variables improved NPV to 90% and PPV to 100%. In conclusion, we validate that angle measures are superior to standard models of predicting CoA. An optimized 3 variable model maintains accuracy of identifying CoA while eliminating false positives.