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.

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.

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.

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.

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.

Accuracy of Fetal Echocardiography in Defining Pulmonary Artery Anatomy and Source of Pulmonary Blood flow in Pulmonary Atresia with Ventricular Septal Defect (PA/VSD).

Precise delineation of central and branch pulmonary artery anatomy, patent ductus arteriosus, and major aorto-pulmonary collateral artery anatomy in the fetal diagnosis of pulmonary atresia with ventricular septal defect is challenging but important to prenatal counseling and postnatal management. We aimed to evaluate the accuracy of fetal echocardiography to determine these anatomical nuances in pulmonary atresia with ventricular septal defect. This was a retrospective, single-institution, 10-year chart review of consecutive prenatal diagnosis of pulmonary atresia with ventricular septal defect for assessment of pulmonary artery, patent ductus arteriosus, and major aorto-pulmonary collateral artery anatomy and comparison with postnatal imaging including echocardiography, cardiac catheterization, and computerized tomography angiography. Twenty-six fetuses were diagnosed with pulmonary atresia with ventricular septal defect during the review period and complete postnatal follow-up was available in 18, all confirming the basic prenatal diagnosis. Fetal echocardiography accurately predicted central and branch pulmonary artery anatomy in 16 (89%) [confluent in 14, discontinuous in 2], patent ductus arteriosus status in 15 (83%) [present in 10, absent in 5], and major aorto-pulmonary collateral arteries in 17 (94%) [present in 9, absent in 8]. Accuracy increased to 100% for pulmonary artery anatomy (16/16) and major aorto-pulmonary collateral artery (17/17) when excluding patients whose anatomy was reported as uncertain on fetal echocardiography. Fetal echocardiography can provide accurate anatomical details in the vast majority of fetuses with pulmonary atresia with ventricular septal defect. This allows for more anatomy-specific counseling, prognostication, and improved selection of postnatally available management options.

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.

Multimodality and multiterritory vascular imaging in a toddler with acquired, complex, and unusual aortic arch anatomy.

A 20-month-old otherwise well child with no cardiac history presented for evaluation of a recent onset cardiac murmur. His initial examination revealed significant right carotid bruit along with a transthoracic echocardiogram (ECHO) concerning for severe transverse arch hypoplasia with unusual appearance and left ventricular hypertrophy with preserved function. This report demonstrates the utility of various modalities including echo, CT, and MRI to assess vascular structures in different vascular territories in a challenging patient who underwent a satisfactory arch repair with unexpected pathology findings.

Feasibility and interpretation of global longitudinal strain imaging in pediatric heart transplant recipients.

Evaluation of myocardial mechanics after heart transplant is important in monitoring allograft function and identifying rejection. Speckle tracking global longitudinal strain (GLS) may be more sensitive to early regional changes from rejection. This study aimed to determine feasibility of GLS in pediatric hearts during surveillance echocardiograms, compare their GLS to published norms (-18% to -22%), and assess association of GLS with other indices of graft function. Retrospective review of transplant echocardiograms from 2013 to 2014. Philips QLAB was used for post-acquisition GLS analysis. Multiple linear regression was used to assess the association of GLS with echocardiographic/catheterization indices, and B-type natriuretic peptide (BNP). Forty-seven patients (84 studies) were included. Calculation of GLS was feasible in 82 studies (97%) with inter- and intra-observer variability of 0.71 and 0.69. Patients (n=9) with rejection had GLS of -16.4% (SD=3.5%) compared to those without [-16.8% (SD=3.7%)]. GLS worsened linearly with increasing Ln(BNP) (P=<.001), left ventricular volume in diastole (P=<.001), septal a' wave (P=<.001), and pulmonary capillary wedge pressure (P=<.001). Speckle tracking-based GLS is feasible and reproducible in pediatric heart recipients and is reduced at baseline. The role of GLS and BNP in detecting early systolic dysfunction warrants further investigation.

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.

Diagnosis of tetralogy of Fallot and its variants in the late first and early second trimester: details of initial assessment and comparison with later fetal diagnosis.

OBJECTIVE: We sought to evaluate the completeness of echocardiographic diagnosis of fetal tetralogy of Fallot (fTOF) at 12-17 weeks gestation, and compare assessment and clinical outcomes to diagnoses made at >17 weeks gestation. METHODS: We identified all fTOF diagnoses made in our experience from 2003 to 2008. Referral indication, anatomic detail by echocardiography and pregnancy outcomes were compared between fetuses diagnosed at ≤ 17 weeks (Group I) and >17 weeks gestation (Group II). A 10-point scoring tool was applied retrospectively to the echocardiograms at initial diagnosis (1 point each was ascribed to visualization of right ventricular outflow obstruction, pulmonary valve, pulmonary arteries including dimensions, pulmonary arterial flow, systemic and pulmonary venous anatomy, atrioventricular valves, ductus arteriosus, ductus flow, aortic arch morphology, sidedness and flow). RESULTS: There were 10 pregnancies in Group I (12-17 weeks) and 25 in Group II (mean gestation at diagnosis 23.5 ± 5.7). The most common reason for referral was extracardiac pathology in Group I (80%) and suspected fetal heart disease on obstetric ultrasound in Group II (64%). Transabdominal imaging was adequate in about half of Group I studies. Mean anatomic diagnosis score in Group I was 6.1(range 2.5-9) and Group II was 8.4 (range 6.5-10). Elective pregnancy termination occurred in 80% in Group I and 33% in Group II. CONCLUSIONS: fTOF can be diagnosed in first and early second trimesters with detailed anatomic assessment possible in most. Referral indication and pregnancy outcome differ considerably between early and later prenatal diagnosis of fTOF.

Partial anomalous left pulmonary artery along with aortic coarctation in an infant with Kabuki syndrome.

We report an antenatally diagnosed fetal coarctation delivered prematurely and confirmed to have coarctation as well as additional nonobstructing anomalous left pulmonary artery branch (aLPA) from right pulmonary artery (RPA) and significant dysmorphic features. The baby underwent an uncomplicated arch repair, but had numerous multisystem and growth related issues prompting a diagnosis of Kabuki syndrome (KS) at 1 year of age. While coarctation and hypoplastic left heart syndrome are observed in this syndrome, this is the second reported case of aLPA in KS and the first with the forme fruste of left-sided obstruction as well as aLPA in this group of patients.