Abstract only Background: Diffuse myocardial fibrosis (DF) in adults with aortic stenosis (AS) is associated with heart failure, arrhythmias, and mortality; however, little is known about its role in ...younger patients with congenital AS. Study aims were to apply for the first time a recently validated cardiac magnetic resonance (CMR) extracellular volume fraction (Vec) measurement technique to quantify DF in children and young adults with AS and to explore associations between DF and indices of diastolic function. Methods: All pts who had a CMR with myocardial T1 measurement, were <30 yrs, and had congenital AS with a peak Doppler gradient ≥20 mm Hg at any time prior to the CMR were included. The Vec, a measure of DF, was calculated in the mid-LV by measuring T1 values for blood pool and myocardium before and after gadolinium with a modified Look-Locker technique, and adjusting for hematocrit. Vec results were compared to published values in healthy volunteers (n=14). Results: Thirty-five pts met inclusion criteria with a median age of 15 yrs (1.7-27), peak AS gradient of 36 mm Hg (0-90), and aortic regurgitation (AR) fraction by CMR of 25% (0-48). Vec was significantly greater in AS pts than volunteers (0.29 ± 0.06 (range, 0.22-0.48) vs. 0.25 ± 0.02 (range, 0.22-0.28), p<0.001) with 13 pts (37%) having an increased Vec (>0.28). Factors associated with higher Vec included any AS intervention (p=0.031), late gadolinium enhancement (p=0.028), and younger age at CMR (r=-0.37, p=0.031). Vec was not significantly associated with AS gradient, AR fraction, indexed LV mass, mass/volume ratio, or ejection fraction at the time of CMR, or a history of cardiac surgery. Increased Vec was significantly correlated with echo indices of diastolic dysfunction: E’ (r=-0.55, p=0.002), E/E’ (r=0.62, p<0.001), and indexed LA volume (r=0.56, p=0.001). In the 24 pts with contemporaneous cath data, Vec did not correlate with LV end-diastolic pressure (r=0.09, p=0.72). Conclusions: In this cohort of children and young adults with congenital AS, an elevated Vec indicating DF was present in approximately one-third. Increased Vec was associated with aortic valve interventions, late gadolinium enhancement, and echo indices of diastolic dysfunction. For congenital AS, therapies to reverse DF may be warranted.
Abstract only
Background:
Technical Performance Score (TPS), a tool based largely on the presence and magnitude of residua on postoperative echocardiograms (echo), has been used for assessing ...surgical repair and correlates with outcomes. The reproducibility of the echo measures that drive TPS classification has not been tested. We evaluated reader variability for echo components of TPS for tetralogy of Fallot (TOF) repair and arterial switch operation (ASO) in 2 centers and measured its effect on TPS.
Hypothesis:
Inter-reader echo measurement variability will not substantially impact TPS classification.
Methods:
Postoperative echos were evaluated in 67 subjects (39 TOF and 28 ASO). Two readers (1 per center) read each echo, blinded to center of origin. To assess intra-reader variability, 25% of echoes were re-reviewed by each reader. Measurements between readers were compared with Intra-class correlation (ICC). TPS Class (1 Optimal
no residua
, 2 Adequate
minor residua
, 3 Inadequate
major residua
) was assigned for each echo review by an independent investigator. The impact of measurement variability on overall TPS variability was compared using weighted Kappa (K) and % raw agreement.
Results:
ICC was highest for Doppler velocity data and lower for measurements of small linear structures such as septal defects and vena contracta
Figure.
Overall TPS demonstrated good agreement (between reader TOF K = 0.82 and ASO K = 0.81). The 2 readers were concordant for TPS Class for 53 subjects (79%) and discordant for Classes 2 vs. 3 in 6 (9%); no readings were discordant between Classes 1 and 3
Table
.
Conclusions:
Although overall TPS demonstrated good agreement, inter-reader variation for echo measurements had a small, but important effect on TPS for ASO and TOF, particularly for the distinction between minor and major residua. Future studies of generalizability and reproducibility of TPS across centers and lesions are needed before TPS could be adopted as a national quality measure.
Multisystem inflammatory syndrome in children (MIS-C) is a severe complication of SARS-CoV-2 infection. It remains unclear how MIS-C phenotypes vary across SARS-CoV-2 variants. We aimed to ...investigate clinical characteristics and outcomes of MIS-C across SARS-CoV-2 eras.
We performed a multicentre observational retrospective study including seven paediatric hospitals in four countries (France, Spain, U.K., and U.S.). All consecutive confirmed patients with MIS-C hospitalised between February 1st, 2020, and May 31st, 2022, were included. Electronic Health Records (EHR) data were used to calculate pooled risk differences (RD) and effect sizes (ES) at site level, using Alpha as reference. Meta-analysis was used to pool data across sites.
Of 598 patients with MIS-C (61% male, 39% female; mean age 9.7 years SD 4.5), 383 (64%) were admitted in the Alpha era, 111 (19%) in the Delta era, and 104 (17%) in the Omicron era. Compared with patients admitted in the Alpha era, those admitted in the Delta era were younger (ES −1.18 years 95% CI −2.05, −0.32), had fewer respiratory symptoms (RD −0.15 95% CI −0.33, −0.04), less frequent non-cardiogenic shock or systemic inflammatory response syndrome (SIRS) (RD −0.35 95% CI −0.64, −0.07), lower lymphocyte count (ES −0.16 × 109/uL 95% CI −0.30, −0.01), lower C-reactive protein (ES −28.5 mg/L 95% CI −46.3, −10.7), and lower troponin (ES −0.14 ng/mL 95% CI −0.26, −0.03). Patients admitted in the Omicron versus Alpha eras were younger (ES −1.6 years 95% CI −2.5, −0.8), had less frequent SIRS (RD −0.18 95% CI −0.30, −0.05), lower lymphocyte count (ES −0.39 × 109/uL 95% CI −0.52, −0.25), lower troponin (ES −0.16 ng/mL 95% CI −0.30, −0.01) and less frequently received anticoagulation therapy (RD −0.19 95% CI −0.37, −0.04). Length of hospitalization was shorter in the Delta versus Alpha eras (−1.3 days 95% CI −2.3, −0.4).
Our study suggested that MIS-C clinical phenotypes varied across SARS-CoV-2 eras, with patients in Delta and Omicron eras being younger and less sick. EHR data can be effectively leveraged to identify rare complications of pandemic diseases and their variation over time.
None.
Multisystem inflammatory syndrome in children (MIS-C) consensus criteria were designed for maximal sensitivity and therefore capture patients with acute COVID-19 pneumonia.
We performed unsupervised ...clustering on data from 1,526 patients (684 labeled MIS-C by clinicians) <21 years old hospitalized with COVID-19-related illness admitted between 15 March 2020 and 31 December 2020. We compared prevalence of assigned MIS-C labels and clinical features among clusters, followed by recursive feature elimination to identify characteristics of potentially misclassified MIS-C-labeled patients.
Of 94 clinical features tested, 46 were retained for clustering. Cluster 1 patients (N = 498; 92% labeled MIS-C) were mostly previously healthy (71%), with mean age 7·2 ± 0·4 years, predominant cardiovascular (77%) and/or mucocutaneous (82%) involvement, high inflammatory biomarkers, and mostly SARS-CoV-2 PCR negative (60%). Cluster 2 patients (N = 445; 27% labeled MIS-C) frequently had pre-existing conditions (79%, with 39% respiratory), were similarly 7·4 ± 2·1 years old, and commonly had chest radiograph infiltrates (79%) and positive PCR testing (90%). Cluster 3 patients (N = 583; 19% labeled MIS-C) were younger (2·8 ± 2·0 y), PCR positive (86%), with less inflammation. Radiographic findings of pulmonary infiltrates and positive SARS-CoV-2 PCR accurately distinguished cluster 2 MIS-C labeled patients from cluster 1 patients.
Using a data driven, unsupervised approach, we identified features that cluster patients into a group with high likelihood of having MIS-C. Other features identified a cluster of patients more likely to have acute severe COVID-19 pulmonary disease, and patients in this cluster labeled by clinicians as MIS-C may be misclassified. These data driven phenotypes may help refine the diagnosis of MIS-C.
This work was funded by the US Centers for Disease Control and Prevention (75D30120C07725) and National Institutes of Health (K12HD047349 and R21HD095228).
Background Heart size and function in children with single right ventricle (RV) anomalies may be influenced by shunt type at the Norwood procedure. We sought to identify shunt-related differences ...during early childhood after staged surgical palliations using echocardiography. Methods We compared echocardiographic indices of RV, neoaortic, and tricuspid valve size and function at 14 months, pre-Fontan, and 6 years in 241 subjects randomized to a Norwood procedure using either the modified Blalock-Taussig shunt or RV-to-pulmonary-artery shunt. Results At 6 years, the shunt groups did not differ significantly in any measure except for increased indexed neoaortic area in the modified Blalock-Taussig shunt. RV ejection fraction improved between pre-Fontan and 6 years in the RV-to-pulmonary artery shunt group but was stable in the modified Blalock-Taussig shunt group. For the entire cohort, RV diastolic and systolic size and functional indices were improved at 6 years compared with earlier measurements, and indexed tricuspid and neoaortic annular area decreased from 14 months to 6 years. The prevalence of ≥moderate tricuspid and neoaortic regurgitation was uncommon and did not vary by group or time period. Diminished RV ejection fraction at the 14-month study was predictive of late death/transplant; the hazard of late death/transplant when RV ejection fraction was <40% was tripled (hazard ratio, 3.18; 95% CI, 1.41-7.17). Conclusions By 6 years after staged palliation, shunt type has not impacted RV size and function, and RV and valvar size and function show beneficial remodeling. Poor RV systolic function at 14 months predicts worse late survival independent of the initial shunt type. Clinical Trial Registration URL: https://www.clinicaltrials.gov . Unique identifier: NCT00115934.
Surgical intervention for children with congenital cardiac disease in the developing world often occurs late. Our objective was to identify factors that placed Guatemalan children at risk for delayed ...care.
We investigated the medical and socioeconomic background of 178 children under the age of 18 years who received their first corrective surgery for congenital cardiac disease at the Unidad de Cirugía Cardiovascular de Guatemala in 2002. A retrospective review of medical records was performed. Each case was stratified into one of three surgical classes based upon customary practice in the United States of America. The outcome we measured was age at surgery, adjusting for the surgical class. Logistic regression was performed and odds ratios calculated.
In univariate analyses, patients presented later for surgery if they were from rural areas (p equals 0.001), did not have social security membership (p equals 0.004), or paid any amount towards the cost of their surgery (p less than 0.001). Age at surgery was also positively correlated with the distance of the home of the patient from the surgical centre (p equals 0.002). For the subset of patients who applied for financial assistance, we found that children presented later for surgery if they required institutional support (p equals 0.001), or came from households of larger size (p less than 0.001).
Guatemalan children with congenital cardiac disease may be at risk for delayed surgical care if they come from rural areas, areas distant from the surgical centre, or from families without membership of social security.
Data are limited regarding adverse reactions after COVID-19 vaccination in patients with a history of multisystem inflammatory syndrome in children (MIS-C). The lack of vaccine safety data in this ...unique population may cause hesitancy and concern for many families and health care professionals.
To describe adverse reactions following COVID-19 vaccination in patients with a history of MIS-C.
In this multicenter cross-sectional study including 22 North American centers participating in a National Heart, Lung, and Blood Institute, National Institutes of Health-sponsored study, Long-Term Outcomes After the Multisystem Inflammatory Syndrome in Children (MUSIC), patients with a prior diagnosis of MIS-C who were eligible for COVID-19 vaccination (age ≥5 years; ≥90 days after MIS-C diagnosis) were surveyed between December 13, 2021, and February 18, 2022, regarding COVID-19 vaccination status and adverse reactions.
COVID-19 vaccination after MIS-C diagnosis.
The main outcome was adverse reactions following COVID-19 vaccination. Comparisons were made using the Wilcoxon rank sum test for continuous variables and the χ2 or Fisher exact test for categorical variables.
Of 385 vaccine-eligible patients who were surveyed, 185 (48.1%) received at least 1 vaccine dose; 136 of the vaccinated patients (73.5%) were male, and the median age was 12.2 years (IQR, 9.5-14.7 years). Among vaccinated patients, 1 (0.5%) identified as American Indian/Alaska Native, non-Hispanic; 9 (4.9%) as Asian, non-Hispanic; 45 (24.3%) as Black, non-Hispanic; 59 (31.9%) as Hispanic or Latino; 53 (28.6%) as White, non-Hispanic; 2 (1.1%) as multiracial, non-Hispanic; and 2 (1.1%) as other, non-Hispanic; 14 (7.6%) had unknown or undeclared race and ethnicity. The median time from MIS-C diagnosis to first vaccine dose was 9.0 months (IQR, 5.1-11.9 months); 31 patients (16.8%) received 1 dose, 142 (76.8%) received 2 doses, and 12 (6.5%) received 3 doses. Almost all patients received the BNT162b2 vaccine (347 of 351 vaccine doses 98.9%). Minor adverse reactions were observed in 90 patients (48.6%) and were most often arm soreness (62 patients 33.5%) and/or fatigue (32 17.3%). In 32 patients (17.3%), adverse reactions were treated with medications, most commonly acetaminophen (21 patients 11.4%) or ibuprofen (11 5.9%). Four patients (2.2%) sought medical evaluation, but none required testing or hospitalization. There were no patients with any serious adverse events, including myocarditis or recurrence of MIS-C.
In this cross-sectional study of patients with a history of MIS-C, no serious adverse events were reported after COVID-19 vaccination. These findings suggest that the safety profile of COVID-19 vaccination administered at least 90 days following MIS-C diagnosis appears to be similar to that in the general population.
BackgroundIn the SVR trial, 1-year (y) transplant (tx)-free survival was better for the Norwood procedure with right ventricle-to-pulmonary artery shunt (RVPAS) vs modified Blalock-Taussig shunt ...(MBTS). At 6 y, we compared tx-free survival, unplanned interventions, morbidities, New York Heart Association (NYHA) Class, and RV ejection fraction (RVEF) by assigned shunt.Methods and ResultsThe SVR trial treated 549 pts. Vital status and medical history were ascertained annually. Tx-free survival in the RVPAS (63.5%) vs MBTS (58.7%) groups did not differ (Figure; log-rank P=.13). Similarly, neither mortality nor tx alone differed by shunt type. By 6 y, RVPAS pts had a higher incidence of any catheter intervention (.38 vs .23/pt-yr, P<.001), balloon angioplasty (P=.014), stent (P=.009), and coiling (P<.001). The % of pts with morbidities by 6 y were similar in the groups, with overall ratespacemaker 3%, thrombosis 16%, stroke 7%, seizures 13%, protein losing enteropathy 3%, plastic bronchitis 0.5%, and 6-y NYHA Class I 71%, II 21%, III 3%, and IV 5%. Among pre-specified subgroups, worse tx-free survival was associated with low birth weight (<2500 g); worse pre-Norwood tricuspid regurgitation (≥2.5 mm jet width); lower surgeon Norwood volume; preterm birth (<37 wks); and combined aortic atresia and pre-term birth (all P<.01). Subgroup x shunt interaction was significant only for surgeon volume levels (P<.05); in the highest volume group (n >15/y), the MBTS was beneficial (P<.04), and in 3 lower volume groups, the RVPAS was qualitatively better. In 6-y echoes read to date, RVEF was similar in the RVPAS vs MBTS groups (46±7, n=55 vs 46±6%, n=48; P=.9).ConclusionsBy 6 y, tx-free survival was an absolute 4.8% higher for pts assigned to the RVPAS vs MBTS group, but the difference no longer reached statistical significance, and they needed more catheter interventions. Rates of death, tx, and morbidities; distribution of NYHA Class; and RVEF were each similar in the shunt groups.
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