Failure to thrive is common in infants with hypoplastic left heart syndrome and its variants and those with poor growth may be at risk for worse surgical and neurodevelopmental outcomes. The etiology of growth failure in this population is multifactorial and complex, but may be impacted by nutritional intervention. There are no consensus guidelines outlining best practices for nutritional monitoring and intervention in this group of infants. The Feeding Work Group of the National Pediatric Cardiology Quality Improvement Collaborative performed a literature review and assessment of best nutrition practices from centers participating in the collaborative in order to provide nutritional recommendations and levels of evidence for those caring for infants with single ventricle physiology.

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.

Myocardial dysfunction and coronary artery dilation have been reported in the acute setting of severe acute respiratory syndrome coronavirus disease-2-related multisystem inflammatory syndrome in children. Through a longitudinal echocardiographic single-center study of 15 children, we report the short-term outcomes of cardiac dysfunction and coronary artery dilation in severe acute respiratory syndrome coronavirus disease-2-related multisystem inflammatory syndrome in children.

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Background-Cardiac catheterization is routinely performed in patients with single ventricle before bidirectional Glenn operation (BDG). There is interest in noninvasive evaluation alone before BDG, but concern for echocardiography successfully imaging the relevant anatomy persists. We evaluated the accuracy of echocardiographic imaging of vascular anatomy. Methods and Results-Diagnostic images of 130 patients who had echocardiography and catheterization before BDG were reviewed; diameters of the pulmonary arteries (PAs) and aortic arch were measured, and stenoses were recorded. Patient and procedural factors associated with echocardiographic imaging were analyzed. Median age at echocardiography was 4 months; the most common diagnosis was hypoplastic left heart syndrome (55%

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Background Infants and young children frequently have difficulty remaining still for an echocardiogram, potentially leading to poor study quality, increasing the likelihood of diagnostic errors. Sedation is believed to improve echocardiography quality; however, its effectiveness has not been demonstrated. We hypothesized that sedation would improve study quality and reduce diagnostic errors. Methods We examined outpatient echocardiograms in children ≤ 36 months from January 2008 – June 2009. We collected variables related to image quality, report completeness and sedation use. Diagnostic errors were identified and categorized. Multivariable analysis identified the odds ratio (OR) and 95% confidence interval (CI) for risk factors for potentially preventable diagnostic errors and the impact of sedation on these errors. Results Among 2,003 echocardiograms, sedation was used in 498 (25%). The overall diagnostic error rate was 6.5%. Most errors (66%) were potentially preventable. Multivariable analysis identified the following risk factors for potentially preventable errors: pre-cardiac procedure (OR 2.19, 95% CI 1.05 to 4.59, P=0.04); moderate anatomic complexity (OR 3.91, 95% CI 2.25 to 6.81, P<0.001); and high anatomic complexity (OR 8.36; 95% CI 3.57 to 19.6, P<0.001). Sedation was independently associated with lower odds for potentially preventable diagnostic error (OR 0.47, 95% CI 0.27 to 0.80, P=0.006). Echocardiograms with sedation had fewer image quality concerns (22% vs. 60%) and fewer incomplete reports (3% vs. 20%) (P<0.001). Conclusions Most echocardiographic diagnostic errors among infants and young children are potentially preventable. Sedation is associated with a lower likelihood of these diagnostic errors, fewer imaging quality concerns, and fewer incomplete reports.