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Pulmonary balloon valvuloplasty (PBV) is the treatment of choice for subjects with isolated pulmonary valve stenosis (IPS). The purpose of this study was to define fetal echocardiographic features associated with an inpatient PBV prior to newborn hospital discharge and characterize resource utilization of IPS fetuses among participating centers. Six center, retrospective case series of singleton fetuses identified between 2010 and 2020 with IPS. Third-trimester echocardiogram data was compared with postnatal data, included pulmonary valve Doppler velocities, pulmonary valve insufficiency and ductus arteriosus flow direction. Comparison between subjects who underwent inpatient PBV during their newborn hospital admission versus those infants referred for outpatient PBV after initial hospital discharge. We analyzed data by logistic regression, student t test and Chi-Square testing with a p value of ≤ 0.05 considered statistically significant. Forty-nine IPS fetuses were identified. Thirty-eight (78%) underwent inpatient PBV at 5 (range 1–58) days and 11 (22%) underwent outpatient PBV at 51.8 (11–174) days. Newborns requiring an inpatient PBV were more likely to have one or more characteristics on 3rd-trimester fetal echocardiogram: left to right or bidirectional ductus arteriosus flow (61% vs 0%), and/or a peak pulmonary valve velocity > 3.0 m/s (odds ratio 16.9, 95% confidence interval 3.02–94.17) with a sensitivity of 90.4% and specificity of 97.7%. Ductus arteriosus flow direction and pulmonary valve peak velocity in the 3rd trimester can successfully predict the need for newborn inpatient PBV. We speculate these findings may be useful in choosing delivery site for the pregnancy complicated by fetal IPS. [ABSTRACT FROM AUTHOR]

Infants with congenital heart disease (CHD) often require supplemental nutrition via tube feeding, even after corrective surgical repair. The need for tube feeding can persist months after discharge home, and outpatient weaning from the tube can be a slow and difficult process. Lack of consensus exists in the literature as to the best approach to this challenge. We describe a single institution's initiative to decrease tube dependency after discharge through an intensive, telehealth-centric program for children with CHD. Of 16 children aged 1–26 months, 94% were able to be successfully tube weaned at home, with median time to tube-free of 12 days. No significant weight loss was noted during or after the wean process. Longer tube wean time was associated with older age at onset and comorbid genetic syndrome. Our telehealth-based intensive tube weaning program was successful but required a multidisciplinary team, targeted pre-participation patient evaluation, and frequent communication. [ABSTRACT FROM AUTHOR]

In patients with surgically repaired tetralogy of Fallot (TOF), reported peak oxygen consumption (VO) is decreased compared with control subjects. The measurement of exercise cardiac output (CO) could be a useful adjunct for assessing cardiovascular fitness. There are few data assessing noninvasive CO, cardiac index (CI), and stroke volume (SV) during exercise for these patients. This study sought to measure noninvasive CI and SV during rest and exercise in children with repaired TOF. The authors compared 21 asymptomatic children with repaired TOF ages 11-17 years during rest and exercise and 42 gender- and age-matched healthy control children without structural heart disease. Using a Bruce exercise protocol, exercise data were measured noninvasively by a novel inert gas rebreathing technique including peak duration and heart rate, as well as VO, CO, CI, and SV measured at 90 % of peak predicted theoretical heart rate (90 % ppHR). Statistical correlation between peak VO and CI was performed. At baseline, there was no statistically significant difference in any of the measures between the groups. At 90 % ppHR, there was an increase in CI during exercise of 140 % in the TOF children and 180 % in the control children. During exercise, SV changed minimally in the patient group, whereas it increased more than 30 % in the control children. At 90 % ppHR, the patient group showed an increase in VO during exercise similar to that of their healthy peers. The patients had a significantly shorter peak exercise duration than normal control subjects. The patients had a lower CI during exercise because they were less able to increase SV. Therefore, at similar heart rates, patients who have had TOF repair must rely on increased peripheral muscle extraction, with a higher arteriovenous oxygen difference (SaO-MvO) during exercise, which may limit peak exercise capacity. In this cohort of TOF patients, noninvasive CI measurement was feasible, and correlation with VO was good. [ABSTRACT FROM AUTHOR]

Infants and children with congenital heart disease (CHD) often require supplemental nutrition via tube feeding before and after surgery. Tube feeding may be required due to poor weight gain, inadequate oral-motor skills, and/or reduced endurance for oral feeding. Our team has described a successful approach to weaning this population (Horsley et al. (2022) Pediatr Cardiol 43:1429–1437). A subgroup of tube-fed patients with CHD who demonstrate oral feeding aversion (OA) presents unique challenges to the tube weaning process. We discuss our team’s experience with tube weaning orally averse children with CHD. Of 36 patients enrolled in the Cincinnati Children’s Heart Institute Cardiology Feeding Tube Wean Clinic, 11 (31%) were determined to have oral feeding aversion (OA) by a speech-language pathologist (SLP). Descriptive data comparing the OA group to the non-averse group was gathered prior to and during the wean. Both groups had the ability to tube wean successfully, although the OA group had lower median oral intake (9 vs 24%) and higher age at start of the wean (9 vs 4 months). Those with OA also had a longer median duration of wean in days (17 vs 12 days) and higher likelihood of return to tube feeds within six-month post-wean (22 vs 0%). Additionally, the OA group had a higher percentage of genetic syndromes (36 vs 16%), although this was not found to be statistically significant in this study. Children with OA present with unique challenges for tube weaning. The results of this study show that weaning children with CHD and OA is possible with a multidisciplinary team who is knowledgeable about this population. [ABSTRACT FROM AUTHOR]

Cardiac dysfunction is a leading cause of morbidity and mortality in Duchenne muscular dystrophy (DMD). Left atrial (LA) function is a poorly understood concept in this patient population, and research suggests underlying structural changes that could affect atrial function. Cardiac magnetic resonance (CMR) imaging may provide an important non-invasive approach to evaluating LA function. This study was a single center retrospective review of consecutive CMR studies over a 1 year period comparing LA phasic function within a cohort of DMD patients, and to those with structurally and functionally normal hearts. LA strain measurements including global reservoir, conduit, boost-pump strain, and LA volumes were obtained retrospectively. Spearman correlation analyses were performed on atrial strain measurements. 107 DMD and 79 normal CMR studies were included. The DMD cohort had worse systolic function (p < 0.001), smaller indexed max LA and left ventricular (LV) volumes (p < 0.001), and greater LA emptying fraction (p < 0.001). In the DMD cohort, emptying fraction decreased with advanced patient age (p < 0.001) and diminishing systolic function (p < 0.001). DMD patients with moderate or severe LV dysfunction demonstrated lower LA emptying fraction (p = 0.002), more impaired 2-chamber LA reservoir (p = 0.003), and LA pump (p = 0.006) and conduit strain (p = 0.018). DMD patients with preserved function have lower indexed LA volumes with higher LA emptying fractions than controls. Progression of disease and age is associated with decreased LA emptying fraction with early manifestations in reservoir and conduit strain. These findings suggest that strain markers of LA compliance and early left ventricular relaxation are associated with worsening cardiomyopathy in the DMD population. [ABSTRACT FROM AUTHOR]

Despite improvement in hemodynamics, children with single ventricle heart disease remain on feeding tubes long after stage 2 palliation (S2P). Use of a hunger provocation method in a multidisciplinary team setting has been successful at weaning these children from feeding tubes. The objective of this study is to describe patient characteristics and outcomes in the single ventricle population who underwent a formal tube weaning process using a standardized hunger provocation method. Single ventricle patients after S2P from six centers were included. Patient data collected included baseline demographics, swallow evaluation results, and feeding characteristics such as percent oral intake at the start of tube wean. Tube wean data included tube weaning process and duration, interruptions to the tube wean, adverse events, and weights before, during, and after the tube wean. 94% (60 of 64) of patients achieved oral independence. The median time to tube wean was 12.5 days. 62% of patients had transient weight loss during the tube wean. 61% of the cohort was taking less than 10% goal volumes by mouth with 90% of those patients successfully tube weaned. All patients with history of aspiration were successfully tube weaned. 75% of successfully weaned patients were above baseline weight at 1-month post-tube wean. The most common cause of tube wean interruption was contraction of a viral illness. Use of a standardized tube weaning process via hunger provocation method is both feasible and safe in the single ventricle population, resulting in successful feeding tube removal in a timely manner with minimal adverse effects. [ABSTRACT FROM AUTHOR]

Infants with heart disease are at high risk of feeding difficulties and complications. Feeding practices amongst acute care cardiology units are not standardized. This study aims to describe feeding practices for infants at the time of discharge from a Pediatric Acute Care Cardiology Collaborative (PAC3) center and practice variation between centers. Discharge encounters for infants in the PAC3 registry between February 2019 and October 2021 were included. Nutrition type and feeding route at discharge were summarized with descriptive statistics and a modified bump plot. Center variation was assessed using funnel plots with control limits set at the 99.9% confidence interval from the group mean. A total of 15,414 encounters across 24 PAC3 centers were recorded from 8313 unique patients (median encounters 1, range 1–25). Nutrition at discharge consisted of standard formula in 8368 (54%), human milk in 6300 (41%), and elemental formula in 3230 (21%), either alone or in combination. Feeds were fortified to ≥ 24 kcal/oz in 12,359 (80%). Discharge supplemental tube feeding was present in 7353 (48%) encounters with 4643 (63%) receiving continuous feeds, 2144 (29%) bolus feeds, and 566 (8%) a combination. Funnel plots demonstrated variability in nutrition type and feeding route at discharge. Infants with heart disease commonly require high calorie nutrition and supplemental tube feedings at discharge. Feeding strategies at discharge vary widely between PAC3 centers. Collaborative approaches to identify best practices in feeding strategies are needed. [ABSTRACT FROM AUTHOR]

Infants with congenital heart disease (CHD) are at risk for developing both benign hematochezia and necrotizing enterocolitis (NEC). Despite these risks there are very few studies that investigate modifiable risk factors such as feeding practices. It remains unclear what feeding practices should be avoided due to higher incidence of CHD-NEC. We aim to assess the feeding practices across three high volume tertiary centers to establish a relationship between various feeding practices and development of NEC. A multicenter retrospective review of feeding practices at the time of documented hematochezia event that occurred between 1/2019 and 1/2021 in infants with CHD who were less than 6 months of age. NEC was defined as Bells Stage 2 or greater. Age, weight, ventricular morphology, primary diagnoses, feeding route, feed change, and formula type were evaluated. 176 hematochezia events occurred in 121 patients, 72% of these events were considered benign hematochezia with the remaining 28% being true NEC. Single ventricle (SV) physiology (p < 0.05), younger age, < 45 days of life, (p < 0.001), and feeding route were statistically associated with true NEC (p < 0.01). Formula type and recent change in feed administration were not associated with NEC. The caloric density of feeds at the time of hematochezia was nearing significance. The majority of hematochezia events are benign in nature, however, there should be heightened awareness in patients who are SV, younger in age, and those who are post-pylorically fed. There may be some risk in using higher caloric density feeds (> 24 kcal/oz), however, additional research is needed to fully establish this relationship. [ABSTRACT FROM AUTHOR]