Management of Hematochezia in Infants with Congenital Heart Disease Admitted to the Acute Care Cardiology Unit: A Multicenter Retrospective Pilot Study.

OBJECTIVE: To assess the evaluation and prevalence of benign hematochezia (BH) vs necrotizing enterocolitis (NEC) in infants with congenital heart disease (CHD) <6 months old admitted to the acute care cardiology unit. STUDY DESIGN: This was a multicenter retrospective review of patient characteristics and evaluation of all hematochezia events in patients with CHD <6 months admitted to acute care cardiology unit at 3 high-volume tertiary care centers from February 2019 to January 2021. NEC was defined by the Bell staging criteria. Patients with gastrointestinal disorders were excluded. RESULTS: In total, 180 hematochezia events occurred in 121 patients; 42 patients had more than 1 event. In total, 61% of affected patients had single-ventricle physiology (38% hypoplastic left heart syndrome). Median age and weight at hematochezia were 38 days (IQR 24, 79) and 3.7 kg (IQR 3.2, 4.4). In total, 77% of hematochezia events were BH, and 23% were NEC. There were no surgical interventions for NEC or deaths from NEC. Those with NEC were significantly younger (34 vs 56 days, P < .01) and smaller (3.7 vs 4 kg, P < .01). Single-ventricle physiology was significantly associated with NEC. Initial bloodwork and diagnostic imaging at each center were assessed. There was no significant difference in white blood cell count or C-reactive protein in those with NEC compared with BH. Blood culture results were all negative. CONCLUSIONS: The majority of infants with CHD with hematochezia have BH over NEC, although single-ventricle and surgical patients remain at greater risk. Infants <45 days are more vulnerable for developing NEC. Bloodwork was noncontributory in the identification of cardiac NEC. Expansion to a prospective study to develop a treatment algorithm is important to avoid overtreatment.

Experiences in Tube Weaning Children with Congenital Heart Disease and Oral Feeding Aversion.

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.

Feeding Practices in Infants with Hematochezia and Necrotizing Enterocolitis on Acute Care Cardiology Units.

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.

Structured Tube Weaning Using the Hunger Provocation Method in Infants with Single Ventricle Heart Defects: A Multicenter Study.

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.

Feeding Variation Among Infants in Acute Care Cardiology Units.

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.

Prenatal Predictors for Pulmonary Balloon Valvuloplasty in the Newborn.

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.

Paediatric resident identification of cardiac emergencies.

OBJECTIVES: Critical CHD is associated with morbidity and mortality, worsened by delayed diagnosis. Paediatric residents are front-line clinicians, yet identification of congenital CHD remains challenging. Current exposure to cardiology is limited in paediatric resident education. We evaluated the impact of rapid cycle deliberate practice simulation on paediatric residents' skills, knowledge, and perceived competence to recognise and manage infants with congenital CHD. METHODS: We conducted a 6-month pilot study. Interns rotating in paediatric cardiology completed a case scenario assessment during weeks 1 and 4 and participated in paired simulations (traditional debrief and rapid cycle deliberate practice) in weeks 2-4. We assessed interns' skills during the simulation using a checklist of "cannot miss" tasks. In week 4, they completed a retrospective pre-post knowledge-based survey. We analysed the data using summary statistics and mixed effect linear regression. RESULTS: A total of 26 interns participated. There was a significant increase in case scenario assessment scores between weeks 1 and 4 (4, interquartile range 3-6 versus 8, interquartile range 6-10; p-value < 0.0001). The percentage of "cannot miss" tasks on the simulation checklist increased from weeks 2 to 3 (73% versus 83%, p-value 0.0263) and from weeks 2-4 (73% versus 92%, p-value 0.0025). The retrospective pre-post survey scores also increased (1.67, interquartile range 1.33-2.17 versus 3.83, interquartile range 3.17-4; p-value < 0.0001). CONCLUSION: Rapid cycle deliberate practice simulations resulted in improved recognition and initiation of treatment of simulated infants with congenital CHD among paediatric interns. Future studies will include full implementation of the curriculum and knowledge retention work.

Initiative to increase family presence and participation in daily rounds on a paediatric acute care cardiology unit.

INTRODUCTION: Family-centred rounds benefit families and clinicians and improve outcomes in general paediatrics, but are understudied in subspecialty settings. We sought to improve family presence and participation in rounds in a paediatric acute care cardiology unit. METHODS: We created operational definitions for family presence, our process measure, and participation, our outcome measure, and gathered baseline data over 4 months of 2021. Our SMART aim was to increase mean family presence from 43 to 75% and mean family participation from 81 to 90% by 30 May, 2022. We tested interventions with iterative plan-do-study-act cycles between 6 January, 2022 and 20 May, 2022, including provider education, calling families not at bedside, and adjustment to rounding presentations. We visualised change over time relative to interventions with statistical control charts. We conducted a high census days subanalysis. Length of stay and time of transfer from the ICU served as balancing measures. RESULTS: Mean presence increased from 43 to 83%, demonstrating special cause variation twice. Mean participation increased from 81 to 96%, demonstrating special cause variation once. Mean presence and participation were lower during high census (61 and 93% at project end) but improved with special cause variation. Length of stay and time of transfer remained stable. CONCLUSIONS: Through our interventions, family presence and participation in rounds improved without apparent unintended consequences. Family presence and participation may improve family and staff experience and outcomes; future research is warranted to evaluate this. Development of high level of reliability interventions may further improve family presence and participation, particularly on high census days.

Are Parent Discharge Readiness Scores Effective for Patients With Congenital Heart Disease After Cardiac Surgery?

OBJECTIVE: To assess discharge readiness and clinical engagement post-discharge in families of children undergoing congenital heart surgery. STUDY DESIGN: This prospective cross-sectional study was performed at a major tertiary pediatric cardiac referral center. Eligible parents and caregivers completed a discharge readiness tool, the Readiness for Hospital Discharge Scale for Parents of Hospitalized Children, via online survey on the day of discharge. Clinical engagement data included subsequent phone calls, clinic visits, emergency department visits, and hospital readmissions. Readiness for Hospital Discharge Scale for Parents of Hospitalized Children scores were measured as follows: very high (9-10), high (8-8.9), moderate (7-7.9), and low (<7). Descriptive statistics were used to describe demographic data. RESULTS: In total, 128 families enrolled between April and December 2021. Parent discharge readiness scores ranged from "high" to "very high." Families with lower socioeconomic status and younger patients (especially single-ventricle infants or "interstage") had a greater proportion of clinic visits, emergency department visits, and hospital readmissions within 30-days postdischarge compared with other groups. CONCLUSIONS: Discharge readiness scores were not associated with clinical engagement. We identified vulnerable populations as evidenced by a greater frequency of clinical engagement in the immediate postoperative period, particularly younger patients and first-time surgeries. Although these visits may be appropriate, novel programs could enhance education and emotional support to prevent delay in seeking care or creating excessive stress and anxiety after discharge.

Lyme carditis presenting as paroxysmal junctional tachycardia and complete atrioventricular block in an adolescent.

We describe a case of a previously healthy adolescent who presented with junctional tachycardia and complete atrioventricular (AV) block due to Lyme carditis. The simultaneous presence of these findings suggested significant inflammation of the AV junction. Junctional tachycardia, particularly if seen in a patient with conduction abnormalities and potential tick exposure, should increase suspicion for Lyme carditis.

Left Atrial Phasic Function via Cardiac Magnetic Resonance Imaging in Patients with Duchenne Muscular Dystrophy.

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.

Evaluation of an Outpatient and Telehealth Initiative to Reduce Tube Dependency in Infants with Complex Congenital Heart Disease.

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.

Maternal and neonatal factors associated with cesarean delivery in a cohort of pregnancies complicated by prenatally diagnosed congenital heart disease.

BACKGROUND: Pregnancies with prenatally diagnosed congenital heart disease (CHD) have increased cesarean delivery (CD) rates, with no outcome improvement. OBJECTIVE: We aim to examine indications for delivery, indications for CD and risk factors associated with CD. STUDY DESIGN: Retrospective cohort of 322 singleton pregnancies prenatally diagnosed with moderate to severe CHD. We compared maternal and fetal factors correlated with delivery route. RESULTS: CD rate was 46% (95% CI 40, 51%). Of all CD, 31.3% (95% CI 23.8, 38.7) were secondary to urgent fetal indications. However, 79.7% of inductions resulted in vaginal delivery (VD). Factors associated with CD include morbid obesity (RR 3.0, 95% CI 1.5, 6.1), diabetes (RR 3.9, 95% CI 2.0, 7.3) and severe pre-eclampsia (6.0, 95% CI 1.7, 21.4). Of the 10 most frequent CHD diagnoses, only hypoplastic-left-heart was associated with CD (OR 1.9, 95% CI 1.02, 3.4). CONCLUSIONS: Although the CD rate is higher in fetal CHD, most indications for CD are maternal.

Increasing Use of the Right Ventricle-to-Pulmonary Artery Shunt for Stage 1 Palliation.

BACKGROUND: Stage 1 palliation of hypoplastic left heart syndrome entails use of the Norwood operation with a modified Blalock-Taussig shunt or a right ventricle-to-pulmonary artery shunt, or the hybrid procedure. Use trends and factors influencing palliation selection remain unclear. We aimed to evaluate these questions and to compare outcomes between types of stage 1 palliation. METHODS: The National Pediatric Cardiology Quality Improvement Collaborative phase 1 (June 2008-August 2016) and phase 2 (August 2016-September 2019) databases were used. Procedure type was assessed by operation year. Baseline characteristics and annual hospital volume were evaluated. Postsurgical admission duration and outcomes were compared. RESULTS: A total of 3497 patients were included, 30.8% with modified Blalock-Taussig shunt, 59.7% with right ventricle-to-pulmonary artery shunt, and 9.5% with hybrid. Use of the right ventricle-to-pulmonary artery shunt increased over time (P = .02). This increase was similar among all hospital volumes. Higher hospital volume (odds ratio [OR], 1.2; 95% CI, 1.1-1.4; P = .003), male sex (OR, 1.3; 95% CI, 1.1-1.6; P = .01), and isolated cardiac disease (OR, 1.33; 95% CI, 1.01-1.55; P = .05) were associated with relatively higher likelihoods of a modified Blalock-Taussig shunt. Mortality/transplant rates before stage 2 palliation were higher with the modified Blalock-Taussig shunt than with the right ventricle-to-pulmonary artery shunt (12.3% vs 9.6%, P = .03). CONCLUSIONS: In stage 1 palliation, use of right ventricle-to-pulmonary artery shunts has increased over time, use of modified Blalock-Taussig shunts has decreased, and use of hybrids was unchanged. The modified Blalock-Taussig shunt has a higher likelihood of use in higher-volume centers, males, and less complex patients but is associated with longer hospitalizations and lower transplant-free survival to stage 2 palliation.

Noninvasive measurement of cardiac output during exercise in children with tetralogy of Fallot.

In patients with surgically repaired tetralogy of Fallot (TOF), reported peak oxygen consumption (VO(2)) 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(2), CO, CI, and SV measured at 90 % of peak predicted theoretical heart rate (90 % ppHR). Statistical correlation between peak VO(2) 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(2) 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(2)-MvO(2)) during exercise, which may limit peak exercise capacity. In this cohort of TOF patients, noninvasive CI measurement was feasible, and correlation with VO(2) was good.

Accelerated Idioventricular Rhythm in a Child With Status Asthmaticus.

Acute asthma exacerbations occur relatively frequently in children. We present the case of a 4-year-old boy who was admitted to our hospital in status asthmaticus and found to have a wide complex rhythm while being treated with inhaled albuterol and intravenous methylprednisolone. This rhythm was diagnosed as accelerated idioventricular rhythm (AIVR), which carries a benign prognosis. It resolved as the medications used to treat his asthma exacerbation were weaned. There was no ventricular ectopy seen on a 24-hour Holter monitor performed 3 months after his hospitalization, suggesting that the AIVR was related to the medications the patient was receiving at the time. This case suggests that albuterol may be a risk factor for the development of AIVR and highlights the importance of recognizing this rhythm to avoid unnecessary and potentially harmful therapies.

A survey of attitudes and experiences of women with heart disease.

OBJECTIVE: Cardiovascular disease is the leading cause of death in women, but little is known about the attitudes and experiences of the 6.4 million American women who have a diagnosis of heart disease. We assessed the knowledge, attitudes, and experiences of women with heart disease and the effect of the disease on their lives. SUBJECTS: A total of 204 women with a self-reported diagnosis of heart disease were the subjects. METHODS: A telephone survey with open-ended questions was used to ask women about their diagnoses, symptoms, interactions with the health care system, knowledge of risks and symptoms, satisfaction with care, and the effect of the disease on their lifestyle, psychosocial well-being, finances, interpersonal relationships, and spirituality. RESULTS: Most of the women (73%) had a diagnosis of coronary artery disease (CAD), with the remainder having diagnoses of other cardiac diseases. Most women took multiple medications and had undergone several diagnostic and therapeutic interventions. Almost half the women had been unaware that they were at risk of CAD and, after the condition was diagnosed, almost one-fourth of the respondents did not seek additional information about their diagnosis or treatment options from their physicians. More than half expressed dissatisfaction with their health care, most often because of physician-related knowledge and communication problems. Many women reported that they were unable or unwilling to make appropriate lifestyle changes after the diagnosis was made because of insufficient social, medical, or educational support. Educational opportunities may have been limited because less than 60% of women with CAD received cardiac rehabilitation services. Respondents reported significant changes in their interpersonal relationships, mental health, and financial and spiritual well-being as a result of having heart disease. CONCLUSION: Heart disease affects many aspects of women's lives. A significant percentage of surveyed women continue to have adverse consequences long after the diagnosis is made. Furthermore, proven beneficial lifestyle changes may not be implemented, most likely because of dissatisfaction with care, lack of educational and rehabilitative resources, symptoms of depression and anxiety, and suboptimal social support. This study identifies several problems that may help explain why women with heart disease have poorer medical outcomes than men with heart disease. Further investigation and better definition of these problems may help improve outcomes among women.