Outcomes Associated With Giant Coronary Artery Aneurysms After Kawasaki Disease: A Single-Center United States Experience.

OBJECTIVE: To determine the long-term outcomes among a cohort of patients with Kawasaki disease (KD) and a history of giant coronary artery aneurysms (CAA) at a single US center. RESULTS: There were 60 patients with KD and giant CAAs identified between 1989 and 2023. The majority of patients were male (71.7%) with median age at diagnosis of 0.9 years (0.2-13.3). Patients were followed for a median of 11 years, up to 34.5 years. MACE occurred in 13 (21.7%) patients at a median of 1.4 years (0.04-22.6) after KD diagnosis. The 10-, 20-, and 30-year MACE-free rates were 75%, 75%, and 60%. Patients with maximal CA z-scores ≥20 or bilateral CAA were more likely to have MACE. During follow-up, 26.7% of CAA regressed to normal luminal diameter at a median of 3.6 years (0.6-12.0). The 10-, 20- and 30-year likelihood of CA regression to normal luminal diameter was 36%, 46%, and 46%. CONCLUSIONS: Over 30 years, MACE occurred in nearly 22% of patients, more often in those with bilateral CAA or CA z-scores ≥20. Despite regression to normal luminal diameter in over 25% of CAA, patients with a history of KD-associated giant CAA require ongoing surveillance for cardiac complications, even years after the initial disease.

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.

Longitudinal Follow-Up of Children With HLHS and Association Between Norwood Shunt Type and Long-Term Outcomes: The SVR III Study.

OBJECTIVE: In the SVR trial (Single Ventricle Reconstruction), newborns with hypoplastic left heart syndrome were randomly assigned to receive a modified Blalock-Taussig-Thomas shunt (mBTTS) or a right ventricle-to-pulmonary artery shunt (RVPAS) at Norwood operation. Transplant-free survival was superior in the RVPAS group at 1 year, but no longer differed by treatment group at 6 years; both treatment groups had accumulated important morbidities. In the third follow-up of this cohort (SVRIII [Long-Term Outcomes of Children With Hypoplastic Left Heart Syndrome and the Impact of Norwood Shunt Type]), we measured longitudinal outcomes and their risk factors through 12 years of age. METHODS: Annual medical history was collected through record review and telephone interviews. Cardiac magnetic resonance imaging (CMR), echocardiogram, and cycle ergometry cardiopulmonary exercise tests were performed at 10 through 14 years of age among participants with Fontan physiology. Differences in transplant-free survival and complication rates (eg, arrhythmias or protein-losing enteropathy) were identified through 12 years of age. The primary study outcome was right ventricular ejection fraction (RVEF) by CMR, and primary analyses were according to shunt type received. Multivariable linear and Cox regression models were created for RVEF by CMR and post-Fontan transplant-free survival. RESULTS: Among 549 participants enrolled in SVR, 237 of 313 (76%; 60.7% male) transplant-free survivors (mBTTS, 105 of 147; RVPAS, 129 of 161; both, 3 of 5) participated in SVRIII. RVEF by CMR was similar in the shunt groups (RVPAS, 51±9.6 [n=90], and mBTTS, 52±7.4 [n=75]; P=0.43). The RVPAS and mBTTS groups did not differ in transplant-free survival by 12 years of age (163 of 277 [59%] versus 144 of 267 [54%], respectively; P=0.11), percentage predicted peak Vo2 for age and sex (74±18% [n=91] versus 72±18% [n=84]; P=0.71), or percentage predicted work rate for size and sex (65±20% versus 64±19%; P=0.65). The RVPAS versus mBTTS group had a higher cumulative incidence of protein-losing enteropathy (5% versus 2%; P=0.04) and of catheter interventions (14 versus 10 per 100 patient-years; P=0.01), but had similar rates of other complications. CONCLUSIONS: By 12 years after the Norwood operation, shunt type has minimal association with RVEF, peak Vo2, complication rates, and transplant-free survival. RVEF is preserved among the subgroup of survivors who underwent CMR assessment. Low transplant-free survival, poor exercise performance, and accruing morbidities highlight the need for innovative strategies to improve long-term outcomes in patients with hypoplastic left heart syndrome. REGISTRATION: URL: https://www. CLINICALTRIALS: gov; Unique identifier: NCT0245531.

Neighborhood Social Vulnerability and Interstage Weight Gain: Evaluating the Role of a Home Monitoring Program.

Background Poor interstage weight gain is a risk factor for adverse outcomes in infants with hypoplastic left heart syndrome. We sought to examine the association of neighborhood social vulnerability and interstage weight gain and determine if this association is modified by enrollment in our institution's Infant Single Ventricle Management and Monitoring Program (ISVMP). Methods and Results We performed a retrospective single-center study of infants with hypoplastic left heart syndrome before (2007-2010) and after (2011-2020) introduction of the ISVMP. The primary outcome was interstage weight gain, and the secondary outcome was interstage growth failure. Multivariable linear and logistic regression models were used to examine the association between the Social Vulnerability Index and the outcomes. We introduced an interaction term into the models to test for effect modification by the ISVMP. We evaluated 217 ISVMP infants and 111 pre-ISVMP historical controls. The Social Vulnerability Index was associated with interstage growth failure (P=0.001); however, enrollment in the ISVMP strongly attenuated this association (P=0.04). Pre-ISVMP, as well as high- and middle-vulnerability infants gained 4 g/d less and were significantly more likely to experience growth failure than low-vulnerability infants (high versus low: adjusted odds ratio [aOR], 12.5 [95% CI, 2.5-62.2]; middle versus low: aOR, 7.8 [95% CI, 2.0-31.2]). After the introduction of the ISVMP, outcomes did not differ by Social Vulnerability Index tertile. Infants with middle and high Social Vulnerability Index scores who were enrolled in the ISVMP gained 4 g/d and 2 g/d more, respectively, than pre-ISVMP controls. Conclusions In infants with hypoplastic left heart syndrome, high social vulnerability is a risk factor for poor interstage weight gain. However, enrollment in the ISVMP significantly reduces growth disparities.

Genetic and clinical variables act synergistically to impact neurodevelopmental outcomes in children with single ventricle heart disease.

BACKGROUND: Recent large-scale sequencing efforts have shed light on the genetic contribution to the etiology of congenital heart defects (CHD); however, the relative impact of genetics on clinical outcomes remains less understood. Outcomes analyses using genetics are complicated by the intrinsic severity of the CHD lesion and interactions with conditionally dependent clinical variables. METHODS: Bayesian Networks were applied to describe the intertwined relationships between clinical variables, demography, and genetics in a cohort of children with single ventricle CHD. RESULTS: As isolated variables, a damaging genetic variant in a gene related to abnormal heart morphology and prolonged ventilator support following stage I palliative surgery increase the probability of having a low Mental Developmental Index (MDI) score at 14 months of age by 1.9- and 5.8-fold, respectively. However, in combination, these variables act synergistically to further increase the probability of a low MDI score by 10-fold. The absence of a damaging variant in a known syndromic CHD gene and a shorter post-operative ventilator support increase the probability of a normal MDI score 1.7- and 2.4-fold, respectively, but in combination increase the probability of a good outcome by 59-fold. CONCLUSIONS: Our analyses suggest a modest genetic contribution to neurodevelopmental outcomes as isolated variables, similar to known clinical predictors. By contrast, genetic, demographic, and clinical variables interact synergistically to markedly impact clinical outcomes. These findings underscore the importance of capturing and quantifying the impact of damaging genomic variants in the context of multiple, conditionally dependent variables, such as pre- and post-operative factors, and demography.

Single ventricle congenital heart disease is a birth defect. In these children, the heart has only one effective blood-pumping chamber instead of two. Surgery can reroute the blood to use only one chamber, but multiple risk factors influence how well a child develops afterwards. Studying these risk factors can be challenging because they are interconnected, i.e. children with a genetic birth defect may be more likely to have a lower birthweight, and hence more likely to spend longer in hospital after surgery. Here, we used a statistical approach not commonly applied to study congenital heart disease and describe that whether a genetic variant (a small difference in a child's DNA) is important for how a child with single ventricle heart disease develops and grows after surgery depends on the presence of other risk factors.

Impact of Ventricular Dysfunction and Atrioventricular Valve Regurgitation on Pre-Fontan Attrition.

BACKGROUND: There is a significant incidence of pre-Fontan attrition-defined as failure to undergo Fontan completion-after superior cavopulmonary connection. This study investigated the impact of at least moderate ventricular dysfunction (VD) and atrioventricular valve regurgitation (AVVR) on pre-Fontan attrition. METHODS: This single-center retrospective cohort study included all infants who underwent Norwood palliation from 2008 to 2020 and subsequently underwent superior cavopulmonary connection. Pre-Fontan attrition was defined as death, listing for heart transplantation before Fontan completion, or unsuitability for Fontan completion. The study's secondary outcome was transplant-free survival. RESULTS: Pre-Fontan attrition occurred in 34 of 267 patients (12.7%). Isolated VD was not associated with attrition. However, patients with isolated AVVR had 5 times the odds of attrition (odds ratio, 5.4; 95% CI 1.8-16.2), and patients with both VD and AVVR had 20 times the odds of attrition (odds ratio, 20.1; 95% CI 7.7-52.8) compared with patients without VD or AVVR. Only patients with both VD and AVVR had significantly worse transplant-free survival compared with patients without VD or AVVR (hazard ratio, 7.7; 95% CI 2.8-21.6). CONCLUSIONS: The additive effect of VD and AVVR is a powerful contributor to pre-Fontan attrition. Future research investigating therapies that can mitigate the degree of AVVR may help improve Fontan completion rates and long-term outcomes.

Feasibility of Digital Stethoscopes in Telecardiology Visits for Interstage Monitoring in Infants with Palliated Congenital Heart Disease.

Infants with staged surgical palliation for congenital heart disease are at high-risk for interstage morbidity and mortality. Interstage telecardiology visits (TCV) have been effective in identifying clinical concerns and preventing unnecessary emergency department visits in this high-risk population. We aimed to assess the feasibility of implementing auscultation with digital stethoscopes (DSs) during TCV and the potential impact on interstage care in our Infant Single Ventricle Monitoring & Management Program. In addition to standard home-monitoring practice for TCV, caregivers received training on use of a DS (Eko CORE attachment assembled with Classic II Infant Littman stethoscope). Sound quality of the DS and comparability to in-person auscultation were evaluated based on two providers' subjective assessment. We also evaluated provider and caregiver acceptability of the DS. From 7/2021 to 6/2022, the DS was used during 52 TCVs in 16 patients (median TCVs/patient: 3; range: 1-8), including 7 with hypoplastic left heart syndrome. Quality of heart sounds and murmur auscultation were subjectively equivalent to in-person findings with excellent inter-rater agreement (98%). All providers and caregivers reported ease of use and confidence in evaluation with the DS. In 12% (6/52) of TCVs, the DS provided additional significant information compared to a routine TCV; this expedited life-saving care in two patients. There were no missed events or deaths. Use of a DS during TCV was feasible in this fragile cohort and effective in identifying clinical concerns with no missed events. Longer term use of this technology will further establish its role in telecardiology.

Attributable mortality benefit of digoxin treatment in hypoplastic left heart syndrome after the Norwood operation: An instrumental variable-based analysis using data from the Pediatric Health Information Systems Database.

BACKGROUND: Observational studies have demonstrated an association between the use of digoxin and reduced interstage mortality after Norwood operation for hypoplastic left heart syndrome (HLHS). Digoxin use has increased significantly but remains variable between different hospitals, independent of case-mix. Instrumental variable analyses have the potential to overcome unmeasured confounding, the major limitation of previous observational studies and to generate an estimate of the attributable benefit of treatment with digoxin. METHODS: A cohort of neonates with HLHS born from January 1, 2007 to December 31, 2021 who underwent Norwood operation at Pediatric Health Information Systems Database hospitals and survived >14 days after operation were studied. Using hospital-specific, 6-month likelihood of administering digoxin as an instrumental variable, analyses adjusting for both unmeasured confounding (using the instrumental variable) and measured confounders with multivariable logistic regression were performed. RESULTS: The study population included 5,148 subjects treated at 47 hospitals of which 63% were male and 46% non-Hispanic white. Of these, 44% (n = 2,184) were prescribed digoxin. Treatment with digoxin was associated with superior 1-year transplant-free survival in unadjusted analyses (85% vs 82%, P = .02). This survival benefit persisted in an instrumental-variable analysis (OR: 0.71, 95% CI: 0.54-0.94, P = .01), which can be converted to an absolute risk reduction of 5% (number needed to treat of 20). CONCLUSIONS: In this observational study of patients with HLHS after Norwood using instrumental variable techniques, a significant benefit in 1-year transplant-free survival attributable to digoxin was demonstrated. In the absence of clinical trial data, this should encourage the use of digoxin in this vulnerable population.

Association of Home Monitoring and Unanticipated Interstage Readmissions in Infants With Hypoplastic Left Heart Syndrome.

Background The impact of home monitoring on unanticipated interstage readmissions in infants with hypoplastic left heart syndrome has not been previously studied. We sought to examine the association of our institution's Infant Single Ventricle Management and Monitoring Program (ISVMP) with readmission frequency, cumulative readmission days, and readmission illness severity and to identify patient-level risk factors for readmission. Methods and Results We performed a retrospective single-center cohort study comparing infants with hypoplastic left heart syndrome enrolled in ISVMP (December 2010-December 2019) to historical controls (January 2007-November 2010). The primary outcome was number of readmissions per interstage days. Secondary outcomes were cumulative interstage readmission days and occurrence of severe readmissions. Inverse probability weighted and multivariable generalized linear models were used to examine the association between ISVMP and the outcomes. We compared 198 infants in the ISVMP to 128 historical controls. Infants in the ISVMP had more than double the risk of interstage readmission compared with controls (adjusted incidence rate ratio, 2.38 [95% CI, 1.50-3.78]; P=0.0003). There was no difference in cumulative interstage readmission days (adjusted incidence rate ratio, 1.02 [95% CI, 0.69-1.50]; P=0.90); however, infants in the ISVMP were less likely to have severe readmissions (adjusted odds ratio, 0.28 [95% CI, 0.11-0.68]; P=0.005). Other factors independently associated with number of readmissions included residing closer to our center, younger gestational age, genetic syndrome, and discharge on exclusive enteral feeds. Conclusions Infants in the ISVMP had more frequent readmissions but comparable readmission days and fewer severe unanticipated readmissions. These findings suggest that home monitoring can reduce interstage morbidity without increasing readmission days.

Neurologic complications of infective endocarditis in children.

OBJECTIVES: To define the frequency and characteristics of acute neurologic complications in children hospitalised with infective endocarditis and to identify risk factors for neurologic complications. STUDY DESIGN: Retrospective cohort study of children aged 0-18 years hospitalised at a tertiary children's hospital from 1 January, 2008 to 31 December, 2017 with infective endocarditis. RESULTS: Sixty-eight children met Duke criteria for infective endocarditis (43 definite and 25 possible). Twenty-three (34%) had identified neurologic complications, including intracranial haemorrhage (25%, 17/68) and ischaemic stroke (25%, 17/68). Neurologic symptoms began a median of 4.5 days after infective endocarditis symptom onset (interquartile range 1, 25 days), though five children were asymptomatic and diagnosed on screening neuroimaging only. Overall, only 56% (38/68) underwent neuroimaging during acute hospitalisation, so additional asymptomatic neurologic complications may have been missed. Children with identified neurologic complications compared to those without were older (48 versus 22% ≥ 13 years old, p = 0.031), more often had definite rather than possible infective endocarditis (96 versus 47%, p < 0.001), mobile vegetations >10mm (30 versus 11%, p = 0.048), and vegetations with the potential for systemic embolisation (65 versus 29%, p = 0.004). Six children died (9%), all of whom had neurologic complications. CONCLUSIONS: Neurologic complications of infective endocarditis were common (34%) and associated with mortality. The true frequency of neurologic complications was likely higher because asymptomatic cases may have been missed without screening neuroimaging. Moving forward, we advocate that all children with infective endocarditis have neurologic consultation, examination, and screening neuroimaging. Additional prospective studies are needed to determine whether early identification of neurologic abnormalities may direct management and ultimately reduce neurologic morbidity and overall mortality.

Initial Experience with Telemedicine for Interstage Monitoring in Infants with Palliated Congenital Heart Disease.

Infants with staged surgical palliation for congenital heart disease are at high-risk for interstage morbidity and mortality; home monitoring programs have mitigated these risks. In 2019, we instituted telemedicine (TM) in our established Infant Single Ventricle Monitoring Program. All consecutive patients discharged following neonatal operation/intervention were monitored until subsequent stage 2 surgical palliation. We offered TM (synchronous video) visits as part of regularly scheduled follow-up, replacing at least one in-person primary care visit with a TM cardiologist visit. We tracked emergency department (ED) visits, hospitalizations, how TM identified clinical concerns, and whether use of TM prevented unnecessary ED visits or expedited in-person assessment. We assessed caregiver and clinician satisfaction. Between 8/2019 and 5/2020, we conducted 60 TM visits for 29 patients. Of 31 eligible patients, 2 families (6.9%) declined. Median monitoring time was 199 days (range 75-264) and median number of TM visits/patient was 2 (range 1-5). In 6 visits (10%), significant clinical findings were identified which avoided an ED visit. Five TM visits led to expedited outpatient assessments, of which 1 patient required hospitalization. There were no missed events or deaths. Median ED visits/patient/month were significantly lower compared to the same calendar period of the prior year (0.0 (0-2.5) vs. 0.4 (0-3.7), p = 0.0004). Caregivers and clinicians expressed high levels of satisfaction with TM. TM for this high-risk population is feasible and effective in identifying clinical concerns and preventing unnecessary ED visits. TM was particularly effective during the COVID-19 pandemic, allowing for easy adaptation of care to ensure patient safety in this fragile cohort.

Combined ventricular dysfunction and atrioventricular valve regurgitation after the Norwood procedure are associated with attrition prior to superior cavopulmonary connection.

Background: Infants with hypoplastic left heart syndrome (HLHS) or a variant are at risk of ventricular dysfunction (VD) and atrioventricular valve regurgitation (AVVR) prior to superior cavopulmonary connection (SCPC). Although the impact of these complications in isolation has been described, their effect in combination on attrition is poorly defined. Methods: A retrospective observational study of patients with HLHS or variants undergoing a Norwood procedure between 2008 and 2020 at a single center was performed. VD and AVVR were defined as moderate or severe when seen on 2 sequential echocardiograms outside the perioperative period. Attrition was defined as death, listing for heart transplant, or unsuitability for SCPC or transplant. Descriptive statistics and regression models were used for analysis. Results: A total of 397 patients were included, of whom 75% had HLHS and 57% had received a Blalock-Thomas-Taussig shunt. Isolated VD occurred in 9% of patients, AVVR occurred in 13%, and both occurred in 6%. Attrition prior to SCPC occurred in 19% of the overall cohort, in 52% of patients with combined VD and AVVR (odds ratio [OR], 5.2; 95% confidence interval [CI], 2.3-12.0; P < .01), 26% of those with VD (OR, 1.5; 95% CI, 0.7-3.3; P = .32), 25% of those with AVVR (OR, 1.5; 95% CI, 0.7-2.9; P = .27), and 15% in those with neither (OR, 0.3; 95% CI, 0.2-0.6; P < .01). Other factors associated with attrition included prematurity, total bypass time at Norwood, and extracorporeal membrane oxygenation after Norwood, whereas later year of Norwood was protective (P < .01 for all). Conclusions: The presence of combined VD and AVVR markedly increases the likelihood of attrition prior to SCPC, identifying a high-risk group.

Occlusion Pressure of the Thoracic Duct in Fontan Patients With Lymphatic Failure: Does Dilatation Challenge Contractility?

BACKGROUND: The Fontan circulation challenges the lymphatic system. Increasing production of lymphatic fluid and impeding lymphatic return, increased venous pressure may cause lymphatic dilatation and decrease lymphatic contractility. In-vitro studies have reported a lymphatic diameter-tension curve, with increasing passive stretch affecting the intrinsic contractile properties of each thoracic duct segment. We aimed to describe thoracic duct occlusion pressure and asses if thoracic duct dilation impairs contractility in individuals with a Fontan circulation and lymphatic failure. METHODS: Central venous pressure and thoracic duct measurements were retrospectively collected from 31 individuals with a Fontan circulation. Thoracic duct occlusion pressure was assessed during a period of external manual compression and used as an indicator of lymphatic vessel contractility. Measurements of pressure were correlated with measurements of the thoracic duct diameter in images obtained by dynamic contrast-enhanced MR lymphangiography. RESULTS: The average central venous pressure and average pressure of the thoracic duct were 17 mm Hg. During manual occlusion, the thoracic duct pressure significantly increased to 32 mm Hg. The average thoracic duct diameter was 3.3 mm. Thoracic duct diameter correlated closely with the central venous pressure. The rise in pressure following manual occlusion showed an inverse correlation with the diameter of the thoracic duct. CONCLUSION: Higher central venous pressures are associated with increasing diameters of the thoracic duct. When challenged by manual occlusion, dilated thoracic ducts display a decreased ability to increase pressure. Dilatation and a resulting decreased contractility may partly explain the challenged lymphatic system in individuals with a Fontan circulation.

The pediatric heart network's study on long-term outcomes of children with HLHS and the impact of Norwood Shunt type in the single ventricle reconstruction trial cohort (SVRIII): Design and adaptations.

BACKGROUND: The Single Ventricle Reconstruction (SVR) Trial was the first randomized clinical trial of a surgical approach for treatment of congenital heart disease. Infants with hypoplastic left heart syndrome (HLHS) and other single right ventricle (RV) anomalies were randomized to a modified Blalock Taussig Thomas shunt (mBTTS) or a right-ventricular-to-pulmonary-artery shunt (RVPAS) at the time of the Norwood procedure. The aim of the Long-term Outcomes of Children with HLHS and the Impact of Norwood Shunt Type (SVR III) study is to compare early adolescent outcomes including measures of cardiac function, transplant-free survival, and neurodevelopment, between those who received a mBTTS and those who received an RVPAS. METHODS: Transplant-free survivors of the SVR cohort were enrolled at 10 to 15 years of age for multifaceted in-person evaluation of cardiac function (cardiac magnetic resonance [CMR], echocardiogram and exercise test) and neurodevelopmental evaluation. Right ventricular ejection fraction measured by CMR served as the primary outcome. Development of arrhythmias, protein losing enteropathy, and other comorbidities were assessed through annual medical history interview. Through the course of SVR III, protocol modifications to engage SVR trial participants were designed to enhance recruitment and retention. CONCLUSIONS: Evaluation of long-term outcomes will provide important data to inform decisions about the shunt type placed at the Norwood operation and will improve the understanding of cardiovascular and neurodevelopmental outcomes for early adolescents with HLHS.

Feeding challenges in the newborn with congenital heart disease.

PURPOSE OF REVIEW: Feeding challenges and growth failure are lifelong issues for infants with congenital heart disease. The purpose of this review is to summarize the literature on the topic from North America. RECENT FINDINGS: Despite recognition of feeding challenges and ongoing national collaboration, >50% of infants with univentricular physiology continue to require supplemental tube feeds at the time of discharge from neonatal surgery. Preoperative feeding is now commonly used in prostaglandin dependent neonates with congenital heart disease. The value of a structured nutritional program with establishment of best practices in nutrition is well recognized in the current era. Despite implementation of these best practices, neonates undergoing cardiac surgery continue to struggle with weight gain prior to discharge. This suggests that there is more to growth than provision of adequate nutrition alone. SUMMARY: The National Pediatric Cardiology Quality Improvement Collaborative continues to play a major role in optimizing nutrition in infants with congenital heart disease. This among other registries underscores the importance of collaboration in improving overall outcomes for children with congenital heart disease. Nurses should be encouraged to lead both clinical and research efforts to overcome feeding challenges encountered by these children.

New insights on growth trajectory in infants with complex congenital heart disease.

PURPOSE: We aimed to describe the weight-for-age Z-score growth trajectory (WAZ-GT) of infants with complex congenital heart disease (cCHD) after neonatal cardiac surgery in the first 4 months of life and assess potential risk factors. METHODS: We utilized data from a previously reported trial of the REACH telehealth home monitoring (NCT01941667) program which evaluated 178 infants with cCHD from 2012 to 2017. Over the first 4 months of life, weekly infant weights were converted to WAZ. WAZ-GT classes were identified using latent class growth modeling. Multinomial logistic regression models were used to examine the associations between potential risk factors and WAZ-GT classes. RESULTS: Four distinct classes of WAZ-GT were identified: maintaining WAZ > 0, 14%; stable around WAZ = 0, 35%; partially recovered, 28%; never recovered, 23%. Compared with reference group "stable around WAZ=0," we identified clinical and sociodemographic determinants of class membership for the three remaining groups. "Maintaining WAZ > 0" had greater odds of having biventricular physiology, borderline appetite, and a parent with at least a college education. "Partially recovered" had greater odds of hospital length of stay>14 days and being a single child in the household. "Never recovered" had greater odds hospital length of stay >14 and > 30 days, tube feeding at discharge, and low appetite. CONCLUSIONS: This study described distinct classes of WAZ-GT for infants with cCHD early in infancy and identified associated determinants. PRACTICE IMPLICATIONS: Findings from this study can be used in the identification of infants at risk of poor WAZ-GT and in the design of interventions to target growth in this vulnerable patient population.

Relationship Between Serum Brain-Type Natriuretic Peptide and Biomarkers of Growth in Infants With Shunt-Dependent Single Cardiac Ventricle.

For infants with shunt-dependent or ductal-dependent single ventricle heart disease, poor growth is common and associated with morbidity and impaired neurodevelopmental outcomes. Although attention has focused on nutrition to promote weight gain, little is known about the relation between heart failure and growth factors. A prospective observational pilot study was performed to assess the relation between heart failure, assessed by brain natriuretic peptide (BNP), and growth factors (insulin-like growth factor 1 [IGF-1] and insulin-like growth factor-binding protein 3) at 3 visits: (1) before discharge from neonatal intervention with the establishment of stable pulmonary blood flow, (2) immediately before superior cavopulmonary connection, and (3) before discharge after superior cavopulmonary connection operation. The relation between BNP and growth factors was analyzed using Spearman pairwise correlations at each visit and modeled over time with a linear mixed-effects model. Correlations were considered worthy of further exploration using a p <0.10, given the exploratory nature of the study. The study included 38 infants (66% male, 68% hypoplastic left heart syndrome). Median BNP was elevated at visit 1 and decreased over time (287 pg/dl [interquartile range 147 to 794], 85 pg/dl [52 to 183], and 90 pg/dl [70 to 138]). Median IGF-1 Z score was <0 at each visit but increased over time (-0.9 [interquartile range -1.1 to 0.1], -0.7 [-1.2 to 0.1], and -0.5 [-1.2 to 0]). Inverse correlations were found between BNP and IGF-1 at visit 1 (r = -0.40, p = 0.097), BNP and IGF-1 and insulin-like growth factor-binding protein 3 at visit 2 (r = -0.33, p = 0.080 and r = -0.33, p = 0.085, respectively) and BNP and IGF-1 Z score at visit 3 (r = -0.42, p = 0.049). Significant relations were likewise found between the change in BNP and the change in IGF-1 between visits 1 and 3 (p = 0.046) and between visits 2 and 3 (p = 0.048). In conclusion, this pilot study demonstrates an inverse correlation between BNP and growth factors, suggesting that the heart failure state associated with this physiology may play a mechanistic role in impaired growth.

Anthropometry Based Growth and Body Composition in Infants with Complex Congenital Heart Disease.

BACKGROUND: Infants with congenital heart disease (CHD) often have poor growth and altered body composition (fat and muscle accretion). AIM: Describe growth patterns in infants with CHD using interval weight, length, head circumference (HC), triceps (TSF), subscapular skinfolds (SSSF), and mid-upper arm circumference (MUAC) measurements. SUBJECTS AND METHODS: A total of 120 infants enrolled: 48% healthy and 58% with CHD (45% single ventricle [SV]; 55% two ventricle [2V] physiology). Weight, length, HC, TSF, SSSF, and MUAC measured at 3-, 6-, 9-, and 12-months of age. RESULTS: CHD infants had lower weight, length, and HC z-scores at 3-, 6-, and 9-months. At 9-months, infants with SV physiology had larger TSF and SSSF z-scores over 2V and healthy infants. Overall MUAC z-scores were smaller at 3- and 6-months in infants with CHD. CONCLUSISON: Infants with CHD have a complex pattern of growth. Longitudinal growth and body composition measurements provide information to better understand this pattern.

Liver lymphatic anatomy and role in systemic lymphatic disease.

OBJECTIVES: To characterize hepatic to systemic lymphatic connections in patients with systemic lymphatic disease using intra-hepatic lymphangiography and to compare outcomes after lymphatic intervention. METHODS: In this retrospective study, patients with intra-hepatic lymphangiography from May 2014 - April 2019 at our institution were included. Imaging review was performed and hepatic lymphatic connections and flow patterns were characterized. Clinical data were reviewed and comparisons between patients undergoing lymphatic intervention with or without abnormal hepatic lymphatics were performed. RESULTS: During the study period, 105 patients underwent intra-hepatic lymphangiography. Primary clinical presentation included ascites (19/105), chylothorax (27/105), plastic bronchitis (PB) (17/105), and protein losing enteropathy (PLE) (42/105). Five categories of hepatic lymphatic connections and flow patterns were identified (%): normal (25%, 26/105), hepatoperitoneal (12%, 13/105), hepatopulmonary (10.5%, 11/105), hepatomesenteric (7.5%, 8/105), and hepatoduodenal (41%, 43/105) with four patients having more than one abnormal pattern. A comparison between clinical presentation and imaging category revealed an increased likelihood of having ascites with hepatoperitoneal (p < .0001), chylothorax/PB with hepatopulmonary (p = .01), and PLE with hepatoduodenal (p < .001) connections. Seventy-six patients had a lymphatic intervention, 24% with normal, and 76% with abnormal liver lymphatics. There was no difference in length of hospital stay or mortality between the two groups, but there was a prolonged time to symptom resolution (p = .006) and persistent symptoms after 6 months (5% vs 44%, p = .002) in the group with abnormal liver lymphatics. CONCLUSION: We identified five liver lymphatic imaging categories with a substantial correlation to presenting lymphatic disease. Abnormal imaging patterns correlated with increased morbidity. Evaluation of liver lymphatics should be considered in patients with a systemic lymphatic disease if central lymphatic imaging is normal. KEY POINTS: • We identified five liver lymphatic imaging patterns: normal, hepatoperitoneal, hepatomesenteric, hepatopulmonary, and hepatoduodenal. • Imaging patterns were correlated with disease presentation (normal - chylothorax/PB, hepatoperitoneal - ascites/chylothorax, hepatopulmonary - chylothorax/PB, hepatoduodenal - PLE). • Abnormal imaging patterns correlated with increased morbidity.