Utility of the NEONATE Score at an Institution that Routinely Performs the Hybrid Procedure for Hypoplastic Left Heart Syndrome.

NEONATE score > 17 has been proposed as a risk factor for interstage mortality/cardiac transplant (IM/T) for patients with single ventricle physiology. Hybrid procedure is assigned 6 points, the highest possible score for that surgical variable. Most centers reserve the hybrid procedure for high-risk patients. Goal of this study was to evaluate the NEONATE score at a center that routinely performs the hybrid procedure. Retrospective chart review of patients undergoing the hybrid procedure was performed (2008-2021). Demographics and variables used for the NEONATE score were collected. Maximization of Youden's J Statistic used to determine cohort-specific optimal threshold for patients undergoing comprehensive Stage II procedure (H-CSII) versus those with IM/T (H-IM/T). Total of 120 patients met inclusion criteria (H-CSII = 105, H-IM/T = 15). Gestational age was median 39 weeks (IQR 38, 39) and birth weight was 3.18 kg (2.91, 3.57). No patient was discharged with opiates or required post-operative extracorporeal circulatory support. Optimal threshold, as selected by maximizing Youden's J Statistic, was 22. Score > 22 had a positive predictive value of 0.33 (95% CI 0.12-0.62), negative predictive values of 0.90 (95% CI 0.83-0.95), and accuracy of 0.83 (95% CI 0.75-0.90) for IM/T. At a center that routinely performs the hybrid procedure, value of > 22 had the highest accuracy. This suggests that the hybrid procedure is not necessarily intrinsically a risk-factor for IM/T, but rather patient selection for the hybrid procedure may play a larger role at centers that do not routinely perform this procedure.

Incidence of aspiration in infants with single-ventricle physiology following hybrid procedure.

BACKGROUND: Swallowing dysfunction is a known complication for infants with complex congenital heart disease (CHD), but few studies have examined swallowing outcomes following the hybrid procedure for stage 1 palliation in children with single ventricle physiology. OBJECTIVES: (1) Identify the incidence of aspiration in all infants with single ventricle physiology who underwent the hybrid procedure and (2) Compare results of clinical bedside and instrumental swallowing evaluations to examine the predictive value of a less invasive swallowing assessment for this population of high-risk infants. METHODS: This was a retrospective cohort chart review study. All patients with single-ventricle physiology who underwent the hybrid procedure received a referral for subsequent instrumental swallow assessment during a 4-year period. Results from clinical bedside evaluations were compared to those of the instrumental assessment. RESULTS: Fifty infants were included in this study. During instrumental swallow assessment, aspiration was observed in 28% of infants following the hybrid procedure. Normal swallowing function was identified in 44% of infants, and 28% demonstrated laryngeal penetration. Neither length of intubation nor prematurity were found to be predictors of aspiration. Thirty-six of these infants were assessed via clinical bedside evaluation prior to the instrumental evaluation. The sensitivity of the clinical bedside evaluation was 0.73 and the specificity was 0.92. CONCLUSIONS: This study reports on a cohort of infants with single ventricle physiology following the hybrid procedure and found the incidence of aspiration to be lower than previously reported. Improved clinical bedside evaluation guidelines are needed so that clinicians can predict more reliably which infants are at risk for aspiration following the hybrid procedure.

Interstage outcomes in single ventricle patients undergoing hybrid stage 1 palliation.

OBJECTIVE: Interstage readmissions are common in infants with single ventricle congenital heart disease undergoing staged surgical palliation. We retrospectively examined readmissions during the interstage period. DESIGN: Retrospective analysis. SETTING: The Heart Center at Nationwide Children's Hospital, Columbus, Ohio. PATIENTS: Newborns undergoing hybrid stage 1 palliation from January 2012 to December 2016 who survived to hospital discharge and were followed at our institution. INTERVENTIONS: All patients underwent hybrid stage 1 palliation. OUTCOME MEASURES: Outcomes included (1) reason for interstage readmission; (2) feeding modality during interstage period; (3) major interstage adverse events; and (4) interstage mortality. RESULTS: Study group comprised 57 patients. Five patients only admitted once during the interstage period for scheduled cardiac catheterization were included in the no readmission group. Therefore, 43 patients (75%) had a total of 87 interstage readmissions. Fourteen patients had 15 major interstage adverse events accounting for 17% of total readmissions. Stroke (n = 1); sepsis (n = 1); pericardial effusion requiring drainage (n = 1); mesenteric ischemia (n = 1); shock (n = 1); and cardiac catheterization requiring intervention (n = 11)-ductal stent balloon angioplasty (n = 3), enlargement of atrial septal defect/stent placement (n = 3), retrograde aortic arch stenosis (n = 4). Thirty-three readmissions were secondary to gastrointestinal/feeding issues; 15 cyanosis; 15 work of breathing; and 9 asymptomatic patients. Four patients suffered interstage deaths (7%). Five patients (9%) spent >30 days in the hospital during the interstage period. Of the 47 newborns (82%) discharged exclusively orally feeding, 74% remained all orally feeding throughout interstage period. No patient discharged with tube feedings learned to eat during the interstage period. CONCLUSION: Interstage readmissions are common in the hybrid patient population. Seventeen percent were secondary to major adverse events. Interstage mortality was 7%. Future studies to identify interventions aimed at decreasing feeding issues and viral bronchiolitis in this tenuous patient population will hopefully improve quality outcomes, reduce readmissions, and lessen health care costs.

Perception scores of siblings and parents of children with hypoplastic left heart syndrome.

OBJECTIVES: Siblings of children with chronic medical conditions endorse a lower quality of life compared to age-matched peers. Caregiver and sibling-self report of adjustment are often discordant. Congenital heart disease significantly affects family life. To date, there have been no studies addressing the functioning of siblings of children with hypoplastic left heart syndrome, one of the most severe forms of congenital heart disease. The goal of this study was to assess the impact of hypoplastic left heart syndrome on sibling's quality of life as well as the caregiver's perception of this effect. STUDY DESIGN: Cross-sectional study using a web-based survey distributed via various listservs targeted towards families of children with hypoplastic left heart syndrome. Employed the Sibling Perception Questionnaire, designed to assess sibling and caregiver perceptions of adjustment to chronic illness. A Negative Adjustment Composite Score was calculated for each respondent, with higher values representing more negative adjustment. RESULTS: Thirty-five caregivers responded. Majority of caregivers were female (74%), white (86%) and college educated (54%). Thirty-two siblings participated, ranging in age from 7 to 30 years of age (12.5 ± 6.3). Most children with hypoplastic left heart syndrome (73%) had undergone the third stage of palliation. Forty-two caregiver-sibling pairs were examined. Caregiver Negative Adjustment Composite Scores were significantly higher than sibling scores, with caregivers reporting more adjustment problems (2.4 ± 0.4) than siblings (2.3 ± 0.3, P < .05). Sibling age was correlated with worse caregiver and sibling scores (r 0.35, P < .05). CONCLUSIONS: Caregivers of children with hypoplastic left heart syndrome perceive their siblings as struggling more than the children self-report. Siblings tend to report worse adjustment as they get older. These data suggest that programs should include support for the entire family through all ages to optimize quality of life.

Differences in midterm outcomes in infants with hypoplastic left heart syndrome diagnosed with necrotizing enterocolitis: NPCQIC database analysis.

INTRODUCTION: Neonates with hypoplastic left heart syndrome (HLHS) are at increased risk for necrotizing enterocolitis (NEC). Initial hospital outcomes are well described, but minimal midterm data exist. Goal of this study was to compare outcomes of HLHS infants with NEC (HLHS-NEC) to HLHS without NEC (HLHS-nNEC) during the interstage period. METHODS: Data were reviewed from 55 centers using the NPC-QIC database. Case-control study with one HLHS-NEC matched to HLHS-nNEC neonates in a 1:3 ratio based on institutional site, type of surgical repair, and gestational age ±1 week was performed. Baseline demographics as well as outcome data were recorded. The t tests or chi-square tests were performed as appropriate. RESULTS: There were 57 neonates in the HLHS-NEC (14 Norwood-BT, 37 Norwood-RVPA, and 6 hybrid) and 171 neonates in the HLHS-nNEC group. There were significant differences between the HLHS-NEC versus HLHS-nNEC for presence of atrioventricular valve regurgitation (7% vs 2%), use of extracorporeal membrane oxygenation (11% vs 2%), hospital stay (60.4 ± 30.0 vs 36.3 ± 33.6 days), Z-score weight at discharge (-2.1 vs -1.6), incidence of no oral intake (33% vs 14%), and use of formula only nutrition at discharge (61% vs 29%), respectively. There were no significant differences between groups in readmission rates due to adverse gastrointestinal events, use of gastrointestinal medications, interstage deaths, or Z-score weight at time of second surgery. HLHS-NEC continued to be more likely to be entirely tube dependent for enteral intake at time prior to the second procedure (39% vs 15%). CONCLUSIONS: Despite similar baseline characteristics, HLHS-NEC infants had significant differences in hospital course compared with HLHS-nNEC neonates. In addition, HLHS-NEC infants were less likely to be fed orally during the entire interstage period. Future studies are needed minimize NEC in this high risk population to possibly improve oral feeds.

Launching a New Strategy for Multidisciplinary Management of Single-Ventricle Heart Defects.

Children born with single-ventricle heart defects, particularly hypoplastic left heart syndrome, have a lifetime high risk of mortality and comorbidities. They have complex medical challenges in addition to their cardiac needs, including growth and feeding complications and neurodevelopmental issues. These concerns require a coordinated effort among specialties to help patients maximize their potential. Additionally, because many complex heart defects are diagnosed prenatally, coordination of care between the pre- and postnatal care teams is imperative. Nursing leadership improves program coordination and efficiency. The purpose of this article is to describe the development and implementation of our hospital's synchronized, multidisciplinary team to support children with single-ventricle heart defects and their families. (Critical Care Nurse. 2018;38[1]:60-71).

Reducing variation in feeding newborns with congenital heart disease.

OBJECTIVE: Enteral feeding is associated with decreased infection rates, decreased mechanical ventilation, decreased hospital length of stay, and improved wound healing. Enteral feeding difficulties are common in congenital heart disease. Our objective was to develop experience-based newborn feeding guidelines for the initiation and advancement of enteral feeding in the cardiothoracic intensive care unit. DESIGN: This is a retrospective analysis of a quality improvement project. SETTING: This quality improvement project was performed in a cardiothoracic intensive care unit. PATIENTS: Newborns admitted to the cardiothoracic intensive care unit for cardiac surgery from January 2011 to May 2015 were retrospectively reviewed. INTERVENTION: Newborn feeding guidelines for the initiation and advancement of enteral feeding were implemented in January 2012. OUTCOME MEASURES: Guideline compliance and clinical variables before and after guideline implementation were reviewed. RESULTS: Compliance with the guidelines increased from 83% in 2012 to 100% in the first two quarters of 2015. Preguidelines (January 2011-December 2011): 45 newborns underwent cardiac surgery; 8 deaths prior to discharge; 1 patient discharged from NICU, therefore, N = 36. Postguidelines (January 2012-May 2015): 131 newborns with 12 deaths, 12 admitted from home, 8 in the NICU, 3 on the floor preop, and 3 back transferred, therefore, N = 93. No difference in feeding preop (post 75% vs pre 69%; P = .5) or full po feeds at discharge (post 78% vs pre 89%; P = .2). Mesenteric ischemia was not statistically different postguidelines (post 6% vs pre 14%; P = .14). Length of hospital stay decreased postguidelines (post 27 + 17 d vs pre 34 + 42 d; P < .001). CONCLUSIONS: Implementation of experience-based newborn feeding guidelines for initiation and advancement of enteral feeding in the cardiothoracic intensive care unit was successful in reducing practice variation supported by increasing guideline compliance. Percentage of patient's full oral feeding at discharge did not change. Length of hospital stay was reduced although cannot be fully attributed to feeding guideline implementation.

Interstage Survival for Patients with Hypoplastic Left Heart Syndrome After ECMO.

There is a reported 5-20 % incidence of extracorporeal membrane oxygenation (ECMO) following stage I palliation for hypoplastic left heart syndrome (HLHS). This study compares the interstage mortality of HLHS patients supported with ECMO (HLHS-ECMO) to those who were not supported with ECMO (HLHS-nECMO) using the National Pediatric Cardiology Quality Improvement Initiative database. Patients with HLHS who survived to hospital discharge after stage I palliation were analyzed. HLHS-ECMO patients were compared to HLHS-non-ECMO patients with respect to demographics, surgical variables, and interstage survival. A total of 931 patients were identified in the database. Sixty-six (7.1 %) patients were supported with ECMO during their stage I palliation admission. There were no statistically significant differences between the groups with respect to demographics or anatomic subtype. HLHS-ECMO patients were more likely to have a preoperative risk factor identified (62 vs. 48 %, p = 0.03) or require ECMO prior to stage I palliation (3 vs. 0.5 %, p = 0.03). HLHS-ECMO patients had a significantly higher incidence of death or transplant versus the HLHS-nECMO group (18 vs. 9 %, p = 0.03). Despite survival to discharge, patients with HLHS requiring ECMO after their palliation continue to have an increased risk of death/cardiac transplant versus patients that do not require ECMO. ECMO use is likely a marker for a high-risk patient group. These patients may benefit from closer follow-up during the interstage period.

Anxiety Scores in Caregivers of Children with Hypoplastic Left Heart Syndrome.

OBJECTIVES: Caring for children with congenital heart disease places significant stress on caregivers. Minimal data exist evaluating stress levels in caregivers of children with hypoplastic left heart syndrome (HLHS). The goal of this study was to obtain baseline stress scores for caregivers of children with HLHS and determine if associations exist between scores and specific caregiver factors. STUDY DESIGN: A cross-sectional study using a web-based survey targeted towards caregivers of children with HLHS was performed. Baseline demographics of the caregiver and child with HLHS were obtained. Caregivers completed three validated questionnaires including the Pediatric Quality of Life Inventory (PedsQL), Parenting Stress Index- Short Form (PSI-SF) and the Pediatric Inventory for Parents (PIP). RESULTS: Four hundred fifty-nine caregivers completed at least one questionnaire. PSI-SF total score was 80.5 ± 23.1 (> 86 = significant stress), PIP frequency total score was 119.0 ± 37.2, and PIP difficulty total score was 118.1 ± 35.7. Lower quality of life was significantly correlated with higher scores on the PSI-SF (r = -0.6), the presence of a developmental issue in the child (r = 0.3) as well as higher scores on the PIP frequency (r = -0.5) and difficulty scales (r = 0.4). Other demographic values for the caregiver and child did not significantly correlate with PSI or PIP total scores. CONCLUSIONS: Anxiety/stress scores of caregivers with children with HLHS are correlated with how well the child is perceived to be doing physically and developmentally by the caregivers. Caregivers with physical and/or developmental concerns may need added psychosocial support.

Caregiver anxiety due to interstage feeding concerns.

INTRODUCTION: Improved weight gain during the interstage (IS) period has been shown to improve overall outcomes in patients with single ventricle physiology (SVP). This emphasis on nutrition may have untoward effects, such as increasing anxiety/stress levels in caregivers, who are already known to be at risk for increased anxiety/stress levels. The goal of this study was to evaluate anxiety/stress levels of caregivers as it pertains to feeding during the IS period and to determine if certain characteristics were associated with higher anxiety/stress scores. METHODS: Caregivers of children with SVP who completed the IS period, defined as the time between the first and second cardiac surgeries, were recruited. Baseline demographics were obtained. Anxiety/stress levels were measured via eight questions using a 0- to 10-point scale. Correlations were performed between demographic variables and anxiety/stress level scores. RESULTS: Fifty-six surveys were completed (39 males, 27 females) on 43 children. Fourteen children required tube feeds during the IS period. There were significant correlations between anxiety/stress scores and caregiver's gender, caregiver's age, caregiver's level of education, percent of time a caregiver spent feeding the child, if caregivers were taking medications for anxiety, and if the child was seen in the emergency room during the IS period. There were no correlation of anxiety/stress scores with caregiver's race, child's underlying cardiac diagnosis, age of child, route of feeding during the IS period, birth order of the child or number of children in the family, relationship status, or distance from the hospital. CONCLUSION: In general, caregivers of children with SVP experience anxiety/stress during the IS period specifically due to feeding concerns. Certain intrinsic and extrinsic characteristics were associated with higher anxiety/stress levels. Future studies are needed to determine how to minimize anxiety/stress levels during this stressful time period.

Survey of how pediatric cardiologists noninvasively evaluate patients with hypoplastic left heart syndrome.

BACKGROUND: The evaluation of right ventricular (RV) function is important in patients with hypoplastic left heart syndrome (HLHS). Echocardiographic qualitative grading has been the prevalent method used in the past, but newer technologies allowing for quantitative assessment of RV function may have changed this fact. The goal of this study was to determine the current routine noninvasive evaluation of patients with HLHS and what, if any, methods are used to assess systolic and diastolic function in this population. METHODS: Web-based survey was conducted using various listservs. Timing of echocardiograms between surgical stages was assessed. Methods of assessing systolic and diastolic function were evaluated. RESULTS: Two hundred seventy-seven physicians who averaged 12.8 ± 9.6 years removed from training responded. Largest percentage of respondents was echocardiographers (44.2%) in a university-based practice (73.3%) from North America (91.7%). There were 54.3% of respondents who performed echocardiograms monthly between stages I and II, 48.8% who performed echocardiograms every 6 months between stages II and III, and 67.0% who performed echocardiograms annually after stage III procedure. The main method for systolic grading was qualitative grading (95.5%) and for diastolic grading were tricuspid blood inflow velocities (56.8%). Qualitative grading was considered the method of choice for systolic grading for 38.8% of respondents and tissue Doppler velocities was the method of choice for diastolic grading for 35.3% of respondents. There were 4.0% of respondents who routinely perform a cardiac magnetic resonance imaging (cMRI) between stages I and II, 8.0% between stages II and III, and 24.2% after stage III procedure. CONCLUSION: Variability in the noninvasive assessment of the RV in patients with HLHS continues to exist. Qualitative RV systolic assessment was still the predominant method used to assess function despite newer imaging techniques to allow for quantification. Future studies are needed to determine which values are most useful in reviewing function in this complex patient population.

Interstage weight gain for patients with hypoplastic left heart syndrome undergoing the hybrid procedure.

BACKGROUND: Weight gain during the interstage (IS) period for hypoplastic left heart (HLHS) patients has been associated with improved outcomes. IS home monitoring has been shown to improve mortality. No data exist on IS weight gain and home monitoring effects on weight gain for HLHS patients undergoing the hybrid procedure. OBJECTIVE: Goal of this study was to describe the weight gain of patients with HLHS undergoing the hybrid procedure during the IS period, to determine if weight parameters were associated with mortality, and to determine if home monitoring improved weight gain. METHODS: Retrospective review was performed. Patients were included if they had the diagnosis of HLHS and underwent hybrid procedure. Baseline demographics, surgical dates, and all IS weights were recorded. RESULTS: Forty-four patients met inclusion criteria, 24 patients had IS monitoring. Time period evaluated was from April 2006 to June 2011. Mean birth weight of the total population was 3.13 ± 0.61 kg, age at hybrid was 5.84 ± 4.10 days, weight z-score at hybrid discharge was -1.66 ± 1.01, age at pre-Stage II was 6.12 ± 1.37 months, IS weight gain was 16.85 ± 5.94 g/day, and weight z-score pre-Stage II was -2.25 ± 1.28. Monitored patients had significantly higher weight z-score pre-Stage II (-1.67 ± 0.98 vs. -2.82 ± 1.28) and lower change in weight z-score (-0.26 ± 0.97 vs. -1.24 ± 1.06). Eight patients died IS. There was a significant difference in weight gain per day in those that survived the IS period (17.87 ± 4.75 g/day vs. 12.28 ± 8.65 g/day). There were no significant differences in weight characteristics in patients that survived the Stage II procedure (n = 28) vs. those that did not (n = 7). CONCLUSION: Home monitoring improved IS weight gain in patients undergoing the hybrid procedure. Decreased weight gain per day was associated with IS mortality.