Supporting parenting during infant hospitalisation for CHD.

OBJECTIVE: To characterise the parenting priorities of mothers and fathers of infants hospitalised with CHD and generate recommendations to support parenting during infant hospitalisation. STUDY DESIGN: Through online crowdsourcing, an innovative research methodology to create an online community to serve as a research sample, 79 parents of young children with CHD responded to questions about parenting during hospitalisation via private social networking site. Responses were analysed using qualitative research methods. RESULTS: Three broad themes were identified: (1) establishing a bond with my baby, (2) asserting the parental role, and (3) coping with fear and uncertainty. Parents value provider support in restoring normalcy to the parenting experience during infant hospitalisation. CONCLUSIONS: Care teams can support parenting during infant hospitalisation by promoting parents' roles as primary caretakers and decision-makers and attending to the emotional impact of infant hospitalisation on the family.

Survival After Norwood Procedure in High-Risk Patients.

BACKGROUND: Multiple single-ventricle populations are noted to be at increased risk for mortality after the Norwood procedure. Preoperative risk factors include low birth weight, restrictive/intact atrial septum, obstructed pulmonary veins, ventricular dysfunction, and atrioventricular valve regurgitation. We report outcomes of the Norwood procedure in standard- and high-risk patients in the recent era. METHODS: All patients born with hypoplastic left heart syndrome between 2006 and 2016 who underwent a Norwood procedure at our institution were included. Patient data were retrospectively reviewed, and Kaplan-Meier analysis was used to evaluate survival between groups. RESULTS: The cohort included 177 patients. Fifty patients were determined high-risk preoperatively: low birth weight (n = 18), ventricular dysfunction/atrioventricular valve regurgitation (n = 13), intact or restrictive atrial septum/obstructed anomalous pulmonary venous return (n = 14), and multiple factors (n = 5). There were 2 (1.6%) deaths before Glenn in the standard-risk group, with a total of 10 (20%) from the high-risk groups (P < .0001). Survival at 1 year differed greatly between groups, with highest being standard risk at 89% and lowest in the intact septum/obstructed veins group at 54%. The difference between groups in long-term survival was significant (P < .001). CONCLUSIONS: Outcomes after the Norwood procedure have improved for standard-risk patients. Those with preoperative risk factors account for most of the early deaths after the Norwood procedure. This high-risk status does not resolve after Glenn, because longer-term survival continues to diverge from the standard-risk group.

Selective Use of Inpatient Interstage Management After Norwood Procedure.

BACKGROUND: We report our intermediate-term results after Norwood procedure, including use of an interstage inpatient management strategy for high-risk patients, and seek to create a predictive model for probability of discharge. METHODS: A single-site retrospective review was conducted for all patients undergoing Norwood procedure from 2006 to 2016 (N = 177). We compared those discharged home with those who either remained hospitalized until Glenn procedure or died before Norwood procedure discharge. Multivariable logistic regression was used to develop a predictive model for discharge. RESULTS: During the study period, 120 (68%) patients were discharged home, 45 (25%) remained hospitalized, and 12 (7%) died before Glenn procedure (median age: 71 days). Interstage survival for those discharged after Norwood procedure was 100%. Longitudinal survival for the cohort was 86%, 81%, and 77% at 1, 5, and 10 years, resepectively. Ten-year survival was significantly greater for the discharged group compared with the interstage inpatients (86% vs 56%, P < .001). A reduced predictive model of discharge included lower gestational age (odds ratio [OR]: 0.95), lower median income for ZIP code (OR: 0.4), lower birth-weight-for-age z-score (OR: 0.56), longer cardiopulmonary bypass time (OR: 0.45), and Blalock-Taussig shunt (OR: 0.32). CONCLUSIONS: Survival up to 10 years after Norwood procedure is good using a strategy of inpatient care for a subset of high-risk patients to mitigate home interstage mortality. A probabilistic model used after Norwood procedure was able to predict interstage discharge with good accuracy, but will require external validation to ensure generalizability. Further work is also needed to determine optimal palliative pathways for the high-risk patients because of the notable attrition beyond successful bidirectional Glenn procedure.

Reintervention following stage 1 palliation: A report from the NPC-QIC Registry.

BACKGROUND: Single ventricle heart disease with aortic arch hypoplasia has high morbidity and mortality, with the greatest risk after stage 1 palliation. Residual lesions often require catheter-based or surgical reintervention to minimize risk. We sought to describe the types, frequency, and risk factors for re-intervention between stage 1 and stage 2 palliation, utilizing the National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC) registry. METHODS: The NPC-QIC registry, consisting of patients discharged after stage 1 palliation, was queried. Hybrid stage 1 palliation patients were excluded from this study. The primary risk factor was shunt type and the primary outcome was re-intervention. RESULTS: Of 1156 patients, (50%) had re-intervention. There was no difference in total rate of re-intervention by shunt type (BT shunt 52% vs. RVPA shunt 48%; P = .17). Patients with a BT shunt had increased re-intervention during stage 1 hospitalization (P =.002). During the interstage period, following discharge from stage 1 palliation, patients with a BT shunt had increased aortic arch re-intervention (P < .005), while patients with an RVPA shunt had increased re-intervention on the shunt and the pulmonary arteries (P = .02). Postoperative mechanical ventilation >14 d (P < .01) was the only risk factor associated with re-intervention by multivariable analysis, regardless of shunt type. CONCLUSIONS: Re-intervention between stage I and stage 2 palliation is common. There is no difference in cumulative frequency of re-intervention between shunt types, though types and timing of re-intervention varied between shunt types. Longitudinal assessment of the NPC-QIC database is important to identify long term outcomes of patients requiring re-intervention.

Risk Factors for Abnormal Developmental Trajectories in Young Children With Congenital Heart Disease.

BACKGROUND: Children with congenital heart disease are at risk for developmental delay. This study sought to identify early risk factors for abnormal developmental trajectories in children with congenital heart disease. METHODS AND RESULTS: Children with congenital heart disease at high risk for developmental delay, without known genetic abnormality, and with ≥3 assessments by the use of the Bayley Scales of Infant and Toddler Development, Third Edition, were studied. Logistic regression was used to assess the impact of patient and clinical factors on cognitive, language, and motor score trajectories; classified as: average or improved if all scores were ≥85 (<1 standard deviation below the mean) or increased to ≥85 and never decreased; or abnormal if all scores were <85, fell to <85 and never improved, or fluctuated above and below 85. Data on 131 children with 527 Bayley Scales of Infant and Toddler Development, Third Edition assessments were analyzed. Subject age was 5.5 to 37.4 months. Overall, 56% had cognitive, language, and motor development in the average range. Delays occurred in single domains in 23%. Multiple domains were delayed in 21%. More cardiac surgeries, longer hospital stay, poorer linear growth, and tube feeding were associated with worse outcomes in all domains (P<0.05). In the multivariable model, the need for tube feeding was a risk factor for having an abnormal developmental trajectory (odds ratio, 5.1-7.9). Minority race and lack of private insurance had significant relationships with individual domains. CONCLUSIONS: Longitudinal developmental surveillance identified early factors that can help quantify the risk of developmental delay over time. Strategies to improve modifiable factors and early therapeutic intervention can be targeted to children at highest risk.

Effect of feeding modality on interstage growth after stage I palliation: a report from the National Pediatric Cardiology Quality Improvement Collaborative.

OBJECTIVES: Achieving adequate growth after stage 1 palliation for children with single-ventricle heart defects often requires supplemental nutrition through enteral tubes. Significant practice variability exists between centers in the choice of feeding tube. The impact of feeding modality on the growth of patients with a single ventricle after stage 1 palliation was examined using the multiinstitutional National Pediatric Cardiology Quality Improvement Collaborative data registry. METHODS: Characteristics of patients were compared by feeding modality, defined as oral only, nasogastric tube only, oral and nasogastric tube, gastrostomy tube only, and oral and gastrostomy tube. The impact of feeding modality on change in weight for age z-score during the interstage period, from stage 1 palliation discharge to stage 2 palliation, was evaluated by multivariable linear regression, adjusting for important patient characteristics and postoperative morbidities. RESULTS: In this cohort of 465 patients, all groups demonstrated improved weight for age z-score during the interstage period with a mean increase of 0.3±0.8. In multivariable analysis, feeding modality was not associated with differences in the change in weight for age z-score during the interstage period (P=.72). Risk factors for poor growth were a diagnosis of hypoplastic left heart syndrome (P=.003), vocal cord injury (P=.007), and lower target caloric goal at discharge (P=.001). CONCLUSIONS: In this large multicenter cohort, interstage growth improved for all groups and did not differ by feeding modality. With appropriate caloric goals and interstage monitoring, adequate growth may be achieved regardless of feeding modality and therefore local comfort and complication risk should dictate feeding modality.