bims-hylehe 2019-09-01 papers

bims-hylehe

Biomed News

on Hypoplastic left heart syndrome

Issue of 2019–09–01
eleven papers selected by
Richard James, University of Pennsylvania

Mental health care for parents of babies with congenital heart disease during intensive care unit admission: Systematic review and statement of best practice.
Care of infants, children and adults with congenital heart disease.
First-Year Outcomes of Critical Congenital Heart Disease Screening in Maryland.
Development of a clinician-parent home care education intervention.
Early Neurodevelopmental Outcomes in Children with Hypoplastic Left Heart Syndrome and Related Anomalies After Hybrid Procedure.
Minimum Travel Distance Among Publicly Insured Infants with Severe Congenital Heart Disease: Potential Impact of In-state Restrictions.
Parental emotional and hands-on work-Experiences of parents with a newborn undergoing congenital heart surgery: A qualitative study.
The accuracy of prenatal diagnosis of major congenital heart disease is increasing.
Individualised developmental care for babies and parents in the NICU: Evidence-based best practice guideline recommendations.
Brain Injury in Infants with Critical Congenital Heart Disease: Insights from Two Clinical Cohorts with Different Practice Approaches.
Evaluation of an Early Language and Literacy Program for Parents of Infants in the NICU.

Early Hum Dev. 2019 Aug 24. pii: S0378-3782(19)30475-X. [Epub ahead of print] 104837

Mental health care for parents of babies with congenital heart disease during intensive care unit admission: Systematic review and statement of best practice.

Nadine A Kasparian, Janice M Kan, Erica Sood, Jo Wray, Harold A Pincus, Jane W Newburger.

   INTRODUCTION: Congenital heart disease (CHD) is one of the most common causes of infant admission to pediatric intensive care and is associated with profound psychological stress for mothers, fathers and their infants. Intensive care unit admission represents an opportunity to offer evidence-based strategies to prevent or minimize severe psychological distress and promote secure bonding and attachment, alongside high-quality infant medical care.
OBJECTIVES: We aimed to identify, synthesize and critically appraise published evidence on the efficacy and cost-effectiveness of mental health interventions delivered in neonatal, pediatric or cardiac intensive care units for parents of infants with CHD. A secondary goal was to develop recommendations for advancing health policy, practice and research in the field.
METHODS: In accordance with a prospectively registered protocol (CRD42019114507), six electronic databases were systematically searched for studies reporting results of a controlled trial of a mental health intervention for parents of infants aged 0-12 months with a congenital anomaly requiring intensive care unit admission. To maximize generalizability of results, trials involving infants with any type of structural congenital anomaly requiring surgery were included. Outcomes included intervention type, process, efficacy, and cost-effectiveness.
RESULTS: Across all forms of congenital anomaly, only five trials met inclusion criteria (four in CHD, one in gastrointestinal malformation). All interventions engaged parents face-to-face, but each had a distinct therapeutic approach (parent-infant interaction and bonding, early pediatric palliative care, psycho-education, parenting skills training, and family-centered nursing). Four of the five trials demonstrated efficacy in reducing maternal anxiety, although the quality of evidence was low. Positive results were also found for maternal coping, mother-infant attachment, parenting confidence and satisfaction with clinical care, as well as infant mental (but not psychomotor) development at 6 months. Mixed results were found for maternal depression and infant feeding. No evidence of efficacy was found for improving parent, infant or family quality of life, physical health or length of infant hospital stay, and there were no data on cost-effectiveness.
CONCLUSIONS: Stronger evidence for the efficacy of mental health interventions to buffer the effects of intensive care unit admission for parents of infants with CHD is urgently needed. Robust, high-quality trials are lacking, despite the established need and demand, and health policies prioritizing parent mental health care in the context of early childhood adversity are needed.

Keywords:  Anxiety; Behavioral health; Cardiac intensive care unit; Congenital anomaly; Congenital heart disease; Guidelines; Mental health; Neonatal intensive care unit; Pediatric intensive care unit; Psychological intervention; Systematic review

DOI:  https://doi.org/10.1016/j.earlhumdev.2019.104837
Nurs Stand. 2019 Jul 26. 34(8): 37-42

Care of infants, children and adults with congenital heart disease.

Kerry Gaskin, Fiona Kennedy.

  Congenital heart disease refers to an anatomical or physiological defect of the heart that is present at birth. Over the past three decades there have been significant advances in surgical, medical and nursing care for infants and children requiring cardiac surgery for congenital heart disease. This has meant that the number of adults with moderate and complex congenital heart disease now exceeds the number of children with the disease. This article details the background and diagnosis of congenital heart disease. It considers the implications for infants and children with congenital heart disease, as well as their parents, such as screening methods, risk factors, parental stress and psychosocial needs. This article also discusses the care of adults with congenital heart disease, including specialist monitoring and patient education.

Keywords:  cardiac arrhythmias; cardiology; cardiorespiratory; child health; heart diseases; heart failure; infants; organ transplantation; pacemakers

DOI:  https://doi.org/10.7748/ns.2019.e11405
Glob Pediatr Health. 2019 ;6 2333794X19868226

First-Year Outcomes of Critical Congenital Heart Disease Screening in Maryland.

Deborah Badawi, Johnna Watson, Steven Maschke, Lawrence Reid.

  Objectives. Newborn screening for critical congenital heart disease (CCHD) was added to the Recommended Uniform Screening Panel in 2011, and states have been gradually adding pulse oximetry as point-of-care screening to panels. Few data are available on the effectiveness of pulse oximetry as a mandated screening. This study describes outcomes of the first year of screening in Maryland. Methods. A web-based data collection tool for screening results and outcomes, eScreener Plus, was utilized. Data collected from the start of screening from September 1, 2012, to December 31, 2013, were analyzed. Well-baby nursery data were evaluated separately from neonatal intensive care unit (NICU) data to determine whether setting influenced effectiveness. Results. In the first 15 months of newborn screening for CCHD in Maryland, 4 asymptomatic infants were diagnosed with a critical cardiac condition by newborn screening. Eleven infants passed but were later identified with a primary or secondary target condition. Seventy-one percent of infants with CCHD were identified prenatally or by clinical signs and symptoms. Pulse oximetry screening for CCHD had a specificity of more than 99% in both the well-baby nursery and the NICU. Sensitivity in the well-baby nursery was 10% and 60% in the NICU. Conclusion. Further investigation and interpretation of specific protocols that were used and outcomes of screening is needed for continued refinement of the well-baby algorithm and NICU protocol development. Pulse oximetry screening in newborns provides valuable clinical information, but many infants with CCHD are still not identified with current protocols.

Keywords:  Critical Congenital Heart Disease; Newborn Screening; Pulse Oximetry Screening

DOI:  https://doi.org/10.1177/2333794X19868226
Cardiol Young. 2019 Aug 30. 1-6

Development of a clinician-parent home care education intervention.

Blake Lane, Samuel P Hanke, Barbara Giambra, Nicolas L Madsen, Sandra L Staveski.

   OBJECTIVES: To examine the strengths and opportunities for improvement of current home care education practices to inform the development of the Home Care for Heart Health intervention, and to develop a web-based intervention for parents and clinicians with complimentary print materials that could provide the right education at the right time to foster a safer transition from hospital to home.
METHODS: An inter-professional focus group of parents, clinicians, and designers was formed to co-create a home care education intervention for parents of children with congenital heart disease (CHD) and their care team. We used the Integrated New Product Development process model created by Jonathon Cagan and Craig Vogel at Carnegie Mellon University to develop the intervention. This process model is a way of thinking that combines horizontal and inter-disciplinary teams, stakeholder-centric focus, and a system of qualitative discovery and development evolving towards quantitative methods of refinement.
RESULTS: Our team developed the Home Care for Heart Health intervention. The evidenced-based intervention includes a quick reference guide for parents of children with CHD, an accompanying app, family-friendly pathways, and clinician education.
CONCLUSION: Using an inter-professional approach, our team of clinicians, parents, and design experts were able to co-create a clinician-parent home care education intervention with broad application and lifelong relevance to the Congenital Heart Disease Community.
PRACTICE IMPLICATIONS: Our intervention has the potential to be used as a model for other home care education interventions for parents of children with chronic illnesses.

Keywords:  Parent home care education; design-thinking methodology; discharge education

DOI:  https://doi.org/10.1017/S1047951119001318
Pediatr Cardiol. 2019 Aug 30.

Early Neurodevelopmental Outcomes in Children with Hypoplastic Left Heart Syndrome and Related Anomalies After Hybrid Procedure.

O M Khalid, T M Harrison.

  The hybrid strategy is one approach to single ventricle palliation. In this study, we reported neurodevelopment at 12 months for two cohorts of children managed with the hybrid and clinical factors associated with neurodevelopment in the entire sample. We performed a retrospective study of children with single ventricle who had undergone a neonatal hybrid procedure. One group included infants with hypoplastic left heart syndrome (HLHS); another group included infants with non-HLHS single ventricle. Neurodevelopment was assessed with 12-month Bayley III. Parametric and non-parametric statistics were used for analysis. Nine infants with HLHS and 15 with non-HLHS were identified. Abnormal neurodevelopment was identified in 11 of 24 (46%), primarily motor (46%). Development did not differ between groups. In the whole sample, higher lactate levels were associated with lower cognitive scores (p = 0.04). Fewer mechanical ventilation days were associated with higher cognitive scores (p = 0.05) after Stage 1 and higher motor scores after Stage 2. Shorter ICU length of stay (p = 0.01), shorter hospital length of stay (p = 0.01), and fewer complications (p = 0.01) after stage 2 were associated with higher motor scores. Higher cognitive (p = 0.02) and language (p = 0.002) scores were associated with higher weight at 12 months. In the largest cohort of single ventricle children treated with neonatal hybrid palliation yet reported, significant neurodevelopmental impairment was identified. No differences in neurodevelopment were found between children with HLHS and those with non-HLHS variants. A multicenter trial is needed to test differences in neurodevelopment between hybrid and Norwood approaches.

Keywords:  Congenital heart disease; Hybrid procedure; Hypoplastic left heart syndrome; Neurodevelopmental outcome

DOI:  https://doi.org/10.1007/s00246-019-02191-3
Pediatr Cardiol. 2019 Aug 28.

Minimum Travel Distance Among Publicly Insured Infants with Severe Congenital Heart Disease: Potential Impact of In-state Restrictions.

Joyce L Woo, Brett R Anderson, Daniel Gruenstein, Rena Conti, Kao-Ping Chua.

  Travel distance to surgical centers may be increased when coverage restrictions prevent children with congenital heart disease (CHD) from receiving care at out-of-state congenital heart surgery centers. We estimated the minimum travel distance to congenital heart surgery centers among publicly insured infants with time-sensitive CHD surgical needs, under two different scenarios: if they were and were not restricted to in-state centers. Using 2012 Medicaid Analytic eXtract data from 40 states, we identified 4598 infants with CHD that require surgery in the first year of life. We calculated the minimum travel distance between patients' homes and the nearest cardiac surgery center, assuming patients were and were not restricted to in-state centers. We used linear regression to identify demographic predictors of distance under both scenarios. When patients were not restricted to in-state centers, mean minimum travel distance was 43.7 miles, compared to 54.1 miles when they were restricted. For 5.9% of patients, the difference in travel distance under the two scenarios exceeded 50 miles. In six states, the difference in mean minimum travel distance exceeded 20 miles. Under both scenarios, distance was positively predicted by rural status, residence in middle-income zip codes, and white/non-Hispanic or American Indian/Alaskan Native race/ethnicity. For some publicly insured infants with severe CHD, facilitating the receipt of out-of-state care could mitigate access barriers. Existing efforts to regionalize care at fewer centers should be designed to avoid exacerbating access barriers among publicly insured CHD patients.

Keywords:  Congenital heart disease; Ethics and policy; Health services research

DOI:  https://doi.org/10.1007/s00246-019-02193-1
J Spec Pediatr Nurs. 2019 Aug 30. e12269

Parental emotional and hands-on work-Experiences of parents with a newborn undergoing congenital heart surgery: A qualitative study.

Mirjam Thomi, Jean-Pierre Pfammatter, Elisabeth Spichiger.

   PURPOSE: To explore both mothers' and fathers' experiences from prenatal or postnatal diagnosis of their newborn's congenital heart disease (CHD) to the first discharge after heart surgery in a Swiss university children's hospital.
DESIGN AND METHOD: A qualitative research approach, based on a constructivist paradigm, was applied to explore participants' experiences and perceptions. Parents of nine children with moderate to severe CHD participated in semistructured joint couple interviews. Data were analyzed inductively via an iterative process, following the steps of thematic analysis.
RESULTS: Between the child's CHD diagnosis and hospital discharge after neonatal cardiac surgery, the overarching theme for parents was being confronted with demanding emotional and hands-on work. This parental work included four themes with subthemes: Parents had to tackle a route through an unknown hospital world from receiving the CHD diagnosis and experiencing delivery to attending to their child in the pediatric intensive care unit and during surgery, as well as during the transfer to and the stay on the pediatric cardiac unit. They experienced an at times challenging interplay with health care professionals, performed teamwork as the nuclear family and managed concerned relatives and friends.
PRACTICE IMPLICATIONS: Health care professionals should establish trustful relationships with parents, while accompanying families continuously, providing consistent, straightforward information, and expressing appreciation for the parents' exceptional emotional and hands-on work. Health care professionals' awareness of parent's experiences is vital to compassionate family-centered care.

Keywords:  congenital heart defect; heart surgery; infant newborn; parents; qualitative research

DOI:  https://doi.org/10.1111/jspn.12269
J Obstet Gynaecol. 2019 Aug 28. 1-8

The accuracy of prenatal diagnosis of major congenital heart disease is increasing.

Rebekka Lytzen, Niels Vejlstrup, Jesper Bjerre, Olav Bjørn Petersen, Stine Leenskjold, James Keith Dodd, Finn Stener Jørgensen, Lars Søndergaard.

  Regular audit of results of prenatal screening for congenital heart disease (CHD) is crucial to ensure reliable prenatal diagnosis. We aimed to assess the accuracy of prenatal diagnosis of major CHD between 1996 and 2013. During the study period, prenatal detection of major CHD improved from 4.5% to 71.0% (p<.001). Prenatal diagnoses on 628 live born children and terminated pregnancies were compared with postnatal findings or autopsy reports. The proportion of correct diagnoses increased throughout the study period from 42.9% in 1996 and reached 88.2% in 2013 (p<.001). A total of 32 foetuses with suspected major CHD were terminated though no major CHD was found at autopsy. In these pregnancies, termination was mainly performed due to other anomalies in the foetus. Along with improved detection of major CHD, the validity of a prenatal diagnosis is increasing. No cases of misinterpreted major CHD resulted in the termination of a healthy foetus in this study. Impact statement What is already known on this subject? Prenatal diagnosis of isolated congenital heart disease (CHD) correlates well with lesions found during autopsy performed in terminated foetuses. Few studies have assessed the accuracy of prenatal diagnosis of major CHD in live born children, cases with associated anomalies and the time trend in validity. What the results of this study add? This study illustrates that the validity of prenatal diagnosis of major CHD is increasing. Prenatal diagnoses in terminated pregnancies as well as in live born children is high except for coarctation of the aorta and atrioventricular septal defects. Chromosomal anomalies are associated with lower accuracy of prenatal diagnosis. What the implications are of these findings for clinical practice and/or further research? Prenatal diagnosis is an accurate tool for detecting major CHD. Misinterpretation has not led to the termination of a healthy foetus; however, this study illustrates that vigilant care should be placed on the cardiac evaluation when termination is considered due to the cardiac defect.

Keywords:  Congenital heart disease; accuracy; autopsy; foetal medicine; prenatal diagnosis; validity

DOI:  https://doi.org/10.1080/01443615.2019.1621814
Early Hum Dev. 2019 Aug 21. pii: S0378-3782(19)30478-5. [Epub ahead of print] 104840

Individualised developmental care for babies and parents in the NICU: Evidence-based best practice guideline recommendations.

Nadine Griffiths, Kaye Spence, Alison Loughran-Fowlds, Bjorn Westrup.

  Advances in neonatal care have improved survival of premature and critically ill infants; and while rates of some long-term neurodevelopmental problems in survivors have improved, such as cerebral palsy, there are others such as learning and behavioural difficulties that have not. The goal of improving long term neurodevelopmental morbidity has led to an increased focus on improving developmental care not only in neonatal long term follow- up clinics but within the NICU itself to capture the period of earliest brain neuroplasticity. The application of a systematic approach to improve practice is considered the most effective strategy for implementing neuroprotective developmentally supportive care. The content of this paper incorporates evidence-based systematic reviews to guide clinicians in the application of developmentally supportive interventions.

Keywords:  Developmental care; Neonate; Neuroprotective care; Neurosupportive developmental care

DOI:  https://doi.org/10.1016/j.earlhumdev.2019.104840
J Pediatr. 2019 Aug 23. pii: S0022-3476(19)30869-8. [Epub ahead of print]

Brain Injury in Infants with Critical Congenital Heart Disease: Insights from Two Clinical Cohorts with Different Practice Approaches.

Nathalie H P Claessens, Vann Chau, Linda S de Vries, Nicolaas J G Jansen, Stephanie H Au-Young, Raymond Stegeman, Susan Blaser, Manohar Shroff, Felix Haas, Davide Marini, Johannes M P J Breur, Mike Seed, Manon J N L Benders, Steven P Miller.

OBJECTIVES: To determine prevalence and risk factors for brain injury in infants with critical congenital heart disease (CHD) from 2 sites with different practice approaches who were scanned clinically.
STUDY DESIGN: Prospective, longitudinal cohort study (2016-2017) performed at Hospital for Sick Children Toronto (HSC) and Wilhelmina Children's Hospital Utrecht (WKZ), including 124 infants with cardiac surgery ≤60 days (HSC = 77; WKZ = 47). Magnetic resonance imaging was performed per clinical protocol, preoperatively (n = 100) and postoperatively (n = 120). Images were reviewed for multifocal (watershed, white matter injury) and focal ischemic injury (stroke, single white matter lesion).
RESULTS: The prevalence of ischemic injury was 69% at HSC and 60% at WKZ (P = .20). Preoperative multifocal injury was associated with low cardiac output syndrome (OR, 4.6), which was equally present at HSC and WKZ (20% vs 28%; P = .38). Compared with WKZ, HSC had a higher prevalence of balloon-atrioseptostomy in transposition of the great arteries (83% vs 53%; P = .01) and more frequent preoperative focal injury (27% vs 6%; P = .06). Postoperatively, 30% of new multifocal injury could be attributed to postoperative low cardiac output syndrome, which was equally present at HSC and WKZ (38% vs 28%; P = .33). Postoperative focal injury was associated with intraoperative selective cerebral perfusion in CHD with arch obstruction at both sites (OR, 2.7). Compared with HSC, WKZ had more arch obstructions (62% vs 35%; P < .01) and a higher prevalence of new focal injury (36% vs 16%; P = .01).
CONCLUSIONS: Brain injury is common in clinical cohorts of infants with critical CHD and related to practice approaches. This study confirms that the high prevalence of brain injury in critical CHD is a clinical concern and does not simply reflect the inclusion criteria of published research studies.

DOI: https://doi.org/10.1016/j.jpeds.2019.07.017
Neonatal Netw. 2019 Jul 01. 38(4): 206-216

Evaluation of an Early Language and Literacy Program for Parents of Infants in the NICU.

Lisa Shanty, Rebecca Dowling, Susan Sonnenschein, Brenda Hussey-Gardner.

   PURPOSE: To evaluate the effect of a NICU parent education program on parents' early language and literacy practices, and on their confidence interpreting and responding to infant signals.
DESIGN: Single group, pre- and post-test, mixed-methods evaluation design.
SAMPLE: One hundred and four parents and other caregivers completed questionnaires before and after the one-hour program. Ten parents participated in follow-up interviews.
MAIN OUTCOME VARIABLES: Before and after sessions, participants reported on frequency of their current and intended early language and literacy practices, and their confidence interpreting and responding to infant signals. Participants also reported program satisfaction. Interview participants reported their behavior change one to two weeks later.
RESULTS: The program significantly increased intention to engage in more early language and literacy practices, and increased parent-reported knowledge of how and when to interact with their infants. The majority of interviewed parents reported engaging in these practices one to two weeks later.

Keywords:  NICU parent education; early language and literacy; neonatal intensive care; parent–infant interaction

DOI:  https://doi.org/10.1891/0730-0832.38.4.206