bims-hylehe Biomed news
on Hypoplastic left heart syndrome
Issue of 2019‒01‒13
twelve papers selected by
Richard James
University of Pennsylvania


  1. Int J Cardiol. 2018 Dec 20. pii: S0167-5273(18)35907-2. [Epub ahead of print]
    Amedro P, Gavotto A, Legendre A, Lavastre K, Bredy C, De La Villeon G, Matecki S, Vandenberghe D, Ladeveze M, Bajolle F, Bosser G, Bouvaist H, Brosset P, Cohen L, Cohen S, Corone S, Dauphin C, Dulac Y, Hascoet S, Iriart X, Ladouceur M, Mace L, Neagu OA, Ovaert C, Picot MC, Poirette L, Sidney F, Soullier C, Thambo JB, Combes N, Bonnet D, Guillaumont S.
      BACKGROUND: Advances in congenital heart disease (CHD) have transferred the mortality from childhood to adulthood. Exercise capacity in young patients with CHD remains lower than in the general population, resulting in deconditioning and impaired quality of life. Evidence based-medicine in cardiac rehabilitation in this age group with CHD remains limited. We present the QUALI-REHAB study rationale, design and methods.METHODS: The QUALI-REHAB trial is a nationwide, multicentre, randomised, controlled study, aiming to assess the impact of a combined centre and home-based cardiac rehabilitation program on the quality of life of adolescents and young adults (13 to 25 years old) with CHD. Patients with a maximum oxygen uptake (VO2max) < 80% and/or a ventilatory anaerobic threshold (VAT) < 55% of predicted VO2max, will be eligible. Patients will be randomised into 2 groups (12-week cardiac rehabilitation program vs. controls). The primary outcome is the change in the PedsQL quality of life score between baseline and 12-month follow-up. A total of 130 patients are required to observe a significant increase of 7 ± 13.5 points in the PedsQL, with a power of 80% and an alpha risk of 5%. The secondary outcomes are: VO2max, VAT, stroke volume, clinical outcomes, physical and psychological status, safety and acceptability.
    CONCLUSION: After focusing on the survival in CHD, current research is opening on secondary prevention and patient-related outcomes. The QUALI-REHAB trial intends to assess if a combined centre and home-based rehabilitation program, could improve the quality of life and the exercise capacity in youth with CHD.
    TRIAL REGISTRATION: Clinicaltrials.gov (NCT03690518).
    Keywords:  Cardiac rehabilitation; Cardiovascular deconditioning; Congenital heart defect; Health education; Health-related quality of life; Secondary prevention
    DOI:  https://doi.org/10.1016/j.ijcard.2018.12.050
  2. Congenit Heart Dis. 2019 Jan 12.
    Hoch JM, Fatusin O, Yenokyan G, Thompson WR, Lefton-Greif MA.
      BACKGROUND: Tube feedings are often needed to achieve the growth and nutrition goals associated with decreased morbidity and mortality in patients with single ventricle anatomy. Variability in feeding method through the interstage period has been previously described, however, comparable information following stage 2 palliation is lacking.OBJECTIVES: To identify types of feeding methods following stage 2 palliation and their influence on length of stay.
    DESIGN: Secondary analysis of the National Pediatric Cardiology Quality Improvement Collaborative registry was performed on 932 patients. Demographic data, medical characteristics, postoperative complications, type of feeding method, and length of stay for stage 2 palliation were analyzed.
    RESULTS: Type of feeding method remained relatively unchanged during hospitalization for stage 2 palliation. Gastrostomy tube fed only patients were the oldest at time of surgery (182.7 ± 57.7 days, P < .001) and had the lowest weight-for-age z scores at admission (-1.6 ± 1.4, P < .001). Oral + gastrostomy tube groups had the longest median bypass times (172.5 minutes, P = .001) and longest length of stay (median 12 days, P < .001). Multivariable modeling revealed that feeding by tube only (P < .001), oral + tube feeding (P ≤ .001), reintubation (P < .001), and prolonged intubation (P < .001) were associated with increased length of stay. Neither age (P = .156) nor weight-for-age z score at admission (P = .066) was predictive of length of stay.
    CONCLUSIONS: Feeding methods established at admission for stage 2 palliation are not likely to change by discharge. Length of stay is more likely to be impacted by tube feeding and intubation history than age or weight-for-age z score at admission. Better understanding for selection of feeding methods and their impact on patient outcomes is needed to develop evidence-based guidelines to decrease variability in clinical practice patterns and provide appropriate counseling to caregivers.
    Keywords:  deglutition; feeding; hypoplastic left heart syndrome; single ventricle; stage 2 palliation
    DOI:  https://doi.org/10.1111/chd.12742
  3. Trends Neurosci. 2019 Jan 04. pii: S0166-2236(18)30316-3. [Epub ahead of print]
    Leonetti C, Back SA, Gallo V, Ishibashi N.
      Congenital heart disease (CHD) is among the most common birth defects. Children with CHD frequently display long-term intellectual and behavioral disability. Emerging evidence indicates that cardiac anomalies lead to a reduction in cerebral oxygenation, which appears to profoundly impact on the maturation of cerebral regions responsible for higher-order cognitive functions. In this review we focus on the potential mechanisms by which dysregulation of cortical neuronal development during early life may lead to the significant cognitive impairments that commonly occur in children with CHD. Further understanding of the mechanisms underlying cortical dysmaturation due to CHD will be necessary to identify strategies for neonatal neuroprotection and for mitigating developmental delays in this patient population.
    Keywords:  congenital; cortex; heart; hypoxia; interneuron; perinatal
    DOI:  https://doi.org/10.1016/j.tins.2018.12.003
  4. Arch Cardiovasc Dis. 2019 Jan 03. pii: S1875-2136(18)30183-9. [Epub ahead of print]
    Cohen S, Jannot AS, Iserin L, Bonnet D, Burgun A, Escudié JB.
      BACKGROUND: The content of electronic medical records (EMRs) encompasses both structured data, such as billing codes, and unstructured data, including free-text reports. Epidemiological and clinical research into adult congenital heart disease (ACHD) increasingly relies on administrative claim data using the International Classification of Diseases (9th revision) (ICD-9). In France, administrative databases use ICD-10, the reliability of which is largely unknown in this context.AIMS: To assess the accuracy of ICD-10 codes retrieved from administrative claim data in the identification and classification of ACHD.
    METHODS: We randomly included 6000 patients hospitalized at least once in 2000-2014 in a cardiology department with a dedicated specialized ACHD Unit. For each patient, the clinical diagnosis extracted from the EMR was compared with the assigned ICD-10 codes. Performance of ICD-10 codes in the identification and classification of ACHD was assessed by estimating sensitivity, specificity and positive predictive value.
    RESULTS: Among the 6000 patients included, 780 (13%) patients with ACHD were manually identified from EMRs (107,092 documents). ICD-10 codes correctly categorized 629 as having ACHD (sensitivity 0.81, 95% confidence interval 0.78-0.83), with a specificity of 0.99 (95% confidence interval 0.99-1). The performance of ICD-10 codes in correctly categorizing the ACHD defect subtype depended on the defect, with sensitivity ranging from 0 (e.g. unspecified congenital malformation of tricuspid valve) to 1 (e.g. common arterial trunk), and specificity ranging from 0.99 to 1.
    CONCLUSIONS: Administrative data using ICD-10 codes is a precise tool for detecting ACHD, and may be used to establish a national cohort. Mining free-text reports in addition to coded administrative data may offset the lack of sensitivity and accuracy when describing the spectrum of congenital heart disease using ICD-10 codes.
    Keywords:  Adult congenital heart disease; Cardiopathie congénitale adulte; Code diagnostic; Diagnostic code; Dossier médical électronique; Electronic medical records; Nomenclature
    DOI:  https://doi.org/10.1016/j.acvd.2018.07.002
  5. J Pediatr Surg. 2018 Dec 13. pii: S0022-3468(18)30788-7. [Epub ahead of print]
    Bubberman JM, van Zoonen A, Bruggink JLM, van der Heide M, Berger RMF, Bos AF, Kooi EMW, Hulscher JBF.
      BACKGROUND: Necrotizing enterocolitis (NEC) predominantly occurs in preterm infants (PT-NEC). In term neonates, NEC occurs more frequently when a congenital heart disease is present (CHDNEC). Our aim was to evaluate differences and similarities in disease characteristics of PT-NEC versus CHD-NEC.METHODS: In this retrospective case-control study we identified all CHD infants who developed NEC Bell's stage ≥2 in our center from 2004 to 2014. We randomly selected (1:2 ratio) PT-NEC infants from the same period. Biochemical and clinical variables were retrieved from patient files.
    RESULTS: We found 18 CHD-NEC infants and selected 36 PT-NEC infants (gestational age 28.3 [25-35.6] weeks vs. 38.6 [31.7-40.7] weeks). Postnatal age at onset was significantly lower in CHD-NEC patients (4 [2-24] vs. 11 [4-41] days, p < 0.001). Lowest pH levels were lower (7.21 [7.01-7.47] vs. 7.27 [6.68-7.39], p = 0.02), and highest CRP levels were higher (112.5 mg/L [5.0-425.0] vs. 66.0 [5.2-189.0], p = 0.05) in PT-NEC vs. CHD-NEC. Anatomic localisation of the disease differed: the colon was significantly more often involved in CHD-NEC versus PT-NEC (86% vs. 33%, p = 0.03). Mortality caused by NEC was not different (22% vs. 11%, p = 0.47).
    CONCLUSION: While outcome of NEC in both groups is similar, the predominant NEC localisation differed between CHD-NEC and PT-NEC patients. This suggests that both variants of the disease have a different underlying pathophysiological mechanism that predisposes different intestinal regions to develop NEC.
    TYPE OF STUDY: Retrospective Case-Control Study.
    LEVEL OF EVIDENCE: Level III.
    Keywords:  CHD; Congenital heart disease; NEC; Necrotising enterocolitis; Prematurity
    DOI:  https://doi.org/10.1016/j.jpedsurg.2018.11.012
  6. J Holist Nurs. 2019 Jan 07. 898010118822069
    Pandya SP.
      This article reports on an experiment in 28 pediatric hospitals across 14 African and Asian cities. The aim was to examine the effect of a customized spiritually sensitive intervention (SSI) on mitigating depressive symptoms among hospitalized children with congenital heart diseases (CHD). Results showed that post-SSI, 1,139 treatment group children were less depressed vis-à-vis the control group. The SSI was more effective for children from African cities, boys, middle class, Christians and Buddhists, those undergoing medical care for ventricular septal defects, children who stayed in the hospital for 2 to 3 weeks and who regularly self-practiced the lessons after hospital discharge. The SSI could be effectively used for holistic pediatric nursing and health care across contexts with CHD affected children, with some culture-specific variations for greater impact.
    Keywords:  congenital heart defects; congenital heart diseases; depression; depressive symptoms; holistic pediatric nursing; hospitalized children; spiritually sensitive intervention
    DOI:  https://doi.org/10.1177/0898010118822069
  7. Int J Med Inform. 2019 Feb;pii: S1386-5056(18)30853-0. [Epub ahead of print]122 7-12
    Nkoy FL, Hofmann MG, Stone BL, Poll J, Clark L, Fassl BA, Murphy NA.
      Background Children with medical complexity (CMC) are a growing population of medically fragile children with unique healthcare needs, who have recurrent emergency department (ED) and hospital admissions due to frequent acute escalations of their chronic conditions. Mobile health (mHealth) tools have been suggested to support CMC home monitoring and prevent admissions. No mHealth tool has ever been developed for CMC and challenges exist. Objective To: 1) assess information needs for operationalizing CMC home monitoring, and 2) determine technology design functionalities needed for building a mHealth application for CMC. Methods Qualitative descriptive study conducted at a tertiary care children's hospital with a purposive sample of English-speaking caregivers of CMC. We conducted 3 focus group sessions, using semi-structured, open-ended questions. We assessed caregiver's perceptions of early symptoms that commonly precede acute escalations of their child conditions, and explored caregiver's preferences on the design functionalities of a novel mHealth tool to support home monitoring of CMC. We used content analysis to assess caregivers' experience concerning CMC symptoms, their responses, effects on caregivers, and functionalities of a home monitoring tool. Results Overall, 13 caregivers of CMC (ages 18 months to 19 years, mean = 9 years) participated. Caregivers identified key symptoms in their children that commonly presented 1-3 days prior to an ED visit or hospitalization, including low oxygen saturations, fevers, rapid heart rates, seizures, agitation, feeding intolerance, pain, and a general feeling of uneasiness about their child's condition. They believed a home monitoring system for tracking these symptoms would be beneficial, providing a way to identify early changes in their child's health that could prompt a timely and appropriate intervention. Caregivers also reported their own symptoms and stress related to caregiving activities, but opposed monitoring them. They suggested an mHealth tool for CMC to include the following functionalities: 1) symptom tracking, targeting commonly reported drivers (symptoms) of ED/hospital admissions; 2) user friendly (ease of data entry), using voice, radio buttons, and drop down menus; 3) a free-text field for reporting child's other symptoms and interventions attempted at home; 4) ability to directly access a health care provider (HCP) via text/email messaging, and to allow real-time sharing of child data to facilitate care, and 5) option to upload and post a photo or video of the child to allow a visual recall by the HCP. Conclusions Caregivers deemed a mHealth tool beneficial and offered a set of key functionalities to meet information needs for monitoring CMC's symptoms. Our future efforts will consist of creating a prototype of the mHealth tool and testing it for usability among CMC caregivers.
    Keywords:  Children with medical complexity; Home monitoring; Information; Needs; mHealth
    DOI:  https://doi.org/10.1016/j.ijmedinf.2018.11.011
  8. Nurs Outlook. 2018 Nov 22. pii: S0029-6554(18)30210-0. [Epub ahead of print]
    Weber A, Harrison TM.
      In 2011, the American Academy of Pediatrics (AAP) published a technical report on the lifelong effects of early toxic stress on human development, and included a new framework for promoting pediatric health: the Ecobiodevelopmental Framework for Early Childhood Policies and Programs. We believe that hospitalization is a specific form of toxic stress for the neonatal patient, and that toxic stress must be addressed by the nursing profession in order to substantially improve outcomes for the critically ill neonate. Approximately 4% of normal birthweight newborns and 85% of low birthweight newborns are hospitalized each year in the highly technological neonatal intensive care unit (NICU). Neonates are exposed to roughly 70 stressful procedures a day during hospitalization, which can permanently and negatively alter the infant's developing brain. Neurologic deficits can be partly attributed to the frequent, toxic, and cumulative exposure to stressors during NICU hospitalization. However, the AAP report does not provide specific action steps necessary to address toxic stress in the NICU and realize the new vision for pediatric health care outlined therein. Therefore, this paper applies the concepts and vision laid out in the AAP report to the care of the hospitalized neonate and provides action steps for true transformative change in neonatal intensive care. We review how the environment of the NICU is a significant source of toxic stress for hospitalized infants. We provide recommendations for caregiving practices that could significantly buffer the toxic stress experienced by hospitalized infants. We also identify areas of research inquiry that are needed to address gaps in nursing knowledge and to propel nursing science forward. Finally, we advocate for several public policies that are not fully addressed in the AAP technical report, but are vital to the health and development of all newborns.
    Keywords:  Buffering; Infant; Neonatal intensive care unit; Stress; Toxic stress
    DOI:  https://doi.org/10.1016/j.outlook.2018.11.002
  9. Semin Thorac Cardiovasc Surg. 2019 Jan 04. pii: S1043-0679(18)30319-8. [Epub ahead of print]
    St Louis JD, Timkovich N, Lenderman S, Jonas RA, Guleserian KJ, Tchervenkov CI, Jacobs JP, Austin EH, Plunkett MD, Myers JL, Hraska V, Sinha P, O'Brien JE, Jacobs ML, Kirklin JK.
      The World Society for Pediatric and Congenital Heart Surgery has endorsed the establishment of an international platform for the exchange of knowledge and experience for those that treat patients with a congenital heart defect. On January 1st, 2017, the release of the World Database for Pediatric and Congenital Heart Surgery opens a new era in evaluation of treatment with congenital heart defects. The contribution of data from countries with established congenital surgical databases will greatly enhance the efforts to provide the most accurate measure of overall surgical outcomes across the globe.
    DOI:  https://doi.org/10.1053/j.semtcvs.2019.01.002
  10. World J Pediatr Congenit Heart Surg. 2019 Jan 10. 2150135118799641
    Carberry T, Murthy R, Hsiao A, Petko C, Moore J, Lamberti J, Hegde S.
      Pulmonary arteriovenous malformations (AVMs) can be a complication of certain postoperative Fontan patients whose hepatic venous blood return is not distributed evenly to both lungs. A ten-year-old female, who had previously undergone staged single ventricle palliation for complex congenital heart disease, underwent a Fontan revision due to significant left-sided pulmonary AVMs and increasing arterial oxygen desaturation. The combination of four-dimensional flow cardiac magnetic resonance imaging and three-dimensional printing enabled presurgical planning for a Fontan takedown and diversion of hepatic venous flow to the azygous vein that resulted in significant clinical improvement.
    Keywords:  3D printing; 4D flow; Fontan; magnetic resonance imaging (MRI); pulmonary arteriovenous malformations
    DOI:  https://doi.org/10.1177/2150135118799641
  11. Hosp Pediatr. 2019 Jan 08. pii: hpeds.2018-0051. [Epub ahead of print]
    Masonbrink AR, Stancil S, Reid KJ, Goggin K, Hunt JA, Mermelstein SJ, Shafii T, Lehmann AG, Harhara H, Miller MK.
      BACKGROUND AND OBJECTIVES: Many hospitalized adolescents are at increased risk for pregnancy complications due to an underlying medical condition, however sexual risk assessment is not consistently performed in this setting. While adolescents and their parents are supportive of sexual health discussion in the inpatient setting, a thorough understanding of factors that influence provision of this care among pediatric hospital physicians is lacking. This formative information is needed to facilitate efforts to improve and standardize clinical care provision. Our objective is to assess the frequency and factors that influence the provision of adolescent sexual and reproductive care by pediatric hospitalists.METHODS: We performed a cross-sectional computerized survey of hospitalists at 5 pediatric hospitals who cared for ≥1 adolescent (14-21 years old) in the past year. Sexual and reproductive care practices were assessed by using a 76-item novel survey informed by the theory of planned behavior. We used descriptive statistics to summarize the data.
    RESULTS: Sixty-eight pediatric hospitalists participated (49% response rate): 78% were women and 65% were aged <40 years. Most (69%) reported treating >46 adolescents annually, including many who are at an increased risk for pregnancy complications due to teratogenic medication use or a comorbid condition. A majority felt that sexual and reproductive services are appropriate, although many endorsed barriers, including concern about follow-up after emergency contraception (63%) and time constraints (53%). Most reported insufficient knowledge regarding contraception (59%), desired contraception education (57%), and were likely to increase contraceptive provision if provided education (63%). Hospitalists rarely provided condoms or referral for an intrauterine device.
    CONCLUSIONS: Pediatric hospitalists frequently care for adolescents who are at risk for pregnancy complications and generally agree that reproductive care is appropriate in the inpatient setting. With these findings, we highlight the critical need for effective comprehensive reproductive health service interventions that are tailored to address the numerous actionable barriers identified in this study.
    DOI:  https://doi.org/10.1542/hpeds.2018-0051
  12. BMC Pediatr. 2019 Jan 08. 19(1): 12
    West SL, Banks L, Schneiderman JE, Caterini JE, Stephens S, White G, Dogra S, Wells GD.
      BACKGROUND: Physical activity (PA) is associated with a diverse range of health benefits. International guidelines suggest that children should be participating in a minimum of 60 min of moderate to vigorous intensity PA per day to achieve these benefits. However, current guidelines are intended for healthy children, and thus may not be applicable to children with a chronic disease. Specifically, the dose of PA and disease specific exercise considerations are not included in these guidelines, leaving such children with few, if any, evidence-based informed suggestions pertaining to PA. Thus, the purpose of this narrative review was to consider current literature in the area of exercise as medicine and provide practical applications for exercise in five prevalent pediatric chronic diseases: respiratory, congenital heart, metabolic, systemic inflammatory/autoimmune, and cancer.METHODS: For each disease, we present the pathophysiology of exercise intolerance, summarize the pediatric exercise intervention research, and provide PA suggestions.
    RESULTS: Overall, exercise intolerance is prevalent in pediatric chronic disease. PA is important and safe for most children with a chronic disease, however exercise prescription should involve the entire health care team to create an individualized program.
    CONCLUSIONS: Future research, including a systematic review to create evidence-based guidelines, is needed to better understand the safety and efficacy of exercise among children with chronic disease.
    Keywords:  Children; Chronic disease; Exercise; Medicine; Pediatric; Physical activity
    DOI:  https://doi.org/10.1186/s12887-018-1377-3