bims-hylehe Biomed News
on Hypoplastic left heart syndrome
Issue of 2019‒12‒15
eight papers selected by
Richard James
University of Pennsylvania


  1. J Pediatr Nurs. 2019 Dec 05. pii: S0882-5963(19)30378-1. [Epub ahead of print]51 1-7
    Golfenshtein N, Hanlon AL, Deatrick JA, Medoff-Cooper B.
      PURPOSE: Developmental delays are among the major morbidities of children with complex congenital heart disease. Parents of infants with complex congenital heart disease experience increased parenting stress levels, which can interfere with parenting processes during infancy. The current study examined associations between infant development and parenting stress in infants with complex congenital heart disease at six and twelve months of age.DESIGN AND METHODS: A secondary analysis of data examined cross-sectional associations between infant's mental and psychomotor development and parenting stress, using general linear regression modeling (N = 75). Data were obtained from a larger prospective cohort study.
    RESULTS: Mental development was negatively associated with the Parent Domain at six months, and with the Parent Domain and Total Stress at twelve months. Psychomotor development was not significantly associated with parenting stress at six and twelve months.
    CONCLUSIONS: Parenting stress in parents of infant with complex congenital heart disease may be among the factors shaping the parent-child relationship during the first year of life, which plays an important role in infant development. A potential bidirectional relationship between parenting stress and infant development may fit a transactional model representing the phenomena.
    PRACTICE IMPLICATIONS: Family interventions aiming to reshape illness perceptions may promote parental adaptive coping and productive parenting practices in populations at risk.
    DOI:  https://doi.org/10.1016/j.pedn.2019.11.012
  2. Can J Cardiol. 2019 Dec;pii: S0828-282X(19)31161-4. [Epub ahead of print]35(12): 1640-1651
    Mackie AS, Fournier A, Swan L, Marelli AJ, Kovacs AH.
      Dramatic increases in survival to adulthood for persons born with congenital heart disease (CHD) have led rise to a corresponding need to provide age-appropriate and developmentally appropriate care across the lifespan. Health care transition is a multidimensional process that ideally begins in early adolescence in the pediatric setting and continues through young adulthood with input from both pediatric and adult CHD providers. Preparation for transition includes the fostering of adolescents' knowledge of their CHD and of self-management and self-advocacy skills needed for lifelong management of chronic disease. Transfer is the event in time when a patient's care and ownership of health records is taken over by the adult health care team; this is just one element of the broader transition process. Transfer typically occurs by age 18 throughout much of Canada. Successful transition is a shared responsibility, requiring engaged pediatric and adult providers and partnership with both young adults and their parents, all of whom may struggle with this process. An interdisciplinary approach to transition is recommended, given that health care transition is a complex process that occurs within the broader context of young adults' lives. This review summarizes existing evidence regarding transition and transfer, offers perspectives from multiple stakeholders, and proposes a transition curriculum of development of CHD education and self-management and self-advocacy skills. Specific recommendations to improve implementation of transition and transfer care within the Canadian context are provided. This review sheds light on the current capacity and challenges of adult CHD providers and proposes directions to move this field forward.
    DOI:  https://doi.org/10.1016/j.cjca.2019.08.014
  3. Front Psychol. 2019 ;10 2666
    David Vainberg L, Vardi A, Jacoby R.
      The present article is based on a qualitative study focusing on parents of children born with congenital heart defects (CHDs) and hospitalized in the children's intensive care unit post-surgery. Our aim was to explore parents' subjective experiences as primary caregivers. Ten semi-structured interviews were conducted and analyzed using interpretative phenomenological analysis according to the instructions of Smith and Osborn. Our analysis yielded eight categories which were grouped into four themes and two main superordinate themes: (1) dialectical tension between positive and negative experiences; and (2) fluctuations between the inner and the outer world. The two superordinate themes intersect such that parents report positive as well as negative experiences within both their inner and outer worlds. Based on our analysis, we found that the experience of having a child undergo surgery for a CHD can be regarded as a chaotic period characterized by uncertainty, confusion, and helplessness. It is therefore no surprise that many parents display negative psychological outcomes which extend beyond the period of hospitalization and may also affect their future parenting and coping. However, within this chaotic and stressful situation, parents had occasional supportive experiences which decreased their emotional distress and isolation and helped them throughout this difficult period. We thus conclude that the support offered to parents during the hospitalization period should be increased by trying to minimize their negative experiences and strengthen their inner coping abilities. These changes cannot be implemented without also addressing the needs of the medical staff in their role as caregivers. Therefore, we propose a holistic model of care which supports both parents as caregivers of children undergoing surgery for CHD and the medical staff involved in their care.
    Keywords:  CHD; ICU; caregiving; children; coping; family-centered care; parents; surgery
    DOI:  https://doi.org/10.3389/fpsyg.2019.02666
  4. Can J Cardiol. 2019 Dec;pii: S0828-282X(19)31343-1. [Epub ahead of print]35(12): 1652-1663
    Haberer K, Silversides CK.
      From adolescence to older age, women with congenital heart disease (CHD) face unique challenges. In this review we explore the ways in which CHD affects women's sexual and reproductive health and, in turn, how their sexual and reproductive history affects the course of their CHD. In adolescence, special attention must be paid to menstrual irregularities and concerns of developing sexuality and self-image. Discussions about sexuality and reproduction are an important part of transition planning and must be done with an awareness of the adolescent's developing understanding and maturity. Pregnancy imposes a hemodynamic load on the heart which may lead to cardiac, obstetric, and fetal/neonatal complications in women with CHD. Prepregnancy counselling must include an assessment of maternal and fetal risk according to several well developed models. Counselling should also include discussions about fertility and alternatives to pregnancy when appropriate. Recommendations for contraception must be made according to the patient's cardiac lesion. In caring for women with CHD during pregnancy, a multidisciplinary cardio-obstetrics team is recommended to optimize care. More research is needed into the long-term impact of pregnancy on the prognosis of patients with CHD. As women with CHD increasingly survive into old age, more attention will need to be directed toward the treatment of menopause and acquired heart disease in this population.
    DOI:  https://doi.org/10.1016/j.cjca.2019.10.009
  5. Semin Pediatr Neurol. 2019 Dec;pii: S1071-9091(19)30081-6. [Epub ahead of print]32 100774
    Bonifacio SL, Van Meurs K.
      Neonatal neurocritical care is an evolving subsubspecialty whose goal is to implement neuroprotective care strategies, continuous bedside monitoring of neurologic function, and therapies in order to reduce the risk of neurologic injury and improve long-term neurodevelopmental outcomes in neonates who require intensive care. The provision of neonatal neurocritical care requires a culture change across a Neonatal Intensive Care Unit (NICU) in which equal importance is placed on the neurologic care and the cardiorespiratory care of a given patient. It is a multidisciplinary framework of care in which neonatologist and pediatric neurologist come together to address the unique needs of NICU patients whose brains are still developing and are vulnerable to injury. Advances in bedside brain monitoring techniques and the use of therapeutic hypothermia for Hupoxic-Ischemic Encephalopathy have accelerated the development of NeuroNICUs across the United States and abroad. Neonatologists, neurologists, neurophysiologists, nurses, and other ancillary members of the team work together to develop guidelines for commonly encountered neurological conditions in the NICU. The use of these guidelines helps provide standardized care across a unit and can reduce morbidity and length of hospital stay.
    DOI:  https://doi.org/10.1016/j.spen.2019.08.010
  6. Pediatrics. 2019 Dec 09. pii: e20183796. [Epub ahead of print]
    McMillan JA, Land M, Tucker AE, Leslie LK.
      The increasing prevalence of behavioral and/or mental health (B/MH) problems among children, adolescents, and young adults is rapidly forcing the pediatric community to examine its professional responsibility in response to this epidemic. Stakeholders involved in pediatric workforce training were brought together in April 2018, invited by the American Board of Pediatrics and the National Academies of Sciences, Engineering, and Medicine, to consider facilitators and barriers for pediatrician training to enhance care for B/MH problems and to catalyze commitment to improvement efforts. During the interactive meeting, parents, young adult patients, and trainees, together with leaders of pediatric training programs and health care organizations, acknowledged the growing B/MH epidemic and discussed past and current efforts to improve training and care, including integrated delivery models. Attendees committed in writing to making a change within their department or organization to improve training. There also was agreement that organizations that set the standards for training and certification bear some responsibility to ensure that future pediatricians are prepared to meet the needs of children and adolescents. Reports on commitments to change 12 months after the meeting indicated that although attendees had encountered a variety of barriers, many had creatively moved forward to improve training at the program or organizational level. This article describes the context for the April 2018 meeting, themes arising from the meeting, results from the commitments to change, and 3 case studies. Taken together, they suggest we, as a pediatric community, can and must collaborate to improve training and, by extension, care.
    DOI:  https://doi.org/10.1542/peds.2018-3796
  7. World J Pediatr Congenit Heart Surg. 2020 Jan;11(1): 14-21
    Vener DF, Abbasi RK, Brown M, Greene N, Guzzetta NA, Jacobs JP, Latham G, Mossad E, Nicolson SC, Twite M, Zhang S, Wise-Faberowski L.
      Multi-institutional databases and registries have proliferated over the last decade in all specialties of medicine. They may be especially helpful in low-frequency/high-acuity fields such as pediatric and congenital heart diseases. The Society of Thoracic Surgeon's Congenital Heart Surgery Database (STSCHSD) is the largest single data set for the congenital heart disease population and includes contemporaneous data from over 120 programs in the United States (and several outside of the United States), capturing greater than 98% of the congenital cardiac surgical procedures in the United States. In 2010, the Congenital Cardiac Anesthesia Society partnered with the STSCHSD to incorporate anesthesia-related elements into the data set. Voluntary site participation in the anesthesia data has grown steadily. Currently, over 60 sites performing more than 60% of cardiac bypass procedures in the STSCHSD are submitting anesthesia data annually into the STSCHSD. Anesthesia data include perioperative medication usage, modalities for hemodynamic and neurologic monitoring, blood product, antifibrinolytic and procoagulant use, and anesthesia-related adverse events. This special article provides a descriptive summary of relevant findings to date, reflecting the wide variety in anesthesia practice patterns present among institutions and illustrates the functionality of a multisite registry in pediatric cardiac anesthesia which can be utilized both locally and nationally.
    Keywords:  adult congenital heart disease; anesthesia (includes agents; blood; care and research); coagulation/anticoagulation; database (all types); pediatric; perioperative care; pharmacology; ultrasound (all applications); ventilation
    DOI:  https://doi.org/10.1177/2150135119884911
  8. Can J Cardiol. 2019 Dec;pii: S0828-282X(19)31150-X. [Epub ahead of print]35(12): 1842-1850
    Moons P, Luyckx K, Kovacs AH, Holbein CE, Thomet C, Budts W, Enomoto J, Sluman MA, Yang HL, Jackson JL, Khairy P, Cook SC, Chidambarathanu S, Alday L, Eriksen K, Dellborg M, Berghammer M, Johansson B, Mackie AS, Menahem S, Caruana M, Veldtman G, Soufi A, Fernandes SM, White K, Callus E, Kutty S, Apers S, .
      BACKGROUND: The prevalence and effects of cigarette smoking and cannabis use in persons with congenital heart disease (CHD) are poorly understood. We (1) described the prevalence of cigarette smoking, cannabis consumption, and co-use in adults with CHD; (2) investigated intercountry differences; (3) tested the relative effects on physical functioning, mental health, and quality of life (QOL); and (4) quantified the differential effect of cigarette smoking, cannabis use, or co-use on those outcomes.METHODS: APPROACH-IS was a cross-sectional study, including 4028 adults with CHD from 15 countries. Patients completed questionnaires to measure physical functioning, mental health, and QOL. Smoking status and cannabis use were assessed by means of the Health Behaviour Scale-Congenital Heart Disease. Linear models with doubly robust estimations were computed after groups were balanced with the use of propensity weighting.
    RESULTS: Overall, 14% of men and 11% of women smoked cigarettes only; 8% of men and 4% of women consumed cannabis only; and 4% of men and 1% of women used both substances. Large intercountry variations were observed, with Switzerland having the highest prevalence for smoking cigarettes (24% of men, 19% of women) and Canada the highest for cannabis use (19% of men, 4% of women). Cigarette smoking had a small negative effect on patient-reported outcomes, and the effect of cannabis was negligible. The effect of co-use was more prominent, with a moderate negative effect on mental health.
    CONCLUSIONS: We found significant intercountry variability in cigarette and cannabis use in adults with CHD. Co-use has the most detrimental effects on patient-reported outcomes.
    DOI:  https://doi.org/10.1016/j.cjca.2019.07.635