bims-hylehe Biomed News
on Hypoplastic left heart syndrome
Issue of 2019‒08‒25
seven papers selected by
Richard James
University of Pennsylvania


  1. BMJ Open. 2019 Aug 18. 9(8): e030506
    Cloete E, Gentles TL, Dixon LA, Webster DR, Agnew JD, Davidkova S, Alsweiler JM, Rogers J, Bloomfield FH, .
      OBJECTIVES: The aim of this study was to conduct New Zealand-specific research to inform the design of a pulse oximetry screening strategy that ensures equity of access for the New Zealand maternity population. Equity is an important consideration as the test has the potential to benefit some populations and socioeconomic groups more than others.SETTING: New Zealand has an ethnically diverse population and a midwifery-led maternity service. One quaternary hospital and urban primary birthing unit (Region A), two regional hospitals (Region B) and three regional primary birthing units (Region C) from three Health Boards in New Zealand's North Island participated in a feasibility study of pulse oximetry screening. Home births in these regions were also included.
    PARTICIPANTS: There were 27 172 infants that satisfied the inclusion criteria; 16 644 (61%) were screened. The following data were collected for all well newborn infants with a gestation age ≥35 weeks: date of birth, ethnicity, type of maternity care provider, deprivation index and screening status (yes/no). The study was conducted over a 2-year period from May 2016 to April 2018.
    RESULTS: Screening rates improved over time. Infants born in Region B (adjusted OR=0.75; 95% CI 0.67 to 0.83) and C (adjusted OR=0.29; 95% CI 0.27 to 0.32) were less likely to receive screening compared with those born in Region A. There were significant associations between screening rates and deprivation, ethnicity and maternity care provider. Lack of human and material resources prohibited universal access to screening.
    CONCLUSION: A pulse oximetry screening programme that is sector-led is likely to perpetuate inequity. Screening programmes need to be designed so that resources are distributed in the way most likely to optimise health outcomes for infants born with cardiac anomalies.
    ETHICS APPROVAL: This study was approved by the Health and Disability Ethics Committees of New Zealand (15/NTA/168).
    Keywords:  congenital heart disease; health policy; neonatology; preventive medicine; public health
    DOI:  https://doi.org/10.1136/bmjopen-2019-030506
  2. Obstet Gynecol. 2019 Sep;134(3): e84-e89
      Perinatal palliative care refers to a coordinated care strategy that comprises options for obstetric and newborn care that include a focus on maximizing quality of life and comfort for newborns with a variety of conditions considered to be life-limiting in early infancy. With a dual focus on ameliorating suffering and honoring patient values, perinatal palliative care can be provided concurrently with life-prolonging treatment. The focus of this document, however, involves the provision of exclusively palliative care without intent to prolong life in the context of a life-limiting condition, otherwise known as perinatal palliative comfort care. Once a life-limiting diagnosis is suspected antenatally, the tenets of informed consent require that the pregnant patient be given information of sufficient depth and breadth to make an informed, voluntary choice for her care. Health care providers are encouraged to model effective, compassionate communication that respects patient cultural beliefs and values and to promote shared decision making with patients. Perinatal palliative comfort care is one of several options along a spectrum of care, which includes pregnancy termination (abortion) and full neonatal resuscitation and treatment, that should be presented to pregnant patients faced with pregnancies complicated by life-limiting fetal conditions. If a patient opts to pursue perinatal palliative comfort care, a multidisciplinary team should be identified with the infrastructure and support to administer this care. The perinatal palliative care team should prepare families for the possibility that there may be differences of opinion between family members before and after the delivery of the infant, and that there may be differences between parents and the neonatal care providers about appropriate postnatal therapies, especially if the postnatal diagnosis and prognosis differ substantially from antenatal predictions. Procedures for resolving such differences should be discussed with families ahead of time.
    DOI:  https://doi.org/10.1097/AOG.0000000000003425
  3. Obstet Gynecol. 2019 Sep;134(3): 660-661
      Perinatal palliative care refers to a coordinated care strategy that comprises options for obstetric and newborn care that include a focus on maximizing quality of life and comfort for newborns with a variety of conditions considered to be life-limiting in early infancy. With a dual focus on ameliorating suffering and honoring patient values, perinatal palliative care can be provided concurrently with life-prolonging treatment. The focus of this document, however, involves the provision of exclusively palliative care without intent to prolong life in the context of a life-limiting condition, otherwise known as perinatal palliative comfort care. Once a life-limiting diagnosis is suspected antenatally, the tenets of informed consent require that the pregnant patient be given information of sufficient depth and breadth to make an informed, voluntary choice for her care. Health care providers are encouraged to model effective, compassionate communication that respects patient cultural beliefs and values and to promote shared decision making with patients. Perinatal palliative comfort care is one of several options along a spectrum of care, which includes pregnancy termination (abortion) and full neonatal resuscitation and treatment, that should be presented to pregnant patients faced with pregnancies complicated by life-limiting fetal conditions. If a patient opts to pursue perinatal palliative comfort care, a multidisciplinary team should be identified with the infrastructure and support to administer this care. The perinatal palliative care team should prepare families for the possibility that there may be differences of opinion between family members before and after the delivery of the infant, and that there may be differences between parents and the neonatal care providers about appropriate postnatal therapies, especially if the postnatal diagnosis and prognosis differ substantially from antenatal predictions. Procedures for resolving such differences should be discussed with families ahead of time.
    DOI:  https://doi.org/10.1097/AOG.0000000000003426
  4. Semin Cardiothorac Vasc Anesth. 2019 Aug 20. 1089253219870635
    Mueller MF, Paul AC, Mann V, Koerner CM, Valeske K, Thul J, Mazhari N, Bauer J, Schranz D, Akintuerk H.
      Background. The absolute number of patients presenting with failure of the hemi-Fontan or Fontan circulation for cardiac transplantation (cTx) will continuously rise. We aimed to analyze the intraoperative differences in patients undergoing cTx for a failing hemi-Fontan or Fontan circulation (group A) with those undergoing cTx for cardiomyopathy and congenital heart disease (group B). Methods. Data of patients undergoing cTx during a 10-year period were compared between group A and group B patients. Results. cTx was performed in 83 patients (group A, n = 21; group B, n = 62). Surgical times including median incision-suture time (549 minutes vs 386 minutes, P < .05) and median organ ischemia time (305 minutes vs 233 minutes, P < .05) were longer in group A patients. After weaning off cardiopulmonary bypass a higher median modified Vasoactive Inotropic Score (16 vs 10, P < .05) was necessary in group A patients. During surgery more fresh frozen plasma (44 mL/kg vs 20 mL/kg, P < .05), platelet concentrates (20 mL/kg vs 14 mL/kg, P < .05), and coagulation factor concentrates were given in group A patients. Mortality during the first 90 days after cTx was higher in group A (23.8% vs 6.5%, P < .05). Conclusion. Patients undergoing cTx for a failing hemi-Fontan or Fontan circulation are challenging. They require an intensive vasoactive and inotropic support; furthermore, special attention should be paid to the management of bleeding complications. cTx for this group of patients is associated with higher 90 days mortality.
    Keywords:  cardiac anesthesia; cardiac transplantation; children; congenital heart disease; univentricular heart
    DOI:  https://doi.org/10.1177/1089253219870635
  5. Eur J Pediatr Surg. 2019 Aug 19.
    Ganji N, Li B, Lee C, Filler R, Pierro A.
      Necrotizing enterocolitis (NEC) is a devastating intestinal disease that continues to have high morbidity and mortality among preterm neonates, despite medical advancements in neonatology and neonatal care. To investigate the pathogenesis of the disease and explore novel form of treatment, a variety of experimental models of NEC have been developed and used by various investigators. These experimental models range from in vitro evaluation of intestinal epithelial cells and intestinal organoids to in vivo models of the disease in neonatal mice, rats, and piglets. Most recently, human-derived intestinal organoids have also been developed and investigated. In this review, we will briefly discuss these experimental models and the contributions that they have made to our understanding of NEC. We will also point to the ischemia/reperfusion (I/R) model of intestinal injury which has been used as an indirect model of NEC by some investigators. Advancements in laboratory research into this devastating disease have continued to expand our knowledge on the pathogenesis and prevention of NEC as well as the effectiveness of therapeutic options for management of this severe disease.
    DOI:  https://doi.org/10.1055/s-0039-1693994
  6. J Pediatr Surg. 2019 Aug 08. pii: S0022-3468(19)30504-4. [Epub ahead of print]
    Kampouroglou G, Velonaki VS, Pavlopoulou I, Drakou E, Kosmopoulos M, Kouvas N, Tsagkaris S, Fildissis G, Nikas K, Tsoumakas K.
      INTRODUCTION: Although important, parental anxiety, health literacy and need-for-information in pediatric surgery outpatient clinics have not been extensively studied. Lower educational attainments, minorities and lower socioeconomic status have been associated with limited health literacy. Parental anxiety has been related to health literacy, sex, education and information needs. The aim of this study is to investigate health literacy and need-for-information and their association to parental anxiety in consultations of pediatric surgery.MATERIALS & METHODS: We conducted an observational, cross-sectional study in the outpatient pediatric surgery clinic from December 2016 to October 2017. Health literacy, anxiety and need-for-information of parents/guardians of children waiting for pediatric surgical consultation were evaluated. Multivariate regression analysis was used to examine the impact of health literacy and need-for-information on parental/guardian anxiety considering sociodemographic and clinical characteristics of the participants.
    RESULTS: Almost half (46.1%) of the 664 parents/guardians recruited had limited or problematic health literacy and 79.8% of the sample was classified as being anxious. Parental/guardian anxiety was associated at the multiple regression analysis with parental health literacy level (β = -0.282, p < 0.001), need-for-information preoperatively (β = 0.907, p < 0.001), educational level (β = -0.716, p = 0.001), sex (β = 1.563, p < 0.001), and severity of the condition of the child (β = 0.379, p < 0.001).
    CONCLUSION: Parents/guardians experience high levels of anxiety, which is associated to health literacy and need-for-information. These factors should be considered in pediatric surgical consultations, aiming to reduce parental anxiety.
    TYPE OF STUDY: Retrospective Study.
    LEVEL OF EVIDENCE: Level II.
    Keywords:  Anxiety; Health literacy; Need-for-information; Parents; Pediatric surgery
    DOI:  https://doi.org/10.1016/j.jpedsurg.2019.07.016
  7. Geburtshilfe Frauenheilkd. 2019 Aug;79(8): 863-872
    Graupner O, Enzensberger C, Axt-Fliedner R.
      Fetal hypoplastic left heart syndrome (HLHS) is a severe congenital heart disease with a lethal prognosis without postnatal therapeutic intervention or surgery. The aim of this article is to give a brief overview of new findings in the field of prenatal diagnosis and the therapy of HLHS. As cardiac output in HLHS children depends on the right ventricle (RV), prenatal assessment of fetal RV function is of interest to predict poor functional RV status before the RV becomes the systemic ventricle. Prenatal cardiac interventions such as fetal aortic valvuloplasty and non-invasive procedures such as maternal hyperoxygenation seem to be promising treatment options but will need to be evaluated with regard to long-term outcomes. Novel approaches such as stem cell therapy or neuroprotection provide important clues about the complexity of the disease. New aspects in diagnostics and therapy of HLHS show the potential of a targeted prenatal treatment planning. This could be used to optimize parental counseling as well as pre- and postnatal management of affected children.
    Keywords:  fetal cardiac function; fetal cardiac interventions; fetal hypoplastic left heart syndrome
    DOI:  https://doi.org/10.1055/a-0828-7968