bims-hylehe Biomed News
on Hypoplastic left heart syndrome
Issue of 2020‒03‒01
four papers selected by
Richard James
University of Pennsylvania


  1. Pediatr Cardiol. 2020 Feb 27.
    Campbell MJ, Quarshie WO, Faerber J, Goldberg DJ, Mascio CE, Blinder JJ.
      This study evaluates the effectiveness of mandatory pulse oximetry screening. The objective is to evaluate whether mandatory pulse oximetry testing had decreased the late critical congenital heart disease (CCHD) diagnosis rate and reduced mortality in neonatal subjects. This was a single center, retrospective cohort study comparing the timing of diagnosis of CCHD between neonates undergoing cardiac surgery in 2009-2010, prior to mandatory pulse oximetry screening, and neonates in 2015-2016, after mandatory pulse oximetry screening was instituted. Follow-up was for 1 year. We defined CCHD as lesions requiring surgical correction within 30 days of life. Exclusions included: pacemaker insertions, vascular ring divisions, closure of patent ductus arteriosus, arterial cutdown, or extracorporeal membrane oxygenation cannulation without structural heart disease as the sole procedure, or if subjects were born at home. Infants diagnosed prior to discharge from birth hospital were defined as early postnatal; late postnatal subjects were diagnosed after birth hospital discharge. In-hospital mortality and 1-year mortality were measured. A total of 527 neonates were included; 251 (47.6%) comprised the pre-mandatory pulse oximetry screening cohort (2009-2010). Only 3.6% of the 2009-2010 cohort and 4.3% of the 2015-2016 cohort were diagnosed late (p = 0.66). One-year mortality decreased during the study period (17.2% in 2009-2010 vs 10.5% in 2015-2016, p = 0.03). There were no deaths in the late CCHD diagnosis groups. Mandatory pulse oximetry screening legislation has not changed the late postnatal diagnosis rate at our institution. Mortality for neonatal CCHD has declined, but this decline is not attributable to mandatory pulse oximetry screening.
    Keywords:  Critical congenital heart disease; Pulse oximetry; Screening
    DOI:  https://doi.org/10.1007/s00246-020-02330-1
  2. Semin Thorac Cardiovasc Surg. 2020 Feb 19. pii: S1043-0679(20)30033-2. [Epub ahead of print]
    Rochelson E, Richmond ME, LaPar DJ, Torres A, Anderson BR.
      Despite improvements in operative and perioperative care, the risk of significant morbidity and mortality for children undergoing Fontan procedures persists. Previous investigations have identified peri-Fontan characteristics that may predict early adverse events. The purpose of this study was to identify characteristics from throughout a patient's lifespan, including all perioperative stages, that might predict early Fontan failure - defined as death, Fontan takedown, or listing for cardiac transplantation before hospital discharge or within 30 postoperative days. A single-center retrospective study of all patients undergoing a Fontan procedure was performed. Patient and intervention-related characteristics were examined from birth through Fontan. Data were described using standard summary statistics. Univariable, logistic regression was used to examine associations with early Fontan failure. In total, 191 patients met inclusion criteria. The incidence of early Fontan failure was 4% (n = 8: six deaths, two Fontan takedowns). Neonatal balloon atrial septostomy was the only patient characteristic significantly associated with Fontan failure. Patients who underwent balloon septostomy had 8.5-times higher odds of Fontan failure (CI 2.6 - 28.1, p < 0.001) than those who did not. Children who require balloon septostomy as neonates remain at higher risk of Fontan takedown, listing for heart transplantation, or death in the early post-Fontan period.
    Keywords:  Balloon atrial septostomy; Congenital heart disease; Congenital heart surgery; Fontan
    DOI:  https://doi.org/10.1053/j.semtcvs.2020.02.018
  3. Pediatr Neurol. 2020 Jan 22. pii: S0887-8994(20)30026-6. [Epub ahead of print]
    Ortinau CM, Shimony JS.
      Altered brain development has been highlighted as an important contributor to adverse neurodevelopmental outcomes in children with congenital heart disease. Abnormalities begin prenatally and include micro- and macrostructural disturbances that lead to an altered trajectory of brain growth throughout gestation. Recent progress in fetal imaging has improved understanding of the neurobiological mechanisms and risk factors for impaired fetal brain development. The impact of the prenatal environment on postnatal neurological care has also gained increased focus. This review summarizes current data on the timing and pattern of altered prenatal brain development in congenital heart disease, the potential mechanisms of these abnormalities, and the association with perioperative neurological complications.
    Keywords:  Brain; Brain development; Brain injury; Congenital heart disease; Fetal; Prenatal
    DOI:  https://doi.org/10.1016/j.pediatrneurol.2020.01.002
  4. Patient Educ Couns. 2020 Feb 10. pii: S0738-3991(20)30063-X. [Epub ahead of print]
    Shaw C, Connabeer K, Drew P, Gallagher K, Aladangady N, Marlow N.
      OBJECTIVE: To investigate whether parent-initiated or doctor-initiated decisions about limiting life-sustaining treatment (LST) in neonatal care has consequences for how possible courses of action are presented.METHOD: Formal conversations (n = 27) between doctors and parents of critically ill babies from two level 3 neonatal intensive care units were audio or video recorded. Sequences of talk where decisions about limiting LST were presented were analysed using Conversation Analysis and coded using a Conversation Analytic informed coding framework. Relationships between codes were analysed using Fisher's exact test.
    RESULTS: When parents initiated the decision point, doctors subsequently tended to refer to or list available options. When doctors initiated, they tended to use 'recommendations' or 'single-option' choice (conditional) formats (p=0.017) that did not include multiple treatment options. Parent initiations overwhelmingly concerned withdrawal, as opposed to withholding of LST (p=0.030).
    CONCLUSION: Aligning parents to the trajectory of the news about their baby's poor condition may influence how the doctor subsequently presents the decision to limit LST, and thereby the extent to which parents are invited to participate in shared decision-making.
    PRACTICE IMPLICATIONS: Explicitly proposing treatment options may provide parents with opportunities to be involved in decisions for their critically ill babies, thereby fostering shared decision-making.
    Keywords:  Conversation analysis; Decision making; End-of-life; Neonates; Parents; Withdrawing; Withholding
    DOI:  https://doi.org/10.1016/j.pec.2020.02.013