bims-hylehe Biomed news
on Hypoplastic left heart syndrome
Issue of 2018‒12‒16
six papers selected by
Richard James
University of Pennsylvania


  1. Semin Pediatr Neurol. 2018 Dec;pii: S1071-9091(18)30034-2. [Epub ahead of print]28 38-47
    Peyvandi S, Donofrio MT.
      This review aims to describe how the complex events of cardiovascular transition may affect the brain of infants with congenital heart disease (CHD). In particular, we describe the vulnerabilities of the neonatal brain in the transitional period during and immediately after delivery and propose management strategies that can potentially influence neurodevelopmental outcomes in this patient population. Delayed brain development has been identified in the third trimester fetus with certain forms of CHD. Neonates with critical CHD are at risk for brain injury even before their neonatal operation. The transitional period from fetal to neonatal life is marked with hemodynamic changes that can affect cerebral blood flow and oxygenation and may exacerbate injury and influence outcome. We propose specific perinatal management strategies tailored to maternal-fetal pairs aimed at optimizing hemodynamic stability, especially cerebral blood flow and oxygenation, to maximize the neurodevelopmental potential of infants with CHD.
    DOI:  https://doi.org/10.1016/j.spen.2018.05.005
  2. Am J Physiol Heart Circ Physiol. 2018 Dec 01. 315(6): H1779-H1788
    Files MD, Arya B.
      The Fontan procedure, which creates a total cavopulmonary anastomosis and represents the final stage of palliation for hypoplastic left heart syndrome, generates a unique circulation relying on a functionally single right ventricle (RV). The RV pumps blood in series around the systemic and pulmonary circulation, which requires adaptations to the abnormal volume and pressure loads. Here, we provide a complete review of RV adaptations as the RV assumes the role of the systemic ventricle, the progression of RV dysfunction to a distinct pattern of heart failure unique to this disease process, and the assessment and management strategies used to protect and rehabilitate the failing RV of Fontan circulation.
    Keywords:  Fontan; congenital heart disease; diastolic dysfunction; hypoplastic left heart syndrome
    DOI:  https://doi.org/10.1152/ajpheart.00336.2018
  3. Pediatr Cardiol. 2018 Dec 10.
    Lawley CM, Winlaw DS, Sholler GF, Martin A, Badawi N, Walker K, Nassar N, Lain SJ.
      The purpose of the study was to evaluate school-age developmental and educational outcomes for children with and without a cardiac procedure in the first year of life to improve understanding of longer-term neurodevelopmental outcomes in children who have had a cardiac procedure for congenital heart disease, the most common serious congenital anomaly. A population-based cohort study using record linkage of state-wide data was undertaken, evaluating children born in New South Wales, Australia, 2001-2007. Those with and without a cardiac procedure in the first year of life with a linked developmental (Australian version Early Development Instrument testing result, age 4-6 years) and/or educational outcome (Australian National Assessment Program result, age 7-9 years) were included. Perinatal, perioperative and sociodemographic factors were examined using multivariable logistic regression models. Of 468,329 eligible children, 768 had a cardiac procedure in the first year of life and 582 were included. For those with a cardiac procedure and developmental outcome (n = 260), 13.1% were classified as having 'special needs' compared to 4.4% without a cardiac procedure. Of those with an educational outcome, after adjusting for perinatal, perioperative and demographic variables, children with a cardiac procedure (n = 396) were twice as likely to score below National Minimum Standard in school literacy and numeracy tests compared to their peers. Significant predictors included low birthweight, parent not completing school and having > 4 re-hospitalisations in their first six years. The developmental and educational trajectory of children who have had a cardiac procedure in their first year remains altered into primary school years. While perioperative factors did not impact outcomes, ongoing health and sociodemographic factors were important in identifying those children at greatest risk.
    Keywords:  Cardiac surgery; Congenital heart disease; Developmental disabilities; Paediatrics; School-age population
    DOI:  https://doi.org/10.1007/s00246-018-2029-y
  4. Semin Pediatr Neurol. 2018 Dec;pii: S1071-9091(18)30036-6. [Epub ahead of print]28 60-65
    Lynch JM, Gaynor JW, Licht DJ.
      Infants born with critical congenital heart disease are at risk for neurodevelopmental morbidities later in life. In-utero differences in fetal circulation lead to vulnerabilities which lead to an increased incidence of stroke, white matter injury, and brain immaturity. Recent work has shown these infants may be most vulnerable to brain injury during the early neonatal period when they are awaiting their cardiac surgeries. Novel imaging and monitoring modalities are being employed to investigate this crucial time period and elucidate the precise timing and cause of brain injury in this population.
    DOI:  https://doi.org/10.1016/j.spen.2018.05.007
  5. J Pediatr. 2018 Dec 06. pii: S0022-3476(18)31585-3. [Epub ahead of print]
    Easson K, Dahan-Oliel N, Rohlicek C, Sahakian S, Brossard-Racine M, Mazer B, Riley P, Maltais DB, Nadeau L, Hatzigeorgiou S, Schmitz N, Majnemer A.
      OBJECTIVE: To compare cognitive, motor, behavioral, and functional outcomes of adolescents born with a congenital heart defect (CHD) and adolescents born preterm.STUDY DESIGN: Adolescents (11-19 years old) born with a CHD requiring open-heart surgery during infancy (n = 80) or born preterm ≤29 weeks of gestational age (n = 128) between 1991 and 1999 underwent a cross-sectional evaluation of cognitive (Leiter International Performance Scale-Revised), motor (Movement Assessment Battery for Children-II), behavioral (Strengths and Difficulties Questionnaire), and functional (Vineland Adaptive Behavior Scale-II) outcomes. Independent samples t tests and Pearson χ2 or Fisher exact tests were used to compare mean scores and proportions of impairment, respectively, between groups.
    RESULTS: Adolescents born with a CHD and adolescents born preterm had similar cognitive, motor, behavioral, and functional outcomes. Cognitive deficits were detected in 14.3% of adolescents born with a CHD and 11.8% of adolescents born preterm. Motor difficulties were detected in 43.5% of adolescents born with a CHD and 50% of adolescents born preterm. Behavioral problems were found in 23.7% of adolescents in the CHD group and 22.9% in the preterm group. Functional limitations were detected in 12% of adolescents born with a CHD and 7.3% of adolescents born preterm.
    CONCLUSIONS: Adolescents born with a CHD or born preterm have similar profiles of developmental deficits. These findings highlight the importance of providing long-term surveillance to both populations and guide the provision of appropriate educational and rehabilitation services to better ameliorate long-term developmental difficulties.
    Keywords:  behavior; cognition; congenital heart defects; functional limitations; motor skills; premature
    DOI:  https://doi.org/10.1016/j.jpeds.2018.11.002
  6. Pediatr Cardiol. 2018 Dec 11.
    Pyngottu A, Werner H, Lehmann P, Balmer C.
      The absence of cardiac symptoms, the improved exercise tolerance and the increased life expectancy are the advantages of a successful cardiac device therapy. Nevertheless, the fact of a lifelong device dependency, the inherent possibility of device malfunction and the progression of the underlying heart disease may impact the health-related quality of life (HRQoL) and the psychological adjustment of these individuals. To date, an overview of findings on these topics is lacking. The objective of this study was to evaluate and summarize the current evidence on generic and disease-specific HRQoL and psychological adjustment in paediatric patients with a pacemaker (PM) or an implantable cardioverter defibrillator (ICD). A systematic literature search was conducted using relevant databases and predefined keywords. Study selection was conducted based on predefined inclusion criteria. Fourteen studies were selected which were of varying methodological quality. Overall, the studies indicated that PM/ICD patients themselves and their parents scored lower than healthy controls on generic HRQoL scales and reported similar disease-related HRQoL as patients with severe forms of congenital heart disease. Regarding psychological adjustment outcome, PM recipients and norms were not significantly different with respect to anxiety and depression symptoms, while ICD patients showed more signs of anxiety than depression. HRQoL and psychological health should be considered when setting therapy goals and evaluating medical treatment success. This approach allows an early intervention in patients at risk of maladaptation, which is especially important in the young patient undergoing crucial developmental stages.
    Keywords:  Adaptation; Adjustment; Cardiac rhythm device; Chronic disease; Outcome
    DOI:  https://doi.org/10.1007/s00246-018-2038-x