bims-hylehe Biomed News
on Hypoplastic left heart syndrome
Issue of 2020‒05‒17
four papers selected by
Richard James
University of Pennsylvania


  1. Eur Child Adolesc Psychiatry. 2020 May 11.
    Wang CC, Weng WC, Chang LY, Chang HY, Wu MH, Wang JK, Lu CW, Lin MT, Chen CA, Chiu SN.
      The objective of this study is to assess the prevalence and risk factors for attention-deficit hyperactivity disorder (ADHD) in a large cohort of patients with congenital heart disease (CHD). Patients (n = 695) with CHD who were aged 6-15 years and visited the outpatient clinics in our hospital from June 2015 to May 2017 were enrolled. Their medical records were collected, and the Chinese version of the Swanson, Nolan, and Pelham rating scale (SNAP-IVc) and a questionnaire about neuropsychiatric care-seeking behavior were completed by parents and counselors. Of the 695 patients, the overall prevalence of ADHD was 12.4%, including 3.2% for the combined subtype, 6.8% for the inattentive-predominant subtype, and 2.4% for the hyperactivity/impulsive-predominant subtype. Only the inattention-predominant subtype was significantly more prevalent than in the general population. The prevalence of the inattention-predominant subtype was highest in the patients with cyanotic CHD, high severity index, and in those who had received surgery or cardiopulmonary bypass. Multivariate regression analysis indicated that the risk factors for inattention-related symptoms included postoperative seizure and previous cardiopulmonary bypass (odds ratio: 3.22 and 3.82; P = 0.027 and < 0.001, respectively). Only 58.7% of the patients with probable ADHD ever sought neuropsychiatric care, and only 27% regularly attended neuropsychiatric clinics. The inattention-predominant subtype of ADHD was more prevalent in our CHD patients, especially in those with cyanotic CHD, higher disease severity index, and in those who had undergone a surgical intervention. The percentage of patients receiving regular neuropsychiatric clinic follow-up was low.
    Keywords:  Attention-deficit hyperactivity disorder; Congenital heart disease; Cyanotic; SNAP-IV; Surgery
    DOI:  https://doi.org/10.1007/s00787-020-01547-y
  2. BMJ Paediatr Open. 2020 ;4(1): e000587
    Hoskote A, Wray J, Banks V, Brown K, Lakhanpaul M.
      Introduction: Children with congenital heart disease have complex medical and neurodevelopmental needs. We aimed to develop a multi-professional consensus-based referral pathway applicable to action the results of the brief developmental assessment (BDA), a validated early recognition tool, that categorises the neurodevelopmental status as green (appropriate for age), amber (equivocal) or red (delayed) in children aged between 4 months and 5 years.Methods: A Delphi consensus survey detailing two scenarios-a child categorised as delayed (red) and another as equivocal (amber) on administration of the BDA at the time of discharge from the tertiary centre-was sent to 80 expert professionals from primary, secondary and tertiary care seeking agreement on next steps and referral pathways. An iterative process was proposed with a pre-defined rule of 75% for consensus.
    Results: The survey was completed by 77 Delphi panel experts in Round 1, 73 in Round 2 and 70 in Round 3. Consensus was achieved (1) for the child with amber or red BDA, the child should be under the care of a paediatrician with expertise in cardiology (PEC) (or general paediatrician if no PEC) based at their local hospital, (2) for the child with red BDA, the PEC should initiate referral to community services at first assessment, (3) for child with amber BDA, a re-assessment by the health visitor should occur within 1-2 months, with referral to community services and notification to the PEC if on-going concerns.
    Conclusions: The Delphi process enabled a consensus to be reached between health professionals on referral pathways for specialist neurodevelopmental assessment/treatment for children with heart disease, in response to amber or red BDA results. The agreed referral pathway, if implemented, could underpin a national guideline to address and intervene on the neurodevelopmental difficulties in children with heart disease.
    Keywords:  cardiology; comm child health; general paediatrics; multidisciplinary team-care; neurodevelopment
    DOI:  https://doi.org/10.1136/bmjpo-2019-000587
  3. Can J Cardiol. 2020 May 05. pii: S0828-282X(20)30440-2. [Epub ahead of print]
    Hemphill NM, Kuan MT, Harris KC.
      Children with congenital heart disease (CHD) are at-risk for both COVID-19 and secondary cardiovascular outcomes. Their increased cardiovascular risk may be mitigated through physical activity, but public health measures implemented for COVID-19 can make physical activity challenging. We objectively measured the impact of the COVID-19 pandemic on physical activity, continuously measured by FitbitTM step-counts, in children with CHD. Step-counts were markedly lower in late March and early April 2020, compared to 2019 and to early March 2020. It is vital to understand how precautions for COVID-19 will impact the health of children with CHD, especially if they persist long-term.
    DOI:  https://doi.org/10.1016/j.cjca.2020.04.038
  4. Semin Thorac Cardiovasc Surg. 2020 May 06. pii: S1043-0679(20)30053-8. [Epub ahead of print]
    Spearman AD, Gupta A, Pan AY, Gronseth EI, Thirugnanam K, Gudausky TM, Foerster SR, Ramchandran R.
      OBJECTIVE: To improve our understanding of pulmonary arteriovenous malformations in univentricular congenital heart disease, our objective was to identify the effects of hepatic vein and superior vena cava constituents on lung microvascular endothelial cells independent of blood flow.METHODS: Paired blood samples were collected from the hepatic vein and superior vena cava in children 0-10 years-old undergoing cardiac catheterization. Isolated serum was subsequently used for in vitro endothelial cell assays. Angiogenic activity was assessed using tube formation and scratch migration. Endothelial cell survival was assessed using proliferation (BrdU incorporation, cell cycle analysis) and apoptosis (caspase 3/7 activity, Annexin-V labeling). Data were analyzed using Wilcoxon signed-rank test and repeated measures analysis.
    RESULTS: Upon incubating lung microvascular endothelial cells with 10% patient serum, hepatic vein serum increases angiogenic activity (tube formation, p=0.04, n=24; migration, p<0.001, n=18), increases proliferation (BrdU, p<0.001, n=32; S-phase, p=0.04, n=13), and decreases apoptosis (caspase 3/7, p<0.001, n=32; Annexin-V, p=0.04, n=12) compared to superior vena cava serum.
    CONCLUSIONS: Hepatic vein serum regulates lung microvascular endothelial cells by increasing angiogenesis and survival in vitro. Loss of hepatic vein serum signaling in the lung microvasculature may promote maladaptive lung microvascular remodeling and pulmonary arteriovenous malformations.
    Keywords:  Congenital heart disease; Lung microvasculature; Pulmonary arteriovenous malformations; pediatric
    DOI:  https://doi.org/10.1053/j.semtcvs.2020.03.004