bims-hylehe Biomed News
on Hypoplastic left heart syndrome
Issue of 2019‒05‒19
five papers selected by
Richard James
University of Pennsylvania


  1. Birth Defects Res. 2019 May 17.
    Harrison TM.
      Worldwide, more than 400,000 infants are born each year with complex congenital heart disease (CCHD) requiring surgical intervention within the first months of life. Although improvements in perioperative care have resulted in increased rates of survival, more than half of infants with CCHD have neurodevelopmental impairments affecting subsequent educational achievements, job opportunities, and mental health. Brain maturity and impaired outcomes in infants with CCHD are similar to those of prematurely born infants. Developmentally supportive care, including foundational application of kangaroo care (KC), improves neurodevelopment in premature infants. Provision of developmentally supportive care with KC during the early hospitalization of infants with CCHD has the potential to similarly improve neurodevelopment. The purposes of the article are to describe common congenital heart defects, describe developmentally supportive care with an emphasis on KC, and to offer specific recommendations for KC and research in infants with CCHD.
    Keywords:  congenital heart disease; developmental care; infant care; kangaroo care; skin-to-skin contact
    DOI:  https://doi.org/10.1002/bdr2.1517
  2. Int J Cardiol. 2019 Apr 26. pii: S0167-5273(19)30378-X. [Epub ahead of print]
    Thomet C, Moons P, Budts W, De Backer J, Chessa M, Diller G, Eicken A, Gabriel H, Gallego P, Giamberti A, Roos-Hesselink J, Swan L, Webb G, Schwerzmann M, .
      BACKGROUND: Clinical guidelines emphasise the need for specialised adult congenital heart disease (ACHD) programmes. In 2014, the working group on Grown-up Congenital Heart Disease of the European Society of Cardiology (ESC) published recommendations on the organisation of specialised care for ACHD. To appraise the extent to which these recommendations were being implemented throughout Europe, we assessed the number of patients in active follow-up and available staff resources in European ACHD programmes.METHODS: We conducted a descriptive, cross-sectional, paper-based survey of specialised ACHD centres in Europe in late 2017 concerning their centre status in 2016. Data from 96 ACHD centres were analysed. We categorised ACHD programmes into seven different centre types based on their staff resources and composition of interdisciplinary teams.
    RESULTS: Only four centres fulfilled all medical and non-medical staffing requirements of the ESC recommendations. Although 60% of the centres offered all forms of medical care, they had incomplete non-medical resources (i.e., specialised nurses, social workers, or psychologists). The participating centres had 226,506 ACHD patients in active follow-up, with a median of 1500 patients per centre (IQR: 800-3400). Six per cent of the patients were followed up in a centre that lacked a CHD surgeon or congenital interventional cardiologist.
    CONCLUSIONS: A minority of European ACHD centres have the full recommended staff resources available. This suggests that as of 2016 either ACHD care in Europe was still not optimally organised, or that the latest ESC recommendations were not fully implemented in clinical practice.
    Keywords:  Adult; Cardiac care facilities; Congenital; Europe; Heart defects; Organisation of care
    DOI:  https://doi.org/10.1016/j.ijcard.2019.04.077
  3. Mil Med. 2019 May 15. pii: usz116. [Epub ahead of print]
    Robinson DL, Craig MS, Wells RS, Liesemer KN, Studer MA.
      INTRODUCTION: The purpose of this study was to assess the evolution of newborn pulse oximetry screening (+POx) among Army, Air Force, and Naval military hospitals (MH), including prevalence, protocol use, quality assurance processes, access to echocardiography, and use of telemedicine. This is a follow-up from a prior study published in 2011.MATERIALS AND METHODS: An Internet-based questionnaire was forwarded to the chief pediatrician at MH worldwide which support newborn deliveries. Descriptive data were reported using percentages. Grouped responses, as applicable, were further compared using the chi-square test. A p-value < 0.05 was considered statistically significant.
    RESULTS: Seventy-eight percent (36/46) of MH supporting deliveries worldwide responded to the survey (17 Army hospitals, 11 Navy Hospitals, 8 Air Force hospitals). All responding hospitals utilize +POx, of which 94% endorsed protocol compliance with the American Academy of Pediatrics guidelines. Nine (25%) hospitals were located outside of the United States. Delivery volumes (infants per month) range between 1-49 (36%), 50-99 (28%), 100-199 (19%), and 200-300 (17%). Eleven hospitals reported regular review of +POx data, with most reviewing them monthly. Four MH share findings with state institutions. Ten hospitals either have a staff pediatric cardiologist or use tele-echocardiography for on-site evaluations. Ten hospitals are located greater than 60 miles from the nearest center with echocardiography capabilities. Of the five hospitals using tele-echocardiography, four confirmed critical congenital heart disease (CCHD) using this practice, and all five reported averting transfer of an infant using this technology. Of the 22 hospitals lacking the ability to obtain on-site echocardiography, 12 (55%) are interested in implementing a tele-echocardiography protocol.
    CONCLUSIONS: All responding MH use +POx, representing significant increase from the 30% of MH reporting use of +POx seven years ago. The majority of MH follow AAP +POx guidelines, and though most have providers review results prior to discharge, only one-third report periodic chart review for quality assurance. Most MH transfer infants with positive +POx results for evaluation due to a lack of on-site echocardiography. Tele-echocardiography was reported as a potential solution to diagnose or rule out CCHD. Over half of remaining hospitals without cardiologists are interested in using this technology to evaluate stable infants with positive CCHD screening.
    Keywords:  CCHD/critical congenital heart disease; Pulse oximetry screening; military; pediatrics
    DOI:  https://doi.org/10.1093/milmed/usz116
  4. J Palliat Med. 2019 May 15.
    Jersak T, Gustin J, Humphrey L.
      There are more adults than children living with congenital heart disease (CHD) due to improvements in surgical and medical CHD management today. In 2011, though, fewer than 30% of adult CHD patients were following up with specialized providers. An ineffective transition from pediatric to adult-focused medical care can result in lapses in CHD medical care, patient noncompliance, and increased risk of late complications. Early involvement of a palliative care team offers development of autonomy, identification of potential barriers to care, and support for patient and family that may improve transition success and quality of life in CHD patients.
    Keywords:  adolescent; communication; congenital heart disease; palliative care; psychosocial issues; transition
    DOI:  https://doi.org/10.1089/jpm.2018.0467