bims-hylehe Biomed News
on Hypoplastic Left Heart Syndrome
Issue of 2018‒06‒17
five papers selected by
Richard James
University of Pennsylvania


  1. Zhonghua Wai Ke Za Zhi. 2018 Jun 01. 56(6): 432-435
      Objective: To discuss the experience of early surgical intervention to critical and complex congenital heart diseases (CHD) in neonates. Methods: A retrospective study of clinical records of patients with congenital heart diseases underwent surgical intervention in one single pediatric cardiac center was performed. From January 2009 to December 2017, 841 critical and complex CHD neonates were identified at Department of Cardiovascular Surgery, Children's Hospital of Fudan University, of which 6.5% were premature. There were 557 male and 284 female patients. The age was (11±9) days, ranging from 0 to 28 days, M(Q(R)): 8(17) days. The weight was (3.26±0.57) kg, ranging from 1.9 to 5.0 kg, M(Q(R)): 3.3(0.7) kg. There were 189 patients associated with other malformation besides CHD. Before surgery, 13.6% (114/841) patients were on ventilation, and severe acidosis was addressed in 136 patients. All patients underwent surgical interventions, including surgical procedures and hybrid procedures. Results: Emergency surgeries were performed in 35 patients. One hundred patients had palliative procedures, other 633 patients had complete repair, while the rest 108 patients underwent hybrid procedures. The in-hospital mortality was 4.0% (34/841). The ventilation time was 1.5 to 1 130.0 hours, M(Q(R)): 94 (116) hours, with the rate of reintubation 3.3% (27/807). The ICU stay time was 1 to 195 days, M(Q(R)): 14 (15) days. Conclusion: The improvement on early screening and diagnosing, referral system, multidisciplinary cooperation and hybrid invention skills together contributed to better outcomes of critical and complex congenital heart diseases in neonates.
    Keywords:  Cardiac surgical procedures; Heart defects, congenital; Infant, newborn; Treatment outcome
    DOI:  https://doi.org/10.3760/cma.j.issn.0529-5815.2018.06.009
  2. Environ Int. 2018 Jun 07. pii: S0160-4120(18)30280-0. [Epub ahead of print]118 211-221
      BACKGROUND/OBJECTIVE: Few studies have assessed the effect of ambient heat during the fetal development period on congenital heart defects (CHDs), especially in transitional seasons. We examined and compared the associations between extreme heat and CHD phenotypes in summer and spring, assessed their geographical differences, and compared different heat indicators.METHODS: We identified 5848 CHD cases and 5742 controls (without major structural defects) from the National Birth Defects Prevention Study, a US multicenter, population-based case-control study. Extreme heat events (EHEs) were defined by using the 95th (EHE95) or 90th (EHE90) percentile of daily maximum temperature and its frequency and duration during postconceptional weeks 3-8. We used a two-stage Bayesian hierarchical model to examine both regional and study-wide associations. Exposure odds ratios (ORs) were calculated using multivariate logistic regression analyses, while controlling for potential confounding factors.
    RESULTS: Overall, we observed no significant relationships between maternal EHE exposure and CHDs in most regions during summer. However, we found that 3-11 days of EHE90 during summer and spring was significantly associated with ventricular septal defects (VSDs) study-wide (ORs ranged: 2.17-3.24). EHE95 in spring was significantly associated with conotruncal defects and VSDs in the South (ORs: 1.23-1.78). Most EHE indicators in spring were significantly associated with increased septal defects (both VSDs and atrial septal defects (ASDs)) in the Northeast.
    CONCLUSION: While generally null results were found, long duration of unseasonable heat was associated with the increased risks for VSDs and ASDs, mainly in South and Northeast of the US. Further research to confirm our findings is needed.
    Keywords:  Cardiovascular defects; Extreme heat indicators; Heat waves; Maternal environmental exposure; Pregnancy; Seasonal effects
    DOI:  https://doi.org/10.1016/j.envint.2018.04.043
  3. Zhonghua Wai Ke Za Zhi. 2018 Jun 01. 56(6): 403-406
      People have made great progress in the surgical treatment of congenital heart disease in China, but the surgical treatment of congenital heart disease in the new era also presents some new features and trends. The data of the incidence, the constituent ratio and the change of diagnosis and treatment strategy of congenital heart disease In recent years have been analyzed to understand characteristics of congenital cardiac surgery. That will be very helpful to clarify the current status of surgical treatment of congenital heart disease in our country, formulate a more reasonable development strategy of congenital cardiac surgery, and promote the development of congenital cardiac surgery in the future.
    Keywords:  Cardiac surgical procedures; Heart defects, congenital
    DOI:  https://doi.org/10.3760/cma.j.issn.0529-5815.2018.06.002
  4. J Pediatr Nurs. 2018 Jun 07. pii: S0882-5963(18)30004-6. [Epub ahead of print]
      PURPOSE: To qualitatively explore neonatal intensive care nurses' experiences with end-of-life photography as part of their bereavement support work with families.DESIGN AND METHODS: An Interpretive Phenomenological Analysis with data collected through a focus group (n = 6) and one semi-structured interview (n = 1) with neonatal nurses from a Level 3/4 NICU in a Canadian pediatric hospital.
    RESULTS: Participants' comfort with EOL photography developed over time through exposure to bereavement scenarios and positive experiences with families. Participants' experienced a feeling of pressure to balance the photography with clinical responsibilities and find the right time to introduce photography while being sensitive to family experiences. Participants experienced EOL photography as something tangible to give families and were satisfied knowing the images might play an important role in the family's healing after the NICU.
    CONCLUSIONS: All participants had come to value EOL photography as a positive and meaningful part of their work with bereaved families. Identified challenges related to balancing the practice with the unpredictable flow and demands of critical care and to developing an appreciation for and comfort with the photography as part of their healing and the families' healing.
    PRACTICAL IMPLICATIONS: Findings contribute insight into care-provider experience that can inform best practices, training, and staff support for palliative and bereavement work in neonatal and pediatric settings. The findings suggest a need to support nurses emotionally and clinically in carrying out this photography as part of their care for families.
    Keywords:  Bereavement; End-of-life; NICU; Nurse experience; Palliative care; Photography
    DOI:  https://doi.org/10.1016/j.pedn.2018.05.011