bims-hylehe Biomed News
on Hypoplastic left heart syndrome
Issue of 2019‒12‒08
six papers selected by
Richard James
University of Pennsylvania


  1. J Am Coll Cardiol. 2019 Dec 10. pii: S0735-1097(19)37989-6. [Epub ahead of print]74(23): 2908-2918
    Nguyen VP, Dolgner SJ, Dardas TF, Verrier ED, McMullan DM, Krieger EV.
      BACKGROUND: The number of adult congenital heart disease (CHD) patients undergoing heart transplantation is increasing rapidly. CHD patients have higher surgical risk at transplantation. High-volume adult CHD transplant centers may have better transplant outcomes.OBJECTIVES: This study aimed to evaluate the effect of center CHD transplant volume and expertise on transplant outcomes in CHD patients.
    METHODS: The authors studied heart transplantations in CHD patients age ≥18 years using the United Network of Organ Sharing (UNOS) database for the primary outcomes of waitlist mortality and post-transplant outcomes at 30 days and 1 year. Transplant centers were assessed by status as the highest CHD transplant volume center in a UNOS region versus all others, presence of Adult Congenital Heart Association accreditation, and adult versus pediatric hospital designation.
    RESULTS: Between January of 2000 and June of 2018, 1,746 adult CHD patients were listed for transplant; 1,006 (57.6%) of these underwent heart transplantation. After adjusting for age, sex, listing status, and inotrope requirement, waitlist mortality risk was lower at Adult Congenital Heart Association accredited centers (hazard ratio: 0.730; p = 0.020). Post-transplant 30-day mortality was lower at the highest volume CHD transplant center in each UNOS region (hazard ratio: 0.706; p = 0.014).
    CONCLUSIONS: Designated expertise in CHD care is associated with improved waitlist outcomes for CHD patients listed for transplantation. Post-transplant survival was improved at the highest volume regional center. These findings suggest a possible advantage of regionalization of CHD transplantation.
    Keywords:  Adult Congenital Heart Association; United Network of Organ Sharing; adult congenital heart disease; center; heart transplantation
    DOI:  https://doi.org/10.1016/j.jacc.2019.09.062
  2. J Spec Pediatr Nurs. 2019 Dec 02. e12283
    Pridham KF, Harrison TM, Brown R, Mussatto K.
      PURPOSE: Describe collaborative caregiving of the parents of an infant with congenital heart disease (CHD) with partner, health-care clinicians, and substitute caregivers (SC).DESIGN AND METHODS: In a national online survey, 53 mothers and 12 fathers described collaborative caregiving for infants after CHD intervention with respect to facets of Bowlby's concept of the internal working model of caregiving. Parents identified concern for 24 everyday common problems or goals (i.e., issues; 10 for partner, 9 for clinician, 5 for SC), rated issue importance and difficulty, indicated an intention for seeking and using help (help use), and identified help source.
    RESULTS: All survey items were a concern to at least 11 parents. Most prominently, all five of the SC items were identified by at least 44% of the parents as a concern. Everyday, common problems and goals were generally reported to be more important than difficult. Two items identified as partner issues, "Talking about life change" and "Handling stress," were a concern for about 50% of parents and identified by at least 74% as very important and 72% as moderately to greatly difficult. Help use was highest for SC items followed by items related to the partner. The partner was most frequently identified as a help source.
    PRACTICE IMPLICATIONS: Nurses are in a position to assess and support parent collaboration within partner, clinician, and SC relationships for everyday problems and goals. The survey questions, with further development and testing, could be used for clinician assessment and tailoring of intervention to support parents' collaborative caregiving for infants with CHD.
    Keywords:  caregiving; collaboration; congenital heart disease; health-care clinicians; help sources; help-seeking; infant; nurses; nursing; parents; substitute caregivers
    DOI:  https://doi.org/10.1111/jspn.12283
  3. J Am Coll Cardiol. 2019 Dec 10. pii: S0735-1097(19)38168-9. [Epub ahead of print]74(23): 2919-2920
    Backer CL, Karamlou T, Welke KF.
      
    Keywords:  Adult Congenital Heart Association; United Network of Organ Sharing; adult congenital heart disease; heart transplantation; regionalization
    DOI:  https://doi.org/10.1016/j.jacc.2019.10.026
  4. Heart. 2019 Dec 05. pii: heartjnl-2019-315680. [Epub ahead of print]
    Meyer M, Brudy L, García-Cuenllas L, Hager A, Ewert P, Oberhoffer R, Müller J.
      Home-based exercise training is a promising alternative to conventional supervised training for patients with congenital heart disease (CHD). Even though the beneficial effect of exercise interventions is well established in patients with CHD, knowledge concerning variety and utility of existing programmes is still lacking. Therefore, the aim of this review is to give an overview about existing home-based exercise interventions in patients with CHD. A systematic search was performed in PubMed, Cochrane, Scopus and PEDro (2008-2018) for relevant clinical trials that provided any kind of home-based exercise with patients with CHD. All articles were identified and assessed by two independent reviewers. Seven articles with 346 paediatric CHD (18 months to 16 years) and five articles with 200 adults with CHD (21-41 years) were included. Most studies performed a supervised home-based exercise intervention with children and adolescents exercising at least three times per week with duration of 45 min for 12 weeks. Reported outcome measurements were health-related quality of life and physical activity, but mostly exercise capacity measured as peak oxygen uptake that improved in four studies (1.2%, 7%, 7.7%, 15%; p<0.05), walking distance in two (3.5%, 19.5%, p<0.05,) or walking time (2 min, p=0.003) in one. The dropout rates were high (15%), and compliance to the training programme was not reported in the majority of the studies (58%). Home-based exercise interventions are safe, feasible and a useful alternative to supervised cardiac rehabilitation for all age groups of patients with CHD. Nevertheless, training compliance represents a major challenge.
    Keywords:  cardiac rehabilitation; complex congenital heart disease; congenital heart disease; eHealth/telemedicine/mobile health; valvular heart disease
    DOI:  https://doi.org/10.1136/heartjnl-2019-315680
  5. Acad Pediatr. 2019 Dec 03. pii: S1876-2859(19)30502-9. [Epub ahead of print]
    Seltzer R, Raisanen JC, Silva TD, Donohue PK, Williams EP, Shepard J, Boss RD.
      OBJECTIVE: To explore how medical decision-making for children with medical complexity (CMC) occurs in the context of foster care (FC).METHODS: Together with a medical FC agency, we identified 15 CMC in medical FC and recruited eligible care team members (biological and foster parents, medical FC nurses, caseworkers in medical FC/ child welfare, and pediatricians) for each child. Semi-structured interviews were conducted, and conventional content analysis was applied to transcripts.
    RESULTS: 58 interviews were completed with 2-5 care team members/child. Serious decision-making related to surgeries and medical technology was common. Themes regarding medical decision-making for CMC in FC emerged: 1) Protocol: decision-making authority is dictated by court order and seriousness of decision, 2) Process: decision-making is dispersed among many team members, 3) Representing the child's interests: the majority of respondents stated that the foster parent represents the child's best interests, while the child welfare agency should have legal decision-making authority, and 4) Perceived barriers: serious medical decision-making authority is often given to individuals who spend little time with the child.
    CONCLUSION: Medical decisions for CMC can have uncertain risk/benefit ratios. For CMC in FC, many individuals have roles in these nuanced decisions; those with ultimate decision-making authority may have minimal interaction with the child. Pediatricians can assist by clarifying who has legal decision-making authority, facilitating team communication to promote truly informed consent, and serving as a resource to decision-makers. Further research should explore how to adapt the traditional model of shared decision-making to meet the needs of this population.
    Keywords:  children with medical complexity; foster care; medical decision-making; shared decision-making
    DOI:  https://doi.org/10.1016/j.acap.2019.11.018
  6. Acta Paediatr. 2019 Dec 04.
    López Maestro M, De la Cruz J, Perapoch-Lopez J, Gimeno-Navarro A, Vázquez-Román S, Alonso-Díaz C, Muñoz-Amat B, Morales-Betancourt C, Soriano-Ramos M, Pallás-Alonso C.
      AIM: To assess, at national level, the implementation of eight principles for infant- and family-centred developmental care (IFCDC) in neonatal units. A European expert group established eight 'Principles of care' in 2018 that define neurodevelopmental and family-centred care.METHODS: The implementation of each principle was assessed by a survey sent to level-III Spanish units. A principle was considered to be implemented if all answers to the principle-associated questions were positive.
    RESULTS: The response rate was 84.5% (65/77). No unit had implemented eight principles. Principle 1 (free parental access) was implemented in 21.5% of the centres; Principle 2 (psychological support) 40%; Principle 3 (pain-management) 7,7%; Principle 4 (environmental influences) 29%; Principle 5 (postural support) 84.6%; Principle 6 (kangaroo-care) 67.7%; Principle 7 (breastfeeding) 23% and Principle 8 (sleep protection) in 46%. In units attending ≥50 Very Low Birth Weight (VLBW) infants four or more principles had been implemented in 31% vs. 13% <50 VLBW neonates (odds ratio 3.0 CI95% 0.9 -10.1, p0.075).
    CONCLUSION: The principle with the highest implementation was related to newborn body positioning. Pain management was the principle with lowest implementation. More principles for IFCDC tend to be implemented in units providing care for a higher number of VLBW infants.
    Keywords:  Developmental Care; Family Care; Pain,Very Low Birth Weight; Visiting Policies NICU
    DOI:  https://doi.org/10.1111/apa.15121