bims-hylehe Biomed News
on Hypoplastic left heart syndrome
Issue of 2019‒05‒12
six papers selected by
Richard James
University of Pennsylvania


  1. Clin Cardiol. 2019 May 10.
    Peng J, Meng Z, Zhou S, Zhou Y, Wu Y, Wang Q, Wang J, Sun K.
      BACKGROUND: Advances have been made in identifying genetic etiologies and maternal risk factors of congenital heart defects (CHDs), while few literatures are available regarding paternal risk factors for CHDs. Thus, we aim to conduct a meta-analysis and systematic review about the non-genetic paternal risk factors for CHDs.METHODS: We searched the PubMed, MEDLINE, and Cochrane Library online databases and identified 31 studies published between 1990 and 2018 according to the inclusion criteria. Paternal risk factors were divided into subgroups, and summarized odd ratios (OR) were calculated.
    RESULTS: Paternal age between 24-29 years decreased the risk of CHDs in the offspring (OR=0.90 [0.82, 0.98]), while paternal age ≥ 35 year-old increased the risk of CHDs (35-39 year-old: OR=1.14 [1.09, 1.19], and ≥ 40 years: OR=1.27 [1.14, 1.42]). Paternal cigarette smoking increased the risk of CHDs in a dose-dependent way. Paternal wine drinking (OR=1.47 [1.05, 2.07]) and exposure to chemical agents or drugs (OR=2.15 [1.53, 3.02]) also increased the risk of CHDs. Some specific paternal occupations were also associated with increased risk for CHDs or CHD subtypes including factory workers, janitors, painters and plywood mill workers.
    CONCLUSIONS: This meta-analysis and systematic review suggested that advanced paternal age, cigarette smoking, wine drinking, exposure to chemical agents or drugs and some specific occupations were associated with an increased risk of CHDs. More measures should be taken to reduce occupational and environment exposures. At the same time, fertility at certain age and establishment of healthy life habits are strongly recommended.
    Keywords:  congenital heart defects; meta-analysis; paternal risk factors
    DOI:  https://doi.org/10.1002/clc.23194
  2. Congenit Heart Dis. 2019 May 08.
    Gerardin J, Raskind-Hood C, Rodriguez FH, Hoffman T, Kalogeropoulos A, Hogue C, Book W.
      OBJECTIVE: Transfer of congenital heart disease care from the pediatric to adult setting has been identified as a priority and is associated with better outcomes. Our objective is to determine what percentage of patients with congenital heart disease transferred to adult congenital cardiac care.DESIGN: A retrospective cohort study.
    SETTING: Referrals to a tertiary referral center for adult congenital heart disease patients from its pediatric referral base.
    PATIENTS: This resulted in 1514 patients age 16-30, seen at least once in three pediatric Georgia health care systems during 2008-2010.
    INTERVENTIONS: We analyzed for protective factors associated with age-appropriate care, including distance from referral center, age, timing of transfer, gender, severity of adult congenital heart disease, and comorbidities.
    OUTCOME MEASURES: We analyzed initial care by age among patients under pediatric care from 2008 to 2010 and if patients under pediatric care subsequently transferred to an adult congenital cardiologist in this separate pediatric and adult health system during 2008-2015.
    RESULTS: Among 1514 initial patients (39% severe complexity), 24% were beyond the recommended transfer age of 21 years. Overall, only 12.1% transferred care to the referral affiliated adult hospital. 90% of these adults that successfully transferred were seen by an adult congenital cardiologist, with an average of 33.9 months between last pediatric visit and first adult visit. Distance to referral center contributed to delayed transfer to adult care. Those with severe congenital heart disease were more likely to transfer (18.7% vs 6.2% for not severe).
    CONCLUSION: Patients with severe disease are more likely to transfer to adult congenital heart disease care than nonsevere disease. Most congenital heart disease patients do not transfer to adult congenital cardiology care with distance to referral center being a contributing factor. Both pediatric and adult care providers need to understand and address barriers in order to improve successful transfer.
    Keywords:  adult congenital heart disease; transfer of care; transition
    DOI:  https://doi.org/10.1111/chd.12780
  3. JAMA Netw Open. 2019 May 03. 2(5): e193667
    Ramage K, Grabowska K, Silversides C, Quan H, Metcalfe A.
      Importance: With the help of medical advances, more women with adult congenital heart disease (ACHD) are becoming pregnant. Adverse maternal, obstetric, and neonatal events occur more frequently in women with ACHD than in the general obstetric population. Adult congenital heart disease is heterogeneous, yet few studies have assessed whether maternal and neonatal outcomes differ across ACHD subtypes.Objective: To assess the association of ACHD and its subtypes with pregnancy, maternal, and neonatal outcomes.
    Design, Setting, and Participants: This cross-sectional study used data from the Discharge Abstract Database, which contains information on all hospitalizations in Canada (except Quebec) from fiscal years 2001-2002 through 2014-2015. Discharge Abstract Database information was linked with maternal and infant hospital records across Canada. All women who gave birth in hospitals during the study period were included in the study. Data were analyzed from December 18, 2017, to March 22, 2019.
    Exposures: Women with ACHD were identified using diagnostic and procedural codes. Subtypes of ACHD were classified using the Anatomic and Clinical Classification of Congenital Heart Defects scheme.
    Main Outcomes and Measures: Primary outcomes were defined a priori and included severe maternal morbidity (measured using the Maternal Morbidity Outcomes Indicator), neonatal morbidity and mortality (measured using the Neonatal Adverse Outcomes Indicator), ischemic placental disease, preterm birth, congenital anomalies, and small-for-gestational-age births. Absolute and relative rates of each outcome were calculated overall and by ACHD subtype. Logistic regression using generalized estimating equations assessed crude and adjusted odds ratios (aORs) for each outcome in women with ACHD compared with women without ACHD after adjustment for comorbidities, mode of delivery, and study year.
    Results: The 2114 women with ACHD included in the analysis (mean [SD] age, 29.4 [5.7] years) had significantly higher odds of maternal morbidity (aOR, 2.7; 95% CI, 2.2-3.4) and neonatal morbidity and mortality (aOR, 1.8; 95% CI, 1.6-2.1) compared with women without ACHD (n = 2 682 451). Substantial variation was observed between women with different subtypes of ACHD. For example, the aORs of preterm birth (<37 weeks) varied from 0.4 (95% CI, 0.4-0.5) for women with anomalies of atrioventricular junctions and valves to 4.7 (95% CI, 2.9-7.5) for women with complex anomalies of atrioventricular connections.
    Conclusions and Relevance: These results suggest that women with different subtypes of ACHD are not uniformly at risk for adverse maternal and neonatal outcomes. Although some women with ACHD can potentially expect healthy pregnancies, it appears that clinical care should be modified to address the heightened risks of certain ACHD subtypes.
    DOI:  https://doi.org/10.1001/jamanetworkopen.2019.3667
  4. J Pediatr Psychol. 2019 May 09. pii: jsz021. [Epub ahead of print]
    Siciliano RE, Prussien KV, Lee CA, Patel NJ, Murphy LK, Compas BE, Jordan LC.
      OBJECTIVE: Despite surgical palliation, children with hypoplastic left heart syndrome (HLHS) have compromised cardiac functioning and increased risk for cognitive deficits. We quantitatively reviewed the empirical data from this literature.METHODS: The present meta-analysis included 13 studies reporting cognitive function for children with HLHS between the ages of 2 years and 6 months and 17 years that used standardized assessments of Full Scale IQ (FSIQ), Verbal IQ (VIQ), and Performance IQ (PIQ). Differences in cognitive function were assessed relative to normative data, and we examined sample mean age and publication year as moderators.
    RESULTS: Large effects were found for FSIQ (g = -.87, 95% CI [-1.10, -.65], M = 86.88) and PIQ (g = -.89, 95% CI [-1.11, -.68], M = 86.56), and a medium effect was found for VIQ (g = -.61, 95% CI [-.84, -.38], M = 90.82). All models demonstrated significant heterogeneity. Meta-regression analyses of effect size via Hedges' g on child age revealed a significant effect on FSIQ (coefficient = -.07, 95% CI [-.12, -.01], p < .01, R2 = .40) indicating a loss of 1.1 FSIQ points across studies with each increased year of mean sample age.
    CONCLUSIONS: Deficits in FSIQ may reflect chronic brain injury or failure to make expected gains as children age. This review highlights the importance of early intervention in this population, and the need for longitudinal studies analyzing more specific domains of cognitive function and potential moderators.
    Keywords:  HLHS; age; cognitive deficit; congenital heart disease; meta-analysis
    DOI:  https://doi.org/10.1093/jpepsy/jsz021
  5. Congenit Heart Dis. 2019 May 09.
    Awh K, Venuti MA, Gleason LP, Rogers R, Denduluri S, Kim YY.
      OBJECTIVE: To determine the prevalence and predictors of nonattendance in an ACHD outpatient clinic, and to examine the relationship between nonattendance and emergency department (ED) visits, hospitalizations, and death.METHODS: Patients ≥ 18 years who had scheduled appointments at an ACHD outpatient clinic between August 1, 2014 and December 31, 2014 were included. The primary outcome of interest was nonattendance of the first scheduled appointment of the study period, defined as "no-show" or "same-day cancellation." Secondary outcomes of interest were ED visits, hospitalizations, and death until December 2017.
    RESULTS: Of 527 scheduled visits, 55 (10.4%) were nonattended. Demographic and socioeconomic characteristics such as race, income, and insurance type were associated with non-attendance (all P values < .05), whereas age, gender, and disease complexity were not. On multivariable analysis, predictors of nonattendance were black race (adjusted odds ratio [AOR] 4.95; P < .001), other race (AOR 3.54; P = .003), and history of no-show in the past (AOR 4.95; P < .001). Compared to patients who attended clinic, patients with a nonattended visit had a threefold increased odds of multiple ED visits and a significantly lower rate of ED-free survival over time. There were no significant differences in hospitalizations or death by attendance.
    CONCLUSIONS: ACHD clinic nonattendance is associated with race and prior history of no-show, and may serve as a marker of higher ED utilization for patients with ACHD.
    Keywords:  adult congenital heart disease; emergency department; outcome
    DOI:  https://doi.org/10.1111/chd.12784
  6. Congenit Heart Dis. 2019 May 08.
    Werner O, El Louali F, Fouilloux V, Amedro P, Ovaert C.
      OBJECTIVE: Medical information provided to parents of a child with a congenital heart disease can induce major stress. Visual analog scales have been validated to assess anxiety in the adult population. The aim of this study was to analyze parental anxiety using a visual analog scale and to explore the influencing factors.DESIGN: This prospective cross-sectional study.
    SETTING: Tertiary care regional referral center for congenital heart disease of Marseille-La Timone university hospital.
    PATIENTS: Parents of children with a congenital heart disease, as defined by the ACC-CHD classification, referred for cardiac surgery or interventional cardiac catheterization, were offered to participate.
    INTERVENTION AND OUTCOME MEASURE: The parental level of anxiety was assessed using a visual analog scale (0-10) before intervention and after complete information given by the cardiologist, the surgeon or the anesthetists.
    RESULTS: Seventy-three children [7 days-13 years], represented by 49 fathers and 71 mothers, were included in the study. A total of 42 children required cardiac surgery and 31 children underwent interventional cardiac catheterization. The mean score of maternal anxiety was significantly higher than the paternal anxiety (8.2 vs 6.3, P < .01). A high level of maternal anxiety (visual analog scale > 8) was associated with paternal anxiety (P = .02), the child's comorbidity (P = .03), the distance between home and referral center (P = .04), and the level of risk adjustment for congenital heart surgery (P = .01). In multivariate analysis, maternal anxiety was associated with paternal anxiety (OR = 4.9; 95% confidence interval [1.1-19.2]), and the level of risk adjustment for congenital heart surgery (OR = 11.4; 95% confidence interval [1.2-116.2]). No significant association was found between parental anxiety and prenatal diagnosis.
    CONCLUSION: This study highlighted several factors associated with the parental anxiety. Identifying the parents at risk of high stress can be useful to set up psychological support during hospitalization.
    Keywords:  anxiety; congenital heart disease; parents; visual analog scale
    DOI:  https://doi.org/10.1111/chd.12777