bims-hylehe Biomed News
on Hypoplastic left heart syndrome
Issue of 2019‒10‒20
three papers selected by
Richard James
University of Pennsylvania


  1. Pediatrics. 2019 Oct 18. pii: e20191010. [Epub ahead of print]
    Goldberg CS, Hu C, Brosig C, Gaynor JW, Mahle WT, Miller T, Mussatto KA, Sananes R, Uzark K, Trachtenberg F, Pizarro C, Pemberton VL, Lewis AB, Li JS, Jacobs JP, Cnota J, Atz AM, Lai WW, Bellinger D, Newburger JW, .
      OBJECTIVES: We measured behavioral, quality of life (QoL), and functional status outcomes for 6-year-old children with hypoplastic left heart syndrome enrolled in the Single Ventricle Reconstruction Trial. We sought to compare these outcomes with those in the normative population and to analyze risk factors for worse outcomes within the single-ventricle group.METHODS: Parent-response instruments included the Vineland Adaptive Behavior Scales, Second Edition (Vineland-II) (primary outcome), Behavior Assessment System for Children 2, Pediatric Quality of Life Inventory 4.0, and other measures of QoL and functional status. We compared subjects with those in the normative sample using 1-sample Wilcoxon rank tests and assessed outcome predictors using multivariable regression.
    RESULTS: Of 325 eligible patients, 250 (77%) participated. Compared with population norms, participants had lower scores on the Vineland-II motor skills domain (90 ± 17 vs 100 ± 15; P < .001), with 11% scoring >2 SDs below the normative mean. On nearly all major domains, more study subjects (3.3%-19.7%) scored outside the normal range than anticipated for the general population. Independent risk factors for lower Vineland-II scores included perioperative extracorporeal membrane oxygenation, male sex, use of regional cerebral perfusion, catheterization after stage 2 operation, visual problems, seizure history, and more complications after 2 years (R 2 = 0.32). Independent predictors of worse Behavior Assessment System for Children 2 (R 2 = 0.07-0.20) and Pediatric Quality of Life Inventory 4.0 (R 2 = 0.17-0.25) domain scores also included sociodemographic factors and measures of morbidity and/or greater course complexity.
    CONCLUSIONS: At 6 years, children with hypoplastic left heart syndrome had difficulty in areas of adaptive behavior, behavioral symptoms, QoL, and functional status. Principal risks for adverse outcomes include sociodemographic factors and measures of greater course complexity. However, models reveal less than one-third of outcome variance.
    DOI:  https://doi.org/10.1542/peds.2019-1010
  2. J Perinatol. 2019 Oct 14.
    Adams AD, Aggarwal N, Fries MH, Donofrio MT, Iqbal SN.
      OBJECTIVE: We sought to determine if fetuses with prenatally diagnosed congenital heart disease (CHD) were more likely to undergo cesarean delivery in the setting of a non-reassuring fetal heart rate tracing (NRFHT) and to determine if those fetuses were more likely to have a fetal acidosis.STUDY DESIGN: A retrospective cohort study of neonates prenatally diagnosed with CHD from August 2010 to July 2016. The control group consisted of gestational age matched controls without CHD.
    RESULTS: Each group consisted of 143 patients. The most common reason for cesarean delivery was a NRFHT (control 31% vs CHD 35%, p = 0.67). Fetal acidosis was a rare outcome occurring in only five controls (3.5%) and 11 cases (7.7%) (p = 0.12).
    CONCLUSION: These findings demonstrate that with multidisciplinary care coordination, fetuses with a prenatal diagnosis of CHD have similar cesarean rates, labor and delivery management, and delivery room compromise as healthy fetuses.
    DOI:  https://doi.org/10.1038/s41372-019-0528-1