bims-hylehe Biomed News
on Hypoplastic left heart syndrome
Issue of 2020‒06‒28
four papers selected by
Richard James
University of Pennsylvania


  1. J Pediatr. 2020 Jul;pii: S0022-3476(20)30322-X. [Epub ahead of print]222 28-34.e4
    Castellanos DA, Lopez KN, Salemi JL, Shamshirsaz AA, Wang Y, Morris SA.
      OBJECTIVE: To examine state-wide population trends in preterm delivery of children with critical congenital heart disease (CHD) over an 18-year period. We hypothesized that, coincident with early advancements in prenatal diagnosis, preterm delivery initially increased compared with the general population, and more recently has decreased.STUDY DESIGN: Data from the Texas Public Use Data File 1999-2016 was used to evaluate annual percent preterm delivery (<37 weeks) in critical CHD (diagnoses requiring intervention at <1 year of age). We first evaluated for pattern change over time using joinpoint segmented regression. Trends in preterm delivery were then compared with all Texas livebirths. We then compared trends examining sociodemographic covariates including race/ethnicity, sex, and neighborhood poverty levels.
    RESULTS: Of 7146 births with critical CHD, 1339 (18.7%) were delivered preterm. The rate of preterm birth increased from 1999 to 2004 (a mean increase of 1.69% per year) then decreased between 2005 and 2016 (a mean decrease of -0.41% per year). This represented a faster increase and then a similar decrease to that noted in the general population. Although the greatest proportion of preterm births occurred in newborns of Hispanic ethnicity and non-Hispanic black race, newborns with higher neighborhood poverty level had the most rapidly increasing rate of preterm delivery in the first era, and only a plateau rather than decrease in the latter era.
    CONCLUSIONS: Rates of preterm birth for newborns with critical CHD in Texas first were increasing rapidly, then have been decreasing since 2005.
    Keywords:  birth defects; ethnicity; fetal echocardiography; health disparities; population-based; poverty; prenatal diagnosis; race
    DOI:  https://doi.org/10.1016/j.jpeds.2020.03.003
  2. Pediatr Cardiol. 2020 Jun 21.
    Jimenez E, Zaban N, Sharma N, Narasimhan S, Martin-Chafee C, Lohr JL, Cortez D.
      Long-term right ventricular pacing is associated with left ventricular dysfunction and cardiomyopathy, particularly in pediatric patients and those with congenital heart disease (CHD). Research has shown that pacing-induced cardiomyopathy can be reversed with nonselective or selective His bundle pacing in adults, however, the information available about the use of this type of therapy in pediatrics and CHD is scarce. We performed a retrospective chart review of all the cases of His or left bundle pacing at the University of Minnesota, division of Pediatric Cardiology from January of 2019 to April of 2020. Parametric data are presented as mean ± standard deviation. Non-parametric data are presented as median value with interquartile ranges. Eight patients, ages 8 to 18 years (median of 11.5) and weight from 21.5 to 81.6 kg (median of 40 kg) underwent this procedure successfully. The most common structural heart disease was a repaired peri-membranous ventricular septal defect. Three patients (37.5%) had selective and three (37.5%) had nonselective His bundle pacing, and two patients (25%) had left bundle pacing. There were two cases of pacing-induced cardiomyopathy and each had a 14% and 16% improvement of the ejection fraction after nonselective His bundle pacing. There were no procedural complications. Selective and nonselective His bundle, as well as left bundle pacing may be a feasible procedure in pediatric patients with and without CHD. This procedure may improve pacing-induced cardiomyopathy in this population.
    Keywords:  Congenital heart disease; His bundle pacing; Pediatric
    DOI:  https://doi.org/10.1007/s00246-020-02398-9
  3. Ultrasound Obstet Gynecol. 2020 Jun 23.
    Paladini D, Finarelli A, Donarini G, Parodi S, Lombardo V, Tuo G, Birnbaum R.
      OBJECTIVE: The primary objective of this study is to assess whether foetuses with congenital heart disease (CHD) have smaller frontal brain areas than normal controls. Secondary objective is to evaluate whether there is any difference among CHD with different haemodynamics.METHODS: Retrospective cross-sectional study, including 421 normal foetuses and 101 fetuses with isolated CHD. The study group was subdivided according to the CHD haemodynamics into the following subcategories: 1) Hypoplastic left heart syndrome (HLHS) and other forms of functionally univentricular heart defects; 2) Transposition of the Great Arteries; 3) conotruncal defects and other CHDs with large shunts; 4) right ventricular outflow tract obstruction, without a hypoplastic right ventricle; 5) left outflow tract obstruction; 6) others. The transventricular axial view of the fetal head was used as reference view, on which the Frontal Antero-Posterior Diameter (FAPD) and the Occipito-Frontal Diameter (OFD) were measured, assuming the former as representative of the frontal lobes' area. The FAPD/OFD ratio was then calculated (FAPD/OFD*100). These two variables (FAPD and FAPD/OFD Ratio) were then evaluated in the study and control group. Statistics included Kruskal-Wallis and Mann-Whitney U tests for two groups' comparison. Adjustment for gestational age both via multiple linear regression model and by using the a posteriori matching based on the propensity score was also employed.
    RESULTS: In normal foetuses, FAPD showed a linear positive correlation with gestational age. In foetuses with CHD, the FAPD was shorter than in normal foetuses at all gestational ages, with the difference increasing after 30 gestational weeks. The FAPD/OFD Ratio was significantly lower in foetuses with CHD than in normal foetuses (p < 0.0001) at all gestational ages, with no differences among the various CHD categories, which all showed lower FAPD/OFD Ratios than normals (p < 0.0001).
    CONCLUSIONS: Fetuses with CHD show a shorter FAPD and a lower FAPD/OFD than normal foetuses. This impaired growth of the frontal area of the brain seems to occur in all types of CHD, regardless of their haemodynamics. This article is protected by copyright. All rights reserved.
    Keywords:  Central Nervous System; cavum septi pellucidi; congenital heart disease; fetus; frontal lobe; ultrasound
    DOI:  https://doi.org/10.1002/uog.22127
  4. Eur J Cardiothorac Surg. 2020 Jun 23. pii: ezaa117. [Epub ahead of print]
    Hunt ML, Ittenbach RF, Kaplinski M, Ravishankar C, Rychik J, Steven JM, Fuller SM, Nicolson SC, Spray TL, Gaynor JW, Mascio CE.
      OBJECTIVES: The objective of this study was to estimate hospital mortality and length of stay (LOS) for children with hypoplastic left heart syndrome undergoing superior cavopulmonary connection (SCPC).METHODS: All hypoplastic left heart syndrome interstage survivors who underwent SCPC between 1 January 1988 and 31 December 2017 were included. The study period was divided into 4 eras based on changes in operative or medical management. Mortality rates were estimated using standard binomial proportions. Adjusted and unadjusted logistic regression models were used to identify risk factors for mortality and LOS.
    RESULTS: The most common procedures for the cohort (n = 958) were Hemi-Fontan (57.3%) or Bidrectional Glenn shunt (35.7%). The mortality was 4.1% overall and decreased in all 3 later eras compared to era 1. Factors associated with mortality in a multiple covariate model included longer total support time, earlier gestational age, longer LOS at the Norwood Procedure and need for additional procedures. Overall, the median LOS was 7.0 days with a decrease from eras 1 to 2 and plateaued in eras 3 and 4. Predictors of longer LOS included genetic anomaly, longer Norwood LOS, additional procedures, lower weight at surgery and longer total support time. The type of SCPC was not associated with mortality or LOS.
    CONCLUSIONS: In this large cohort of patients with hypoplastic left heart syndrome undergoing SCPC, hospital mortality has decreased significantly. LOS initially declined but plateaued in recent eras. The risk factors for mortality and longer LOS are related to patient and procedural complexity, especially the need for additional procedures at the time of SCPC.
    Keywords:  Hypoplastic left heart syndrome; Outcomes; Single ventricle; Superior cavopulmonary connection
    DOI:  https://doi.org/10.1093/ejcts/ezaa117