bims-hylehe Biomed News
on Hypoplastic left heart syndrome
Issue of 2020‒04‒12
three papers selected by
Richard James
University of Pennsylvania


  1. J Thorac Dis. 2020 Mar;12(3): 1174-1183
    Karamlou T, Najm HK.
      Since initial descriptions of staged palliation for hypoplastic left heart syndrome (HLHS) in the 1980's, much has been learned about the pathophysiology of the single ventricle circulation. New therapies that leverage systems biology and clinical derivatives have been developed. While in-hospital mortality and morbidity for babies with HLHS have continued to improve, there remains a long (and daresay winding) road ahead to achieve ideal outcomes. Important variation in even these abbreviated in-hospital metrics persists among institutions and currently utilized prediction models explain only a small amount of this variation. Moreover, long-term survival and neurodevelopmental health for patients with HLHS are infrequently reported and remain suboptimal despite improved in-hospital outcomes. This focused review will describe the evolution of national outcomes for HLHS over time and the potential factors motivating improved time-related mortality. Emerging modifiable risk-factors that hold promise in terms of moving the needle for long-term success, including social determinants of health and the delineation of genetic profiles, will be discussed. Specifically, this review will integrate contemporary data based on the first murine HLHS models that suggest a genetically elicited modular phenotype with environmental factors known to impact the initial durability of surgical therapies. A comprehensive approach to the management of HLHS, which leverages both proactive transplantation and hybrid palliation, in addition to traditional Norwood palliation, will be emphasized to extend and match management to the complete spectrum of patient risk-profiles. Finally, we will explore the critical role that national collaboratives and quality reporting initiatives have played in improving outcomes and shifting the focus to more meaningful long-term survival and neurodevelopment.
    Keywords:  Congenital heart disease (CHD); hypoplastic left heart; outcomes; single ventricle
    DOI:  https://doi.org/10.21037/jtd.2019.10.75
  2. Ann Thorac Surg. 2020 Apr 01. pii: S0003-4975(20)30431-8. [Epub ahead of print]
    Parker DM, Everett AD, Stabler ME, Jacobs ML, Jacobs JP, Vricella L, Thiessen-Philbrook H, Parikh CR, Manlhiot C, Brown JR.
      BACKGROUND: Approximately 10% to 20% of children are readmitted after congenital heart surgery. Very little is known about biomarkers as predictors of risk of unplanned readmission following pediatric congenital heart surgery. Novel cardiac biomarker ST2 may be associated with risk of unplanned readmission. ST2 concentrations are believed to reflect cardiovascular stress and fibrosis. Our objective was to explore the relationship between pre- and postoperative ST2 biomarker levels and risk of readmission within one year after congenital heart surgery.METHODS: We prospectively enrolled pediatric patients <18 years of age who underwent at least one congenital heart operation at Johns Hopkins Hospital from 2010 - 2014. Plasma samples were collected immediately prior to surgery and at the end of bypass. We used Kaplan-Meier survival analysis and multivariable Cox regression models adjusting for variables used in the Society of Thoracic Surgeons (STS) Congenital Heart Surgery Database mortality risk model.
    RESULTS: In our cohort (N=145), there were 39 children with readmissions within 365 days. The median time to unplanned readmission was 54 days (IQR: 10 - 153). Kaplan-Meier analysis demonstrated a significant difference across terciles of pre- and post-operative ST2 biomarker levels. After adjustment, elevated serum levels of ST2 measured preoperatively and postoperatively were associated with increased risk of readmission (hazard ratio range 2.5 to 3.7, all p<0.05).
    CONCLUSIONS: Elevated levels of ST2 are significantly associated with increased risk of unplanned readmission within one-year after pediatric congenital heart surgery. Novel serum biomarker ST2 can be used for risk stratification or estimating postsurgical prognosis.
    Keywords:  biomarkers; congenital heart surgery; pediatric congenital heart disease; prediction; readmission
    DOI:  https://doi.org/10.1016/j.athoracsur.2020.02.056
  3. J Thorac Dis. 2020 Mar;12(3): 1194-1203
    Hermsen JL, Roldan-Alzate A, Anagnostopoulos PV.
      Three-dimensional printing (3Dp) has a broad array of medical applications and has been applied extensively in congenital heart disease given the variety and complexity of lesions encountered. 3Dps are unique when compared to other imaging modalities in that they are theoretically equally interpretable by radiologists, cardiologists and surgeons. The literature regarding 3Dp in congenital heart disease is recent and burgeoning. This review, categorized by application, should provide the reader with a comprehensive, albeit not complete, overview of 3Dp in congenital heart disease during the last decade.
    Keywords:  Three-dimensional printing (3Dp); cardiac surgery; congenital heart defect; rapid prototyping; simulation
    DOI:  https://doi.org/10.21037/jtd.2019.10.38