bims-hylehe Biomed News
on Hypoplastic left heart syndrome
Issue of 2020‒08‒09
five papers selected by
Richard James
University of Pennsylvania


  1. Ann Thorac Surg. 2020 Aug 04. pii: S0003-4975(20)31286-8. [Epub ahead of print]
    Serfas JD, Thibault D, Andersen ND, Chiswell K, Jacobs JP, Jacobs ML, Krasuski RA, Lodge AJ, Turek JW, Hill KD.
      BACKGROUND: Fontan failure often requires surgical therapy in the form of Fontan revision or heart transplantation. We sought to characterize national trends in the surgical burden of Fontan failure and identify risk factors for adverse outcomes.METHODS: Fontan patients undergoing Fontan revision or transplant from 1/2010-6/2018 were included. We evaluated baseline characteristics and outcomes and used multivariable logistic regression to identify risk factors for operative mortality and composite mortality/major morbidity.
    RESULTS: 1135 patients underwent Fontan revision (n=598) or transplant (n=537) at 100 centers. Transplants increased from 34 in 2010 to 76 in 2017, largely due to increase in patients with hypoplastic left heart syndrome (HLHS, 18 in 2010 to 49 in 2017), while Fontan revision decreased (75 in 2010 to 49 in 2017). Transplant patients were younger (median 14yrs vs 18yrs, p< 0.0001), more often had preoperative risk factors (66% vs. 40%, p<0.0001), and more often had HLHS (51% vs 15%, p< 0.0001). Operative mortality and composite major morbidity/mortality were 7.6% and 35% for transplant and 7.1% and 22% for Fontan revision. Multivariable risk factors for mortality included older age (OR 1.08/year, p=0.0065), presence of preoperative risk factors (OR 3.33, p=0.0022), and concomitant pulmonary artery reconstruction (OR 2.7, p=0.0288) for Fontan revision but only older age (OR 1.06/year, p=0.0199) for transplant.
    CONCLUSIONS: Both transplantation and Fontan revision are associated with high morbidity and mortality. There has been evolution of practices in surgical therapy for Fontan failure, perhaps related to rising prevalence of HLHS staged palliation.
    Keywords:  Fontan; Fontan revision; congenital heart disease; heart transplantation
    DOI:  https://doi.org/10.1016/j.athoracsur.2020.05.174
  2. J Thorac Cardiovasc Surg. 2020 Jun 25. pii: S0022-5223(20)31549-X. [Epub ahead of print]
    Tjoeng YL, Jenkins K, Deen JF, Chan T.
      OBJECTIVE: Prior studies demonstrate an association between nonwhite race/ethnicity, insurance status, and mortality after pediatric congenital heart surgery. The influence of severity of illness on that association is unknown. We examined the relationship between race/ethnicity, severity of illness, and mortality in congenital cardiac surgery, and whether severity of illness is a mechanism by which nonwhite patients experience increased surgical mortality.METHODS: We performed a retrospective cohort study of children younger than age 18 years old undergoing cardiac surgery admitted to the intensive care unit (n = 40,545) between 2009 and 2016 from the Virtual Pediatric Systems (VPS, LLC, Los Angeles, Calif) database. Multivariate regression models were constructed to examine the role of severity of illness as a mediator between race/ethnicity and mortality in children undergoing cardiac surgery.
    RESULTS: In multivariate models examining severity of illness scores, African-American patients had statistically significant higher severity of illness scores when compared with their white counterparts. In multivariate models of intensive care unit mortality after adjustment for covariates, African-American patients had a higher odds of postoperative mortality (odds ratio, 1.40, 95% confidence interval, 1.04-1.89) when compared with white children. This increased odds of mortality was mediated through higher severity of illness, because adjustment for severity of illness removed this survival disadvantage for black patients.
    CONCLUSIONS: Although African-American children undergoing cardiac surgery had higher postoperative mortality, this survival difference appears to be mediated via severity of illness. Preoperative and intraoperative factors may be drivers for this survival disparity.
    Keywords:  congenital heart disease; congenital heart surgery; ethnicity; health disparities; mortality; race; severity of illness
    DOI:  https://doi.org/10.1016/j.jtcvs.2020.06.015
  3. J Thorac Cardiovasc Surg. 2020 Jul 04. pii: S0022-5223(20)31981-4. [Epub ahead of print]
    d'Udekem Y, Thambo JB, Iyengar AJ, Rychik J.
      
    Keywords:  AHA scientific statements; Fontan procedure; clinical guidelines; congenital heart defects; univentricular circulation
    DOI:  https://doi.org/10.1016/j.jtcvs.2020.03.186
  4. Cardiol Young. 2020 Aug 04. 1-6
    Rodts ME, Unaka NI, Statile CJ, Madsen NL.
      BACKGROUND: CHD is the most common birth defect type, with one-fourth of patients requiring intervention in the first year of life. Caregiver understanding of CHD may vary. Health literacy may be one factor contributing to this variability.METHODS: The study occurred at a large, free-standing children's hospital. Recruitment occurred at a free-of-charge CHD camp and during outpatient cardiology follow-up visits. The study team revised the CHD Guided Questions Tool from an eighth- to a sixth-grade reading level. Caregivers of children with CHD completed the "Newest Vital Sign" health literacy screen and demographic surveys. Health literacy was categorised as "high" (Newest Vital Sign score 4-6) or "low" (score 0-3). Caregivers were randomised to read either the original or revised Guided Questions Tool and completed a validated survey measuring understandability and actionability of the Guided Questions Tool. Understandability and actionability data analysis used two-sample t-testing, and within demographic group differences in these parameters were assessed via one-way analysis of variance.
    RESULTS: Eighty-two caregivers participated who were largely well educated with a high income. The majority (79.3%) of participants scored "high" for health literacy. No differences in understanding (p = 0.43) or actionability (p = 0.11) of the original and revised Guided Questions Tool were noted. There were no socio-economic-based differences in understandability or actionability (p > 0.05). There was a trend towards improved understanding of the revised tool (p = 0.06).
    CONCLUSIONS: This study demonstrated that readability of the Guided Questions Tool could be improved. Future work is needed to expand the study population and further understand health literacy's impact on the CHD community.
    Keywords:  CHD; Health literacy; patient and family education
    DOI:  https://doi.org/10.1017/S1047951120002243
  5. Pediatr Cardiol. 2020 Aug 01.
    Sethasathien S, Silvilairat S, Sittiwangkul R, Makonkawkeyoon K, Pongprot Y.
      Although the Fontan operation can improve outcomes, surviving patients still face Fontan-associated liver disease (FALD). The aim of this study was to determine the associated factors of FALD in relation to ultrasound liver elastography. A cross-sectional study was conducted for all patients on whom an ultrasound upper-abdomen and ultrasound liver elastography was performed at more than 1 year after the Fontan operation. The data consisted of the age at operation, type of Fontan operation, and laboratory data such as gamma-glutamyl transferase (GGT), aspartate aminotransferase-to-platelet ratio index (APRI), and fibrosis-4 (FIB-4) score. Cardiovascular evaluations included echocardiography and cardiac catheterization. Eighty patients with a median age of 12 years (range 5-36 years) were eligible for the study. The prevalence of FALD was 41%. For the purpose of univariate logistic regression analysis, the age at the Fontan operation, time elapsed since the Fontan operation, previous Glenn shunt, presence of fenestration, mean pulmonary artery pressure and IVC pressure post-Fontan operation, platelet count, GGT, and FIB-4 score were considered to be factors significantly associated with FALD. Following an adjustment by multivariate logistic regression analysis, age greater than 7 years at the time of Fontan procedure, time elapsed of more than 9 years since the procedure, and GGT level > 130 U/L were found to be significantly associated with FALD. Patients with these factors should be investigated for FALD.
    Keywords:  APRI; FIB-4 score; Fontan operation; Fontan-associated liver disease; Ultrasound liver elastography
    DOI:  https://doi.org/10.1007/s00246-020-02422-y