bims-hylehe Biomed News
on Hypoplastic left heart syndrome
Issue of 2020‒01‒12
nine papers selected by
Richard James
University of Pennsylvania


  1. Am J Cardiol. 2019 Dec 09. pii: S0002-9149(19)31347-5. [Epub ahead of print]
    Raskind-Hood C, Saraf A, Riehle-Colarusso T, Glidewell J, Gurvitz M, Dunn JE, Lui GK, Van Zutphen A, McGarry C, Hogue CJ, Hoffman T, Rodriguez Iii FH, Book WM.
      Improved treatment of congenital heart defects (CHDs) has resulted in women with CHDs living to childbearing age. However, no US population-based systems exist to estimate pregnancy frequency or complications among women with CHDs. Cases were identified in multiple data sources from 3 surveillance sites: Emory University (EU) whose catchment area included 5 metropolitan Atlanta counties; Massachusetts Department of Public Health (MA) whose catchment area was statewide; and New York State Department of Health (NY) whose catchment area included 11 counties. Cases were categorized into one of 5 mutually exclusive CHD severity groups collapsed to severe versus not severe; specific ICD-9-CM codes were used to capture pregnancy, gestational complications, and nongestational co-morbidities in women, age 11 to 50 years, with a CHD-related ICD-9-CM code. Pregnancy, CHD severity, demographics, gestational complications, co-morbidities, and insurance status were evaluated. ICD-9-CM codes identified 26,655 women with CHDs, of whom 5,672 (21.3%, range: 12.8% in NY to 22.5% in MA) had codes indicating a pregnancy. Over 3 years, age-adjusted proportion pregnancy rates among women with severe CHDs ranged from 10.0% to 24.6%, and 14.2% to 21.7% for women with nonsevere CHDs. Pregnant women with CHDs of any severity, compared with nonpregnant women with CHDs, reported more noncardiovascular co-morbidities. Insurance type varied by site and pregnancy status. These US population-based, multisite estimates of pregnancy among women with CHD indicate a substantial number of women with CHDs may be experiencing pregnancy and complications. In conclusion, given the growing adult population with CHDs, reproductive health of women with CHD is an important public health issue.
    DOI:  https://doi.org/10.1016/j.amjcard.2019.12.001
  2. J Thorac Cardiovasc Surg. 2019 Nov 14. pii: S0022-5223(19)32759-X. [Epub ahead of print]
    Ahmed H, Anderson JB, Bates KE, Fleishman CE, Natarajan S, Ghanayem NS, Sleeper LA, Lannon CM, Brown DW, .
      OBJECTIVE: To develop a risk score to predict mortality or transplant in the interstage period.BACKGROUND: The "interstage" period between the stage 1 and stage 2 palliation is a time of high morbidity and mortality for infants with single-ventricle congenital heart disease.
    METHODS: This was an analysis of patients with single-ventricle congenital heart disease requiring arch reconstruction who were enrolled in the National Pediatric Cardiology Quality Improvement Collaborative registry from 2008 to 2015. The primary composite endpoint was interstage mortality or transplant. Multivariable logistic regression and classification and regression tree analysis were performed on two-thirds of the patients ("learning cohort") to build a risk score for the composite endpoint, that was validated in the remaining patients ("validation cohort").
    RESULTS: In the 2128 patients analyzed in the registry, the overall event rate was 9% (153 [7%] deaths, 42 [2%] transplants). In the learning cohort, factors independently associated with the composite endpoint were (1) type of Norwood; (2) postoperative ECMO; (3) discharge with Opiates; (4) No Digoxin at discharge; (5) postoperative Arch obstruction, (6) moderate-to-severe Tricuspid regurgitation without an oxygen requirement, and (7) Extra Oxygen required at discharge in patients with moderate-to-severe tricuspid regurgitation. This model was used to create a weighted risk score ("NEONATE" score; 0-76 points), with >75% accuracy in the learning and validation cohorts. In the validation cohort, the event rate in patients with a score >17 was nearly three times those with a score ≤17.
    CONCLUSIONS: We introduce a risk score that can be used post-stage 1 palliation to predict freedom from interstage mortality or transplant.
    Keywords:  cardiovascular surgery; congenital heart disease; mortality/survival; transplant
    DOI:  https://doi.org/10.1016/j.jtcvs.2019.11.001
  3. Congenit Heart Dis. 2019 Nov;14(6): 1058-1065
    Butto A, Mercer-Rosa L, Teng C, Daymont C, Edelson J, Faerber J, Mejia E, Ravishankar C, Cohen MS.
      OBJECTIVE: Children with single ventricle cardiac disease (SVCD) have poor growth in early life. Tube-assisted feeding (TF) is used to improve weight gain, but its impact on long-term growth remains unknown. We sought to compare the longitudinal growth of SVCD patients receiving TF after initial cardiac surgery with those fed entirely by mouth.DESIGN: We conducted a retrospective cohort study of SVCD patients who underwent initial surgical palliation between 1999 and 2009. We defined TF as the use of nasogastric, gastrostomy, or jejunostomy TF. We compared maximal attained growth z-scores for each year of life between TF and non-TF patients. A secondary analysis compared surgical and clinical factors between groups.
    RESULTS: A total of 134 patients were included; 64% were male and 68% underwent the Norwood operation. One third of patients (44) received TF. Adjusting for age, TF patients had an average of 0.56 lower weight-for-age z-score (WAZ) than non-TF patients (P = 0.007) through the age of 6 years. Longitudinal height was not affected by TF status (P = 0.15). In a subanalysis of Norwood patients, TF patients had lower WAZ at initial hospital discharge despite longer LOS. TF patients had diminished WAZ after adjusting for complications, interstage hospitalizations, and timing of subsequent operations.
    CONCLUSIONS: In this single-center study, patients with SVCD requiring TF at discharge from initial surgical palliation had diminished WAZ at discharge and on long-term follow-up, despite controlling for other identifiable risk factors. Further investigation is needed to understand the mechanisms underlying this phenomenon and to risk stratify infants who go home on TF.
    Keywords:  Norwood; growth; single ventricle; tube-assisted feeds
    DOI:  https://doi.org/10.1111/chd.12843
  4. Can J Cardiol. 2019 Nov 04. pii: S0828-282X(19)31386-8. [Epub ahead of print]
    Tunuguntla H, Conway J, Villa C, Rapoport A, Jeewa A.
      Durable ventricular assist devices (VADs) have significantly improved survival to transplantation among children with advanced stages of heart failure. The fundamental goals of VAD therapy include decreasing mortality, minimizing adverse events, and improving quality of life. As the pediatric VAD experience has evolved with reduced device related complications and improved survival, VAD therapy is being considered not only as a bridge to transplantation (BTT) but also as a bridge to decision (BTD) and as destination therapy (DT). Data regarding pediatric DT VAD are limited to anecdotal or case reports of children being supported for long periods with VADs and by default being classified as DT VAD. This article reviews current trends in the use of DT VAD and adverse events in children vs adults on VAD, and provides a framework for patient selection with the use of a multidisciplinary approach including palliative care. The general approach to determining DT VAD candidacy should include: 1) a reasonable success that the patient will survive the peri- and postoperative state; and 2) a high likelihood that the patient will be able to be discharged out of hospital and have adequate caregiver support. Patients with muscular dystrophy and failing Fontan physiology are examples of pediatric populations for whom DT VAD may be considered and which require unique considerations.
    DOI:  https://doi.org/10.1016/j.cjca.2019.10.033
  5. Dev Med Child Neurol. 2020 Jan 07.
    Dorner RA, Boss RD, Burton VJ, Raja K, Lemmon ME.
      AIM: To determine whether, and how, neonatal intensive care unit (NICU) parents want to receive early neurodevelopmental screening information about their child's future risk of cerebral palsy and other disabilities.METHOD: This was a qualitative interview study. Parents of hospitalized infants born preterm completed semi-structured interviews. Data were analysed using a directed content analysis approach.
    RESULTS: Thematic saturation was achieved after 19 interviews. Four themes characterized parent perceptions of early neurodevelopmental screening: (1) acceptability: most parents were in favour of neurodevelopmental screening if parents could refuse; (2) disclosure of results: parents want emotional preparation for results, especially false positives; (3) emotional burden of uncertainty: parents of children in the NICU balance taking their infant's illness 'day by day' and preparing for an uncertain future. Parents expressed distress with screening that increased uncertainty about the future; and (4) disability: prior experience with disability informs parent concerns.
    INTERPRETATION: Parents interpret the risks and benefits of NICU developmental screening through the lens of prior experiences with disability. Most expressed interest in screening and emphasized a desire for autonomy, pretest counselling, and emotional preparation.
    WHAT THIS PAPER ADDS: Most parents with infants in the neonatal intensive care unit expressed interest in early screening for developmental disability. Prior experience with disability informed concerns about specific deficits. Parents emphasized a desire for autonomy, pretest counselling, and emotional preparation.
    DOI:  https://doi.org/10.1111/dmcn.14457
  6. J Perinatol. 2020 Jan 07.
    Kenaley KM, Rickolt AL, Vandersteur DA, Ryan JD, Stefano JL.
      BACKGROUND: There are many barriers to parental skin-to-skin contact for critically ill neonates. Our aims were to decrease median time to first parental hold of neonates requiring respiratory support from 6.4 to 3 days, and to increase the percentage of neonates held within the first 24 h after birth from 6 to 75%.METHODS: Lean Six Sigma methodology was used to identify barriers to holding and opportunities for improvement.
    INTERVENTION: A multifactorial improvement bundle was implemented to reduce the time to first parental hold of critically ill neonates.
    RESULTS: Median time to first parental hold was reduced from 6.4 to 1.2 days (p < 0.01). Infants held within the first 24 h after birth increased from 6 to 35%. There was no increase in adverse events associated with parental holding.
    CONCLUSIONS: Implementation of an improvement bundle resulted in a significant reduction in time to first parental hold of infants requiring respiratory support.
    DOI:  https://doi.org/10.1038/s41372-019-0569-5
  7. J Am Heart Assoc. 2020 Jan 07. 9(1): e012529
    Rathgeber SL, Guttman OR, Lee AF, Voss C, Hemphill NM, Schreiber RA, Harris KC.
      Background Information is evolving on liver disease in pediatric patients with Fontan physiology. The purpose of this investigation is to evaluate the spectrum of liver disease in a pediatric population of patients with Fontan physiology and evaluate transient elastography (TE) as a noninvasive marker of liver disease. Methods and Results We prospectively enrolled all children with Fontan physiology. All patients underwent comprehensive liver evaluation including liver enzymes (alanine aminotransferase, aspartate aminotransferase, gamma-glutamyl transferase, alkaline phosphatase), aspartate transaminase to platelet ratio index, albumin, bilirubin, international normalized ratio, complete blood cell count, abdominal ultrasound, and TE. Transjugular liver biopsies and hemodynamic measurements were performed in a subset of patients. A total of 76 children (median, 11.7; interquartile range, 8.4-14.8 [56% male]) were evaluated, with 17 having a transjugular liver biopsy (median 14.8 years; interquartile range, 14.3-17.4). All biopsies showed pathological changes. The severity of liver pathology did not correlate with TE. There was a positive correlation between TE and time since Fontan (R=0.42, P<0.01), aspartate transaminase to platelet ratio index (R=0.29, P=0.02), aspartate transaminase (R=-0.42, P<0.01), and platelets (R=-0.42, P<0.01). Splenomegaly on abdominal ultrasound was correlated with TE (z=-2.2, P=0.03), low platelet count (z=1.9, P=0.05), low aspartate transaminase (z=1.9, P=0.02), and low alkaline phosphatase (z=2.4, P=0.02). Conclusions Liver disease was ubiquitous in our cohort of pediatric patients with Fontan Physiology. Given the correlation between TE and time from Fontan, TE shows potential as a prospective marker of liver pathology. However, individual measurements with TE do not correlate with the severity of pathology. Given the prevalence of liver disease in this population, protective measures of liver health as well as routine liver health surveillance should be implemented with consideration for hepatology consultation and biopsy in the event of abnormal liver biochemical markers or imaging.
    Keywords:  Fontan procedure; congenital heart disease; liver
    DOI:  https://doi.org/10.1161/JAHA.119.012529
  8. J Pediatr. 2019 Dec 24. pii: S0022-3476(19)31479-9. [Epub ahead of print]
    Cohn LN, Pechlivanoglou P, Lee Y, Mahant S, Orkin J, Marson A, Cohen E.
      OBJECTIVE: To assess health outcomes of parents caring for children with chronic illnesses compared with parents of healthy children.STUDY DESIGN: We searched OvidSP MEDLINE, EBM Reviews-Cochrane Central Register of Controlled Trials, EMBASE, and EBSCOHost CINAHL through September 2019. Included were English-language studies reporting health conditions or mortality of parents of affected children compared with healthy controls.
    RESULTS: Of 12 181 screened publications, 26 met inclusion criteria. Eight studies reported on anxiety, 23 on depression, 1 on mortality, and 1 on cardiovascular disease. Parents of chronically ill children had greater anxiety (standardized mean difference 0.42; 95% CI 0.24-0.60; P < .001) and depression scores (standardized mean difference 0.35; 95% CI 0.26-0.45; P < .001) than parents of healthy children. Thirty-five percent of parents of affected children met cut-offs for clinical depression, compared with 19% in the control (relative risk 1.75; 95% CI 1.55-1.97). Fifty-seven percent of such parents met cut-offs for anxiety, compared with 38% in the control (relative risk 1.40; 95% CI 1.18-1.67). One study of mothers of children with congenital anomalies reported a greater mortality risk than a comparison (adjusted hazard ratio 1.22; 95% CI 1.15-1.29), and another reported that these mothers experience an increased risk of cardiovascular disease (adjusted hazard ratio 1.15; 95% CI 1.07-1.23).
    CONCLUSIONS: Parents of chronically ill children experience poorer mental health (more anxiety and depression), and mothers of those with congenital anomalies may have greater risk of cardiovascular disease and mortality than parents of unaffected children. Our findings suggest a need for targeted interventions to attenuate adverse parental caregiver health outcomes.
    PRIOR REGISTRATION: PROSPERO CRD42018094657 (https://www.crd.york.ac.uk/prospero/display_record.php?RecordID=94657).
    DOI:  https://doi.org/10.1016/j.jpeds.2019.10.068