bims-hylehe Biomed News
on Hypoplastic left heart syndrome
Issue of 2019‒09‒22
nine papers selected by
Richard James
University of Pennsylvania


  1. Eur J Prev Cardiol. 2019 Sep 17. 2047487319876231
    Holbein CE, Peugh J, Veldtman GR, Apers S, Luyckx K, Kovacs AH, Thomet C, Budts W, Enomoto J, Sluman MA, Lu CW, Jackson JL, Khairy P, Cook SC, Chidambarathanu S, Alday L, Eriksen K, Dellborg M, Berghammer M, Johansson B, Mackie AS, Menahem S, Caruana M, Soufi A, Fernandes SM, White K, Callus E, Kutty S, Moons P, .
      BACKGROUND: Health behaviours are essential to maintain optimal health and reduce the risk of cardiovascular complications in adults with congenital heart disease. This study aimed to describe health behaviours in adults with congenital heart disease in 15 countries and to identify patient characteristics associated with optimal health behaviours in the international sample.DESIGN: This was a cross-sectional observational study.
    METHODS: Adults with congenital heart disease (n = 4028, median age = 32 years, interquartile range 25-42 years) completed self-report measures as part of the Assessment of Patterns of Patient-Reported Outcomes in Adults with Congenital Heart disease - International Study (APPROACH-IS). Participants reported on seven health behaviours using the Health Behaviors Scale-Congenital Heart Disease. Demographic and medical characteristics were assessed via medical chart review and self-report. Multivariate path analyses with inverse sampling weights were used to investigate study aims.
    RESULTS: Health behaviour rates for the full sample were 10% binge drinking, 12% cigarette smoking, 6% recreational drug use, 72% annual dental visit, 69% twice daily tooth brushing, 27% daily dental flossing and 43% sport participation. Pairwise comparisons indicated that rates differed between countries. Rates of substance use behaviours were higher in younger, male participants. Optimal dental health behaviours were more common among older, female participants with higher educational attainment while sports participation was more frequent among participants who were younger, male, married, employed/students, with higher educational attainment, less complex anatomical defects and better functional status.
    CONCLUSIONS: Health behaviour rates vary by country. Predictors of health behaviours may reflect larger geographic trends. Our findings have implications for the development and implementation of programmes for the assessment and promotion of optimal health behaviours in adults with congenital heart disease.
    Keywords:  Heart defects; congenital; health behaviour; patient-reported outcome measures; prevention; risk factors
    DOI:  https://doi.org/10.1177/2047487319876231
  2. Cardiol Young. 2019 Sep 16. 1-7
    Bratt EL, Järvholm S, Ekman-Joelsson BM, Johannsmeyer A, Carlsson SÅ, Mattsson LÅ, Mellander M.
      INTRODUCTION: A diagnosis of congenital heart disease (CHD) in offspring triggers psychological distress in parents. Results of previous studies have been inconsistent regarding the psychological impact of a prenatal versus a postnatal diagnosis. The aim of this study was to evaluate the influence of the time of diagnosis on levels of parental distress.METHODS: Pregnant women and their partners with a fetus diagnosed with complex CHD, parents of children with postnatally diagnosed CHD, and pregnant women and their partners with uncomplicated pregnancies were invited to participate. Data were collected during pregnancy and 2-6 months after delivery using the Hospital Anxiety and Depression Scale, sense of coherence, life satisfaction, and Dyadic Adjustment Scale.
    RESULTS: During pregnancy, the prenatal group scored lower sense of coherence compared to controls (p=0.044). Postnatally the prenatal group scored lower on sense of coherence compared to the postnatal group and controls (p=0.001; p=0.001). Postnatally, the prenatal and postnatal groups had higher levels of anxiety compared to controls (p=0.025; p=0.0003). Life satisfaction was lower in the prenatal group compared to that in the postnatal group and in controls (p=0.000; p=0.0004).
    CONCLUSION: Parents with a prenatal diagnosis of CHD in offspring report a low sense of coherence already during pregnancy which decreased further at follow-up. The same group reported a lower satisfaction with life compared to parents of a child with postnatal diagnosis of CHD and parents of a healthy child. This motivates further efforts to improve counselling and support during pregnancy and for parents after a prenatal diagnosis.
    Keywords:  Anxiety; congenital; depression; heart defects; infant; newborn; parents; prenatal diagnosis; sense of coherence
    DOI:  https://doi.org/10.1017/S1047951119001781
  3. Paediatr Anaesth. 2019 Sep 18.
    Fang A, Allen KY, Marino BS, Brady KM.
      Patients who have undergone congenital heart surgery may appear outwardly normal, but their neurodevelopmental outcomes are often abnormal. Short- and long-term survival of patients with complex congenital heart disease (CHD) has improved significantly over the past several decades.1,2 This has led to a paradigm shift from mortality reduction to morbidity prevention. This review will define the neurodevelopmental and psychosocial phenotype in CHD surgical survivors, and examine the impact of surgical, cardiopulmonary bypass, and monitoring techniques, as well as post-operative care on neurodevelopmental outcome.
    Keywords:  Congenital heart disease; neurodevelopment
    DOI:  https://doi.org/10.1111/pan.13744
  4. Ann Thorac Surg. 2019 Sep 11. pii: S0003-4975(19)31359-1. [Epub ahead of print]
    Tanem J, Rudd N, Rauscher J, Scott A, Frommelt MA, Hill GD.
      BACKGROUND: Multiple single ventricle populations are noted to be at increased risk for mortality following the Norwood Procedure. Pre-operative risk factors include low birth weight (LBW), restrictive/intact atrial septum, obstructed pulmonary veins, ventricular dysfunction and atrioventricular valve (AVV) regurgitation. We report outcomes of the Norwood procedure in standard and high risk patients in the recent era.METHODS: All patients born with Hypoplastic Left Heart Syndrome (HLHS) between 2006 and 2016 who underwent a Norwood procedure at our institution were included. Retrospective review of patient data was performed and Kaplan-Meier analysis was used to evaluate survival between groups.
    RESULTS: The cohort included 177 patients. Fifty patients were determined high risk preoperatively: LBW (n=18), ventricular dysfunction/AVV regurgitation (n=13), intact or restrictive atrial septum/obstructed anomalous pulmonary venous return (n=14), and multiple factors (n=5). There were 2 (1.6%) deaths prior to Glenn in the standard risk group with a total of 10 (20%) from the high risk groups (p<0.0001). One year survival differed greatly between groups with highest being standard risk at 89% and lowest in the intact septum/obstructed veins group at 54%. There was a significant difference between groups in long term survival (p<0.001).
    CONCLUSIONS: Outcomes following the Norwood procedure have improved for standard risk patients. Those with pre-operative risk factors account for the majority of early mortality following the Norwood procedure. This high risk status does not resolve after Glenn, as longer term survival continues to diverge from the standard risk group.
    DOI:  https://doi.org/10.1016/j.athoracsur.2019.07.070
  5. J Am Coll Cardiol. 2019 Sep 24. pii: S0735-1097(19)35507-X. [Epub ahead of print]74(12): 1570-1579
    Savla JJ, Faerber JA, Huang YV, Zaoutis T, Goldmuntz E, Kawut SM, Mercer-Rosa L.
      BACKGROUND: There is ongoing debate about the best strategy to treat patients with tetralogy of Fallot who are symptomatic in the neonatal period.OBJECTIVES: The aim of this study was to compare the outcomes of complete versus staged surgery (i.e., initial palliative procedure for possible later complete repair).
    METHODS: A retrospective cohort study was performed using the Pediatric Health Information System database, including patients who underwent complete or staged tetralogy of Fallot repair prior to 30 days of age. The primary outcome was death during 2-year follow-up after the initial procedure. Inverse probability-weighted Cox and logistic regression models were used to examine the association between surgical approach group and mortality while accounting for patient- and hospital-level factors. Causal mediation analyses examined the role of intermediate variables.
    RESULTS: A total of 2,363 patients were included (1,032 complete and 1,331 staged). There were 239 deaths. Complete neonatal repair was associated with a significantly higher risk for mortality during the 2-year follow-up period (hazard ratio: 1.51; 95% confidence interval: 1.05 to 2.06), between 7 and 30 days after the initial procedure (hazard ratio: 2.29; 95% confidence interval: 1.18 to 4.41), and during the initial hospital admission (odds ratio: 1.72; 95% confidence interval: 1.15 to 2.62). Post-operative cardiac complications were more common in the complete repair group and mediated the differences in 30-day and 2-year mortality.
    CONCLUSIONS: Complete surgical repair for neonates with tetralogy of Fallot is associated with a significantly higher risk for early and 2-year mortality compared with the staged approach, after accounting for patient and hospital characteristics. Post-operative cardiac complications mediated these findings.
    Keywords:  Pediatric Health Information System; cardiac surgery; causal mediation analysis; comparative effectiveness; congenital heart disease; inverse probability weighting
    DOI:  https://doi.org/10.1016/j.jacc.2019.05.057
  6. BMJ Paediatr Open. 2019 ;3(1): e000499
    Ishihara T, Tanaka H.
      Objectives: The primary objective is to clarify the clinical profiles of paediatric patients who died in intensive care units (ICUs) or paediatric intensive care units (PICUs), and the secondary objective is to ascertain the demographic differences between patients who died with and without chronic conditions.Methods: In this retrospective multicentre cohort study, we collected data on paediatric death from the Japanese Registry of Pediatric Acute Care (JaRPAC) database. We included patients who were ≤16 years of age and had died in either a PICU or an ICU of a participating hospital between April 2014 and March 2017. The causes of death were compared between patients with and without chronic conditions.
    Results: Twenty-three hospitals participated, and 6199 paediatric patients who were registered in the JaRPAC database were included. During the study period, 126 (2.1%) patients died (children without chronic illness, n=33; children with chronic illness, n=93). Twenty-five paediatric patients died due to an extrinsic disease, and there was a significant difference in extrinsic diseases between the two groups (children without chronic illness, 15 (45%); children with chronic illness, 10 (11%); p<0.01). Cardiovascular disease was the most common chronic condition (27/83, 29%). Eighty-three patients (85%) in the chronic group died due to an intrinsic disease, primarily congenital heart disease (14/93, 15%), followed by sepsis (13/93, 14%).
    Conclusions: The majority of deaths were in children with a chronic condition. The major causes of death in children without a chronic illness were due to intrinsic factors such as cardiovascular and neuromuscular diseases, and the proportion of deaths due to extrinsic causes was higher in children without chronic illness.
    Keywords:  accident & emergency; epidemiology; intensive care; mortality
    DOI:  https://doi.org/10.1136/bmjpo-2019-000499
  7. Qual Health Res. 2019 Sep 16. 1049732319869909
    Svensson MK, Wahlberg A, Gislason GH.
      There have been substantial advances in the diagnostics and treatment of congenital heart defects (CHDs) in recent decades, and this has improved survival significantly. Consequently, there is a growing interest in how CHDs affect the daily lives of children and youth. We examine life with CHDs as a particular kind of living from the perspectives of both children and youth with CHDs and their families through a systematic review of existing qualitative research. Based on a meta-ethnographic analysis of 20 articles (identified through PubMed, EMBASE, EBSCOhost, PSYCHinfo, Scopus, and Web of Science from January 7 to 12, 2016), we argue that living with CHDs is characterized by chronic paradoxes arising out of the transitions, normalities, and futures that families have to navigate.
    Keywords:  children’s health; chronic illness; chronic paradoxes; congenital heart defects; families; meta-ethnography; qualitative research; systematic review
    DOI:  https://doi.org/10.1177/1049732319869909
  8. HEC Forum. 2019 Sep 18.
    Gerdes H, Lantos J.
      When should doctors seek protective custody to override a parent's refusal of potentially lifesaving treatment for their child? The answer to this question seemingly has different answers for different subspecialties of pediatrics. This paper specifically looks at different thresholds for physicians overriding parental refusals of life-sustaining treatment between neonatology, cardiology, and oncology. The threshold for mandating treatment of premature babies seems to be a survival rate of 25-50%. This is not the case when the treatment in question is open heart surgery for a child with congenital heart disease. Most cardiologists would not pursue legal action when parents refuse treatment, unless the anticipated survival rate after surgery is above 90%. In pediatric oncology, there are case reports of physicians requesting and obtaining protective custody for cancer treatment when the reported mortality rates are 40-50%. Such differences might be attributed to differences in care, a reasonable prioritization of quality of life over survival, or the role uncertainty plays on prognoses, especially for the extremely young. Nonetheless, other, non-medical factors may have a significant effect on inconsistencies in care across these pediatric subspecialties and require further examinations.
    Keywords:  Cardiology; Decision-making; End-of-life care; Neonatology; Oncology
    DOI:  https://doi.org/10.1007/s10730-019-09384-6
  9. Am J Transplant. 2019 Sep 17.
    Mao CY, Mahle WT.
      Pediatric heart transplant candidates continue to face high waitlist mortality compared to other pediatric solid organ transplant candidates. Furthermore, waitlist mortality for congenital heart disease (CHD) candidates is significantly higher than non-congenital heart disease candidates. (1) In an attempt to improve waiting list mortality for all pediatric heart candidates and reduce this disparity for CHD candidates, the Organ Procurement and Transplantation Network (OPTN) implemented new allocation policies in March 2016.
    DOI:  https://doi.org/10.1111/ajt.15596