bims-hylehe Biomed news
on Hypoplastic left heart syndrome
Issue of 2018‒11‒25
fourteen papers selected by
Richard James
University of Pennsylvania


  1. Expert Rev Cardiovasc Ther. 2018 Nov 20.
    Ntiloudi D, Zanos S, Gatzoulis MA, Karvounis H, Giannakoulas G.
      INTRODUCTION: Patients with congenital heart disease (CHD), who develop pulmonary arterial hypertension (PAH), live longer and have better quality of life compared to the past due to PAH specific therapy and improved tertiary care. Areas covered: Clinical examination, objective assessment of functional capacity, natriuretic peptide levels, cardiac imaging and haemodynamics all play a pivotal role in the evaluation, general care and management of PAH specific therapy. This review discusses the epidemiology and pathophysiology of PAH-CHD, and provides hints for the optimal evaluation of these patients. Expert commentary: Further research should be performed in the field of PAH-CHD, as there are many of areas lacking evidence that should be addressed in the future. Networking, especially among the tertiary expert centres, could play a key role in this direction.
    Keywords:  Congenital Heart Disease; Diagnosis; Epidemiology; Pathophysiology; Pulmonary Arterial Hypertension
    DOI:  https://doi.org/10.1080/14779072.2019.1550716
  2. Rev Port Cardiol. 2018 Nov 16. pii: S0870-2551(17)30802-8. [Epub ahead of print]
    Areias ME, Peixoto B, Santos I, Cruz L, Regadas A, Pinheiro C, Monteiro H, Araújo S, Carvalho T, Miranda J, Moura C, Soares J, Viana V, Quintas J, Areias JC.
      INTRODUCTION AND OBJECTIVES: The objectives of this study were to assess the neuropsychological performance (NP) of adolescents and young adults with congenital heart disease (CHD), comparing them with a group of healthy controls, to determine whether there are different neurocognitive phenotypes in CHD, and to identify their relation to sociodemographic, neonatal, clinical and psychological adjustment variables.METHODS: A total of 217 CHD patients (116 male, aged 15.73±2.68 years) and 80 controls (35 male, age 16.76±2.22 years) underwent an extensive neuropsychological assessment and analysis of psychological adjustment.
    RESULTS: CHD patients had significantly poorer NP than healthy controls in all neurocognitive domains. Three different phenotypes of NP in CHD patients were identified: non-impaired (NI), moderately impaired (MI) and globally impaired (GI). They differed in all dimensions of NP. The GI cluster showed fewer years of schooling (p=0.025) and lower neonatal indicators such as head circumference (p=0.019), 1-min Apgar score (p=0.006), birth weight (p=0.05) and length (p=0.034) than the NI cluster. In the MI and GI clusters, there were more cyanotic forms of disease, including tetralogy of Fallot and transposition of the great arteries. The GI cluster presented more difficulties with psychological adjustment, including social (p=0.038), attention (p=0.001) and aggressive (p=0.003) problems.
    CONCLUSIONS: CHD patients have poorer NP than controls. NP in the CHD group can be classified in three clusters that reflect different levels of neuropsychological functioning, which is sensitive to social, neonatal and psychological adjustment variables.
    Keywords:  Ajustamento psicossocial; Cardiopatia congénita; Congenital heart defects; Deficiências neurodesenvolvimentais; Mental health; Morbidade psiquiátrica; Neurodevelopmental disabilities; Psychiatric morbidity; Psychosocial adjustment; Qualidade de vida; Quality of life; Saúde mental
    DOI:  https://doi.org/10.1016/j.repc.2017.11.017
  3. Ann Thorac Surg. 2018 Nov 17. pii: S0003-4975(18)31668-0. [Epub ahead of print]
    McHugh KE, Mahle WT, Hall MA, Scheurer MA, Moga MA, Triedman J, Nicolson SC, Amula V, Cooper DS, Schamberger M, Wolf M, Shekerdemian L, Burns KM, Ash KE, Hipp DM, Pasquali SK, .
      BACKGROUND: The Pediatric Heart Network Collaborative Learning Study (PHN CLS) increased early extubation rates after infant Tetralogy of Fallot (TOF) and coarctation (CoA) repair across participating sites by implementing a clinical practice guideline (CPG). The impact of the CPG on hospital costs has not been studied.METHODS: PHN CLS clinical data were linked to cost data from Children's Hospital Association by matching on indirect identifiers. Hospital costs were evaluated across active and control sites in the pre- and post-CPG periods using generalized linear mixed effects models. A difference-in-difference approach was used to assess whether changes in cost observed in active sites were beyond secular trends in control sites.
    RESULTS: Data were successfully linked on 410/428 (96%) of eligible patients from 4 active and 4 control sites. Mean adjusted cost/case for TOF repair was significantly reduced in the post-CPG period at active sites ($42,833 vs. $56,304, p<0.01) and unchanged at control sites ($47,007 vs. $46,476, p=0.91), with an overall cost reduction of 27% in active vs. control sites (p=0.03). Specific categories of cost reduced in the TOF cohort included clinical (-66%, p<0.01), pharmacy (-46%, p=0.04), lab (-44%, p<0.01), and imaging (-32%, p<0.01). There was no change in costs for CoA repair at active or control sites.
    CONCLUSIONS: The early extubation CPG was associated with a reduction in hospital costs for infants undergoing repair of TOF, but not CoA repair. This CPG represents an opportunity to both optimize clinical outcome and reduce costs for certain infant cardiac surgeries.
    DOI:  https://doi.org/10.1016/j.athoracsur.2018.10.019
  4. Aust J Rural Health. 2018 Nov 19.
    O'Brien EM, Law D, Celermajer D, Grant PW, Waites J.
      OBJECTIVE: The number of adults with congenital heart disease has increased with improvements in surgical and medical management, posing a challenge for regional and rural settings, which might have difficulties accessing specialised professionals with congenital heart disease services. This study aims to ascertain the demographics and management of adults with congenital heart disease seen by a cardiology practice in regional Australia to better understand the needs of regional adults with congenital heart disease.DESIGN: A descriptive study using data from clinical notes collected between April 2013 and April 2016.
    SETTING: A private cardiology practice in Coffs Harbour, New South Wales. The practice has a treating cardiologist, senior sonographer, visiting cardiothoracic surgeon and visiting paediatric cardiologist.
    PARTICIPANTS: One-hundred-and-one adults with congenital heart disease (age: 16-88 years; 55 women).
    MAIN OUTCOME MEASURES: Congenital heart disease defect, lesion severity, referral reason, past surgery, pulmonary hypertension, cardiac surgery during the study, mortality, adherence with follow-up plans and specialist referral.
    RESULTS: Sixty-six patients had simple congenital heart disease, 24 had congenital heart disease of moderate complexity and 11 had congenital heart disease of great complexity. Most were referred for surveillance, seven were referred due to pregnancy and eight were new diagnoses. Six patients died, nine had cardiac operations and five were treated for pulmonary arterial hypertension. Overall adherence to the treating cardiologist's follow-up plans was 84%. All patients with congenital heart disease of great complexity were referred to congenital heart disease specialists.
    CONCLUSION: A substantial number of patients had complex pathology, new diagnoses or required surgery, highlighting the importance of developing integrated services with the close support of outside specialists in managing adults with congenital heart disease in regional settings.
    Keywords:  cardiac care facilities; congenital; delivery of health care; heart defects; referral and consultation; regional health
    DOI:  https://doi.org/10.1111/ajr.12446
  5. Congenit Heart Dis. 2018 Nov 19.
    Lapum JL, Fredericks S, Bailey B, Yau TM, Graham J, Marelli AJ.
      OBJECTIVE: The number of adults with congenital heart disease (CHD) has increased substantially because of medical advances that are extending life expectancy beyond childhood. A historical investigation is timely not only because of this significant demographic shift, but also because stakeholder engagement is increasingly important in shaping research directions. The study questions were: From the perspective of stakeholders, what is known about the medical treatment for the adult CHD cohort in Canada and how has it changed over time?DESIGN/METHODS: We harnessed the use of historical research methods and conducted interviews with 21 key informants in order to articulate a contextualized understanding of the evolution of adult CHD treatment in Canada. The informants recruited were currently or previously located in and/or involved in treatment in seven provinces and two territories across Canada including patients, families, advocates, researchers, and practitioners located in disciplines that included cardiology, cardiovascular surgery, nursing, psychology, dietetics, and kinesiology.
    RESULTS: Alongside findings that highlighted the significant demographic shift, the findings highlight key themes related to temporal shifts in treatment, emergence of organizational structures and use of evidence, comprehensive approaches to care, and future directions. A critical finding that requires immediate attention is the significantly disproportionate resources to the number of adults living with CHD, and as a result, the real risk of premature death for this population.
    CONCLUSIONS: The insights provided behoove the community of stakeholders to think creatively on how to draw attention to the inadequacy of resources and the unique and diverse needs of this population.
    Keywords:  adults; congenital heart disease; heart disease; historical methods; qualitative research
    DOI:  https://doi.org/10.1111/chd.12716
  6. Crit Care Nurs Clin North Am. 2018 Dec;pii: S0899-5885(18)30973-0. [Epub ahead of print]30(4): 457-466
    Gephart SM, Moore EF, Fry E.
      Although a unit-adopted standardized feeding protocol (SFP) for neonates is standard of care, implementation strategies for SFPs vary across neonatal and pediatric intensive care. Besides improving growth and reducing feeding interruptions, SFPs reduce risk for necrotizing enterocolitis in infants with heart disease or born premature. The purpose of this article is to bridge the gap between recommended and actual care using SFPs.
    Keywords:  Clinical decision support; Congenital heart disease; Implementation science; Necrotizing enterocolitis; Neonate; Standardized feeding protocol
    DOI:  https://doi.org/10.1016/j.cnc.2018.07.003
  7. J Pediatr Health Care. 2018 Nov 15. pii: S0891-5245(18)30200-1. [Epub ahead of print]
    Schniepp HE, Cassidy B, Godfrey K.
      INTRODUCTION: Infants with congenital heart disease can be critically ill; prolonged hospitalization is common. The purpose of this project was to create and then evaluate the efficacy of education programs for staff nurses and medical providers aimed at improving infant immunization administration during hospitalization for congenital heart disease.METHODS: This project used pre- and post-education program chart review to evaluate infant immunization rates at discharge. Pretests and posttests were administered to cardiac care staff to determine knowledge change. Paired t test, chi-square test, and descriptive statistics were used.
    RESULTS: The pretest and posttest surrounding the education programs indicated significant knowledge change for both groups. Immunization rates were improved before compared with after the education program (60% vs. 88%, respectively). There was significant improvement in immunization documentation on the discharge summary (p < .001).
    DISCUSSION: The findings suggest that customized education presentations to cardiac care staff can increase the administration of recommended immunizations to hospitalized infants and documentation on discharge summaries.
    Keywords:  Congenital heart disease; immunization; infant; pediatric critical care
    DOI:  https://doi.org/10.1016/j.pedhc.2018.08.003
  8. J Heart Lung Transplant. 2018 Oct 31. pii: S1053-2498(18)31731-5. [Epub ahead of print]
    Peng DM, Koehl DA, Cantor RS, McMillan KN, Barnes AP, McConnell PI, Jordan J, Andersen ND, St Louis JD, Maeda K, Kirklin JK, Kindel SJ.
      BACKGROUND: The reported ventricular assist device (VAD) experience in the pediatric congenital heart disease (CHD) population is limited. We sought to describe contemporary use and outcomes of VADs in children with CHD and compare these outcomes to those of non-CHD children.METHODS: Patients enrolled in the Pediatric Interagency Registry for Mechanical Circulatory Support (Pedimacs) between September 19, 2012 through June 30, 2017 were included. CHD was classified as biventricular vs single ventricle (Stages 1, 2, or 3). Outcomes were compared between groups and multivariable analysis was used to identify factors associated with mortality on the device.
    RESULTS: Among the 471 patients enrolled, 108 (24%) had CHD (45 biventricular and 63 single ventricle). CHD patients were younger (5.7 ± 5.7 years vs 9.8 ± 6.5 years; p < 0.0001) and smaller (0.8 ± 0.5 m2 vs 1.2 ± 0.7 m2; p < 0.0001) compared with non-CHD patients. CHD patients were more likely to receive a paracorporeal continuous-flow VAD (36.1% vs 12.9%; p < 0.0001) and less likely to receive an implantable continuous-flow VAD (27.8% vs 55.0%; p < 0.0001) compared with non-CHD patients. After 6 months on a VAD, CHD patients had higher mortality (36.4% vs 12.1%) and a lower transplantation rate (29.1% vs 59.9%) than non-CHD patients (p < 0.0001). In the multivariable analysis, CHD was the factor most strongly associated with mortality on VAD (hazard ratio [HR] = 2.9; p < 0.0001), whereas the factors implantable continuous-flow device and high-volume center were protective (HR = 0.3, p < 0.0001, and HR = 0.6, respectively; p = 0.02).
    CONCLUSIONS: VAD use in children with CHD is associated with increased mortality and decreased transplant rates compared to children without CHD. For the subgroup of children with CHD who received implantable continuous-flow VADs, survival rates were higher and comparable to those of children without CHD. Increased experience correlated with better survival in pediatric VADs.
    Keywords:  congenital heart disease; heart failure; heart transplantation; outcomes; pediatric heart failure; pediatric heart transplantation; pediatrics; ventricular assist device
    DOI:  https://doi.org/10.1016/j.healun.2018.10.008
  9. Heart Fail Clin. 2019 Jan;pii: S1551-7136(18)30074-6. [Epub ahead of print]15(1): 87-96
    Bradley EA, Saraf A, Book W.
      Heart failure remains the most common cause of morbidity and mortality in adults with congenital heart disease (CHD). Although gender-specific outcomes are not robust, it seems that women with CHD may be more affected by late heart failure (HF) than men. A specialized and experienced adult CHD team is required to care for these women as they age, including assessment for reversible causes of HF and in the management of pregnancy, labor, and delivery.
    Keywords:  Adult congenital heart disease; Heart failure; Pregnancy; Women
    DOI:  https://doi.org/10.1016/j.hfc.2018.08.009
  10. BMC Pediatr. 2018 Nov 19. 18(1): 359
    Kondo M, Ohishi A, Baba T, Fujita T, Iijima S.
      BACKGROUND: Delayed diagnosis of critical congenital heart disease (CCHD) carries a serious risk of mortality, morbidity, and handicap. As echocardiography is commonly used to diagnose congenital heart disease (CHD), echocardiographic investigations in newborns may be helpful in detecting CCHD earlier and with higher sensitivity than when using other screening methods. The present study aimed to evaluate the effectiveness of echocardiographic screening for CCHD in a tertiary care center.METHODS: A retrospective chart review was conducted among newborns delivered at Hamamatsu University Hospital between June 2009 and May 2016. The study included consecutive newborns who underwent early echocardiographic screening (within the first 5 days of life) performed by pediatric cardiologists, were born at ≥36 weeks of gestation, had a birthweight ≥2300 g, and were cared for in the well-baby nursery. Newborns admitted to the neonatal intensive care unit, as well as those with prenatal diagnosis of CHD and/or clinical symptoms or signs of CHD were excluded. Four CHD outcome categories were defined: critical, serious, clinically significant, and clinically non-significant.
    RESULTS: A total of 4082 live newborns were delivered during the study period. Of 3434 newborns who met the inclusion criteria and had complete echocardiography data, 104 (3.0%) were diagnosed as having CHD. Among these, none was initially diagnosed as having critical or serious CHD. Of the 95 newborns who continued follow-up with a cardiologist, 61 (64%) were determined to have non-significant CHDs that resolved within 6 months of life. Review of excluded newborns revealed nine cases of critical or serious CHD; among these newborns, six were diagnosed prenatally and three had some clinical signs of CHD prior to hospital discharge.
    CONCLUSIONS: In our tertiary care center, echocardiography screening within the first 5 days of life did not help improve CCHD detection rate in newborns without prenatal diagnosis or clinical signs of CHD. Echocardiographic screening may be associated with increased rate of false-positives (defects resulting in clinically non-significant CHDs) in newborns without prenatal diagnosis or suspicion of CHD.
    Keywords:  Critical congenital heart disease; Echocardiography; Newborn; Screening
    DOI:  https://doi.org/10.1186/s12887-018-1344-z
  11. J Clin Psychol Med Settings. 2018 Nov 20.
    Rodríguez-Rey R, Alonso-Tapia J.
      Research on parental psychological effects related to a child's critical illness has focused on studying negative outcomes, while the possibility of posttraumatic growth (PTG), defined as the perception of positive changes after a traumatic event, has been overlooked. This study explores the degree of parental PTG after a child's hospitalization in a pediatric intensive care unit (PICU) and the role of resilience, emotions, perceived severity of the child's condition and stress in predicting PTG. In the first 48 h after their child's discharge from a PICU, N = 196 parents were assessed for resilience, emotions, perceived stress, and the degree to which they perceived their child's condition as severe. 6 months later N = 143 parents were assessed PTG. 6 months post discharge, 37.1% of parents reported PTG at least to a medium degree. Path analyses with latent variables showed that the psychological variables assessed at discharge predicted between 20 and 21% of the total variance in PTG. Resilience affected PTG indirectly, through the bias of positive emotions. PTG is a frequent phenomenon. Psychological interventions aimed at encouraging parental PTG after a child's critical admission should focus on boosting resilience and positive emotions.
    Keywords:  Critically ill children; Parent emotions; Parent stress; Parents; Pediatric intensive care unit; Posttraumatic growth; Resilience
    DOI:  https://doi.org/10.1007/s10880-018-9594-3
  12. J Surg Res. 2018 Dec;pii: S0022-4804(18)30455-4. [Epub ahead of print]232 298-307
    Miller R, Tumin D, Tobias JD, McKee C.
      BACKGROUND: As management of congenital heart disease (CHD) improves, children with CHD increasingly present for noncardiac surgery. Prior studies report conflicting results on the association between CHD and adverse outcomes in noncardiac surgery. Studies reporting no such association predominantly analyze older children and adolescents. We evaluated whether the association between CHD and adverse surgical outcomes was attenuated by increased age.METHODS: Patients aged 0-17 y, undergoing elective noncardiac surgery, were identified in the National Surgical Quality Improvement Program-Pediatric registry. CHD status was classified as none, minor, major, or severe. Multivariable logistic regression evaluated the association of CHD status with the occurrence of any postoperative complication for groups defined by quintiles of patient age (<1, 1-3, 4-8, 9-13, and 14-17 y). Specific complications included cardiac arrest, reintubation, infection, renal failure, neurological complication, thromboembolic complication, reoperation, 30-d unplanned revisit, 30-d prolonged hospital stay, and mortality.
    RESULTS: The analysis included 131,164 children, of whom 6420 had minor CHD, 3825 had major CHD, and 963 had severe CHD. The overall rate of complications was 9%. In multivariable analysis, children <1 y old had greater risk of postoperative complications if they had minor (odds ratio [OR] = 1.97; 95% confidence interval [CI]: 1.70, 2.20; P < 0.001), major (OR = 2.58; 95% CI: 2.28, 2.91; P < 0.001), or severe CHD (OR = 4.37; 95% CI: 3.45, 5.54; P < 0.001). In older age groups, however, the presence of CHD was not independently associated with postoperative complications.
    CONCLUSIONS: In pediatric noncardiac surgery, an independent association of CHD with postoperative complications was only evident among children <1 y old.
    Keywords:  NSQIP-Peds; Risk stratification
    DOI:  https://doi.org/10.1016/j.jss.2018.06.050
  13. Prog Cardiovasc Dis. 2018 Sep - Oct;61(3-4):pii: S0033-0620(18)30183-X. [Epub ahead of print]61(3-4): 273-274
    Krasuski RA.
      
    DOI:  https://doi.org/10.1016/j.pcad.2018.09.001
  14. J Pediatr Psychol. 2018 Nov 17.
    Abda A, Bolduc ME, Tsimicalis A, Rennick J, Vatcher D, Brossard-Racine M.
      Objective: Over the past 20 years, there has been a growing interest in the psychosocial outcomes of children and adolescents born with a congenital heart defect (CHD). This systematic review and meta-analysis aims to appraise and synthesize current literature on the psychosocial outcomes of children and adolescents with severe CHD.Methods: A search of studies examining psychosocial outcomes in children and adolescents with severe CHD was performed. Meta-analyses were used to calculate the prevalence of psychosocial impairments and the standardized mean differences between cases and controls. Results that were not included in the meta-analysis were collated using descriptive statistics.
    Results: A total of 16 studies were included in this review, and results were summarized according to three domains: behavior, social cognition, and self-esteem. Results from a meta-analysis identified that 25% of children and adolescents with CHD presented behavioral problems. Children and adolescents with CHD had more problematic behavior than healthy peers (standardized mean difference; g = 0.71). Young people with CHD had significantly more difficulties inferring on the mental states of others (standardized mean difference; g = 0.72). In contrast, identifying the emotions of others and self-esteem was not statistically different from healthy controls.
    Conclusion: This review and meta-analysis provides evidence to support the presence of psychosocial difficulties in children and adolescents born with a severe CHD giving grounds for the systematic assessment of behavior and social cognition during their clinical follow-up.
    DOI:  https://doi.org/10.1093/jpepsy/jsy085