bims-hylehe Biomed News
on Hypoplastic left heart syndrome
Issue of 2019‒07‒28
nineteen papers selected by
Richard James
University of Pennsylvania

  1. Congenit Heart Dis. 2019 Jul 22.
    Peterson JK, Setty SP, Knight JH, Thomas AS, Moller JH, Kochilas LK.
      OBJECTIVE: Patients with Trisomy 21 (T21) and single ventricle (SV) physiology present unique challenges compared to euploidic counterparts. This study reports postoperative and long-term outcomes in patients with T21 and SV palliation.DESIGN: This retrospective cohort study from the Pediatric Cardiac Care Consortium (PCCC) included patients with T21 (<21 years old) that underwent surgical palliation for SV between 1982 and 2008 and control patients without known genetic anomaly following Fontan palliation for similar diagnoses. Kaplan-Meier survival plots were created based on death events obtained from the PCCC and by linkage with the National Death Index (NDI) and the Organ Procurement and Transplantation Network (OPTN) through 2014 for patients with adequate identifiers.
    RESULTS: We identified 118 children with T21 who underwent initial surgical SV palliation. Among 90 (75.6%) patients surviving their first surgery, 66 (73.3%) underwent Glenn anastomosis and 25 (27.8%) completed Fontan palliation with in-hospital survival of 80.3% and 76.0%, respectively. Fifty-three patients had sufficient identifiers for PCCC-NDI-OPTN linkage. Ten-year survival, conditioned on discharge alive after the Fontan procedure, was 66.7% compared to 92.2% for 51 controls without genetic anomaly (P = .001). Median age at death for T21 patients following initial surgical SV palliation was 2.69 years (IQR 1.34-7.12) with most deaths (89.2%) attributed to the underlying congenital heart disease (CHD).
    CONCLUSIONS: Children with T21 and SV are at high risk for procedural and long-term mortality related to their genetic condition and underlying CHD. Nevertheless, a select group of patients can successfully complete Glenn or Fontan palliation, reaching satisfactory long-term survival.
    Keywords:  Down syndrome; congenital; heart defects; retrospective studies
  2. Ann Thorac Surg. 2019 Jul 16. pii: S0003-4975(19)31035-5. [Epub ahead of print]
    Parker DM, Everett AD, Stabler ME, Vricella L, Jacobs ML, Jacobs JP, Parikh CR, Pasquali SK, Brown JR.
      BACKGROUND: To evaluate the association between preoperative biomarker levels and 365-day readmission or mortality after pediatric congenital heart surgery.METHODS: Children aged 18 years or younger undergoing congenital heart surgery (n=145) at Johns Hopkins Hospital from 2010-2014 were enrolled in the prospective cohort. Novel biomarkers ST2, Galectin-3, NT-proBNP, and GFAP were measured. The composite study endpoint was unplanned readmission within 365 days following discharge or mortality either in-hospital during the surgical admission or within 365 days after discharge. A clinical model based on covariates used in the STS Congenital Heart Surgery Database Mortality Risk Model and an augmented model using the clinical model in conjunction with a novel biomarker panel were evaluated.
    RESULTS: Readmission or mortality within 365-days of surgery occurred among 39 (27%) pediatric patients. The clinical model alone resulted in a c-statistic of 0.719 (95% CI:0.63 - 0.81). The clinical model in conjunction with the log-transformed biomarkers improved the c-statistic to 0.805 (95% CI:0.73 - 0.88). The addition of biomarkers resulted in a significant improvement to the clinical model alone (p value: 0.035).
    CONCLUSIONS: Novel biomarkers may add predictive value when assessing the likelihood of 365-day readmission or mortality after pediatric congenital heart surgery. After adjusting for clinical and novel biomarkers, pre- and post-operative ST2 remained associated with 365-day readmission or mortality. The current STS clinical congenital mortality risk model can be applied to identify children with increased risk of repeat hospitalizations and post-discharge mortality and may inform preventative care interventions that aim to reduce these adverse events.
  3. J Cardiothorac Vasc Anesth. 2019 Jun 15. pii: S1053-0770(19)30568-3. [Epub ahead of print]
    Brown ML, DiNardo JA, Nasr VG.
      The incidence of moderate to severe congenital heart disease (CHD) in the United States is estimated to be 6 per 1,000 live-born, full-term infants. Recent advances in pediatric cardiology, surgery, and critical care have improved significantly the survival rates of patients with CHD leading to an increase in prevalence in both children and adults. Children with CHD significant enough to require cardiac surgery frequently also undergo noncardiac surgical procedures. With this increased demand for procedures that require anesthesia, all anesthesiologists, and more specifically, pediatric anesthesiologists will encounter patients with repaired or unrepaired CHD and other cardiac diseases in their practice. They often are faced with the question, "Is this patient too high risk for anesthesia?" The objective of this literature review is to provide a greater understanding of patients at high risk and to quantify the risk for patients, their families, and clinicians. In addition, specific high-risk lesions (single ventricle, Williams-Beuren syndrome, pulmonary hypertension, cardiomyopathies, and ventricular assist devices) are described.
    Keywords:  congenital heart disease; noncardiac; pediatric; pediatric cardiac anesthesia; risk assessment
  4. Handb Clin Neurol. 2019 ;pii: B978-0-444-64029-1.00016-3. [Epub ahead of print]162 329-345
    Liamlahi R, Latal B.
      Congenital heart disease (CHD) constitutes the most common congenital malformation, with moderate or severe CHD occurring in around 6 in 1000 live births. Due to advances in medical care, survival rates have increased significantly. Thus, the majority of children with CHD survive until adolescence and adulthood. Children with CHD requiring cardiopulmonary bypass surgery are at risk for neurodevelopmental impairments in various domains, including mild impairments in cognitive and neuromotor functions, difficulties with social interaction, inattention, emotional symptoms, and impaired executive function. The prevalence for these impairments ranges from 20% to 60% depending on age and domain ("high prevalence-low severity"). Domains are often affected simultaneously, leading to school problems with the need for learning support and special interventions. The etiology of neurodevelopmental impairments is complex, consisting of a combination of delayed intrauterine brain development and newly occurring perioperative brain injuries. Mechanisms include altered intrauterine hemodynamic flow as well as neonatal hypoxia and reduced cerebral blood flow. The surgical procedure and postoperative phase add to this cascade of factors interfering with normal brain development. Early identification of children at high risk through structured follow-up programs is mandated to provide individually tailored early interventions and counseling to improve developmental health.
    Keywords:  Brain development; Brain injury; Cardiopulmonary bypass surgery; Congenital heart disease; Development; Executive function; Intelligence; Motor; Outcome; Risk factors; Stroke; White matter injury
  5. Acta Paediatr. 2019 Jul 23.
    Cloete E, Bloomfield FH, Cassells SA, de Laat MWM, Sadler L, Gentles TL.
      AIM: Assess the potential additional benefit from pulse oximetry screening in the early detection of critical congenital heart disease in a country with a well-developed antenatal ultrasound screening programme.METHODS: Live-born infants, pregnancy terminations and stillbirths from 20 weeks' gestational age, between 2013 and 2015, with critical cardiac defects defined as primary or secondary targets of pulse oximetry screening were identified. Critical defects were those resulting in the death of a fetus or an infant in the first 28 days after birth, or a defect requiring intervention in the first 28 days.
    RESULTS: 268 infants and fetuses were identified. Antenatal detection rates improved from 69% to 77% over the study period. An associated co-morbidity improved antenatal detection rates. Twenty-seven live-born infants were diagnosed after discharge: 15 aortic arch obstruction (AAO); 10 d-loop transposition of the great arteries (d-TGA), and 2 total anomalous pulmonary venous drainage (TAPVD). Of these, 5 with AAO, 9 with d-TGA and likely both with TAPVD could potentially have been detected with oximetry screening.
    CONCLUSION: The antenatal detection of critical cardiac anomalies continues to improve in New Zealand. Despite high antenatal detection rates for most lesions, universal postnatal oximetry screening has the potential to improve early detection. This article is protected by copyright. All rights reserved.
    Keywords:  Congenital heart disease; newborn; screening strategies
  6. Curr Treat Options Cardiovasc Med. 2019 Jul 25. 21(9): 42
    Grodzinsky A, Florio K, Spertus JA, Daming T, Schmidt L, Lee J, Rader V, Nelson L, Gray R, White D, Swearingen K, Magalski A.
      PURPOSE OF REVIEW: Maternal mortality in the United States is highest among all developed nations and continues to rise. The leading cause of morbidity and mortality during pregnancy and the postpartum period is cardiovascular disease. While there are large European and Canadian studies exploring the care and outcomes of moms with heart disease in pregnancy, there are no large prospective studies to guide the care of this growing group of patients in the US. We review the current approach to the management of patients with heart disease in pregnancy and the gaps in knowledge thereof.RECENT FINDINGS: Currently, antenatal management and delivery planning are highly variable for patients with heart disease in pregnancy and maternal risk models' application to the US patient population is limited by their derivation from an international cohort of patients and their focus on patients with congenital heart disease. As the need for interdisciplinary care between cardiologists and obstetricians becomes evident, and as broad research efforts within this space are very much needed, we propose a research collaborative called the Heart Outcomes in Pregnancy: Expectations (HOPE) for Mom and Baby Registry. The HOPE Registry aims to address key clinical questions surrounding the preconception period, antenatal care, delivery planning and outcomes, and long-term postpartum care and outcomes of these unique patients. We have made progress in recent years by recognizing the clinical need to address and standardize the management of patients with heart disease in pregnancy. We now must initiate and propel US-based cardio-obstetrics research to address key gaps in knowledge and variability in the care of patients with heart disease in pregnancy.
    Keywords:  Cardio-obstetrics; Heart disease in pregnancy
  7. J Pediatr Psychol. 2019 Apr 26. pii: jsz026. [Epub ahead of print]
    Bishop MN, Gise JE, Donati MR, Shneider CE, Aylward BS, Cohen LL.
      OBJECTIVE: Parents of children with chronic medical needs report increased parenting challenges, poor sleep, and maladjustment. The impact of parenting stress on both sleep and adjustment has yet to be evaluated for parents of infants and young children with congenital heart disease (CHD). We studied the relations among parenting stress, sleep, and adjustment in parents of infants and toddlers with CHD. We expected that sleep quality would mediate the relationship between parenting stress and adjustment.METHODS: Sixty-nine parents of infants and toddlers with CHD were evaluated on self-report measures of illness-related parenting stress (Pediatric Inventory for Parents), sleep (Pittsburgh Sleep Quality Index), and psychological adjustment (Brief Symptom Index-18).
    RESULTS: The parents of infants and toddlers with CHD reported elevated levels of parenting stress, sleep problems, and maladjustment. The positive relationship between parenting stress and parent maladjustment was mediated by sleep quality.
    CONCLUSIONS: Findings suggest that parents of infants and toddlers with CHD report high parenting stress, poor sleep, and maladjustment. Analyses indicate the stress-adjustment relationship is mediated by quality of sleep. Given the multiple demands on parents of infants and children with CHD, it is important to attend to parents' overall functioning and mental health. Our findings highlight targets for intervention to improve the well-being of parents of young children with CHD.
    Keywords:  infancy; parent stress; psychosocial functioning; sleep
  8. J Cardiothorac Vasc Anesth. 2019 Jun 13. pii: S1053-0770(19)30522-1. [Epub ahead of print]
    Grogan KL, Goldsmith MP, Masino AJ, Nelson O, Tsui FC, Simpao AF.
      Congenital heart disease (CHD) is one of the most common birth anomalies, and the care of children with CHD has improved over the past 4 decades. However, children with CHD who undergo general anesthesia remain at increased risk for morbidity and mortality. The proliferation of electronic health record systems and sophisticated patient monitors affords the opportunity to capture and analyze large amounts of CHD patient data, and the application of novel, effective analytics methods to these data can enable clinicians to enhance their care of pediatric CHD patients. This narrative review covers recent efforts to leverage analytics in pediatric cardiac anesthesia and critical care to improve the care of children with CHD.
    Keywords:  analytics; cardiac anesthesia; cardiac critical care; machine learning; pediatrics
  9. Ann Thorac Surg. 2019 Jul 20. pii: S0003-4975(19)31036-7. [Epub ahead of print]
    Jacobs ML, Jacobs JP, Hill KD, O'Brien SM, Pasquali SK, Vener D, Kumar SR, Chiswell K, St Louis JD, Mayer JE, Habib RH, Shahian DM, Fernandez FG.
      As the largest congenital and pediatric cardiac surgical clinical data registry in the world, the Society of Thoracic Surgeons Congenital Heart Surgery Database (STS CHSD) serves as a platform for reporting of outcomes and for quality improvement. In addition, it is an important source of data for clinical research and for innovations related to quality measurement. Each year, several teams of investigators undertake analyses of data in the STS CHSD pertaining to the surgical management of specific diagnostic and procedural groups, as well as specific processes of care and their associations with patient characteristics across centers participating in the STS CHSD, and with outcomes. Additional ongoing projects involve the development of new or refined metrics for quality measurement and reporting of outcomes and center level performance. The STS, through its Workforce for National Databases and the STS Research Center and Workforce on Research Development provides multiple pathways through which investigators may propose and carry out outcomes research projects based on STS CHSD data. This article reviews research published within the past year.
  10. Pediatr Cardiol. 2019 Jul 25.
    Agrawal A, Swaminathan S, Guvvala SL, Gorla SR.
      Pediatric cardiology and cardiovascular surgery have witnessed significant advancements over the last two decades. In spite of this progress, congenital heart disease (CHD) still remains as one of the major causes of death in infants and young children in the United States. Many patient-related and patient-independent factors influence the outcomes in patients with CHD, one of which is the geographical location. In the US-Mexico border, management and outcomes of patients with CHD are further complicated by additional problems stemming from complex interplay between two different health systems, and socioeconomic disparities. In this article, the authors evaluate the various interplaying factors and describe the difficulties facing the practicing pediatric cardiologists in a US-Mexico border city.
    Keywords:  Congenital heart disease; Pediatric cardiology; US–Mexico border
  11. Paediatr Perinat Epidemiol. 2019 Jul;33(4): 291-309
    Best KE, Vieira R, Glinianaia SV, Rankin J.
      BACKGROUND: The impact of socio-economic status (SES) on congenital heart disease (CHD)-related mortality in children is not well established.OBJECTIVES: We aimed to systematically review and appraise the existing evidence on the association between SES (including poverty, parental education, health insurance, and income) and mortality among children with CHD.
    DATA SOURCES: Seven electronic databases (Medline, Embase, Scopus, PsycINFO, CINAHL, ProQuest Natural, and Biological Science Collections), reference lists, citations, and key journals were searched.
    STUDY SELECTION AND DATA EXTRACTION: We included articles reporting original research on the association between SES and mortality in children with CHD if they were full papers published in the English language and regardless of (a) timing of mortality; (b) individual or area-based measures of SES; (c) CHD subtype; (d) age at ascertainment; (e) study period examined. Screening for eligibility, data extraction, and quality appraisal were performed in duplicate.
    SYNTHESIS: Meta-analyses were performed to estimate pooled ORs for in-hospital mortality according to health insurance status.
    RESULTS: Of 1388 identified articles, 28 met the inclusion criteria. Increased area-based poverty was associated with increased odds/risk of postoperative (n = 1), neonatal (n = 1), post-discharge (n = 1), infant (n = 1), and long-term mortality (n = 2). Higher parental education was associated with decreased odds/risk of neonatal (n = 1) and infant mortality (n = 5), but not with long-term mortality (n = 1). A meta-analysis of four US articles showed increased unadjusted odds of in-hospital mortality in those with government/public versus private health insurance (OR 1.40, 95% CI 1.24, 1.56). The association between area-based income and CHD-related mortality was conflicting, with three of eight articles reporting significant associations.
    CONCLUSION: This systematic review provides evidence that children of lower SES are at increased risk of CHD-related mortality. As these children are over-represented in the CHD population, interventions targeting socio-economic inequalities could have a large impact on improving CHD survival.
    Keywords:  Congenital heart defects; mortality; poverty; socioeconomic deprivation; survival; systematic review
  12. J Clin Nurs. 2019 Jul 20.
    Coyne I, Sheehan A, Heery E, While AE.
      AIM AND OBJECTIVES: To examine the needs and perspectives regarding healthcare transition for adolescents and young adults with the following long-term conditions: diabetes, cystic fibrosis, and congenital heart disease.BACKGROUND: Transition of adolescents and young adults within healthcare services has become increasingly important as more children are surviving into adulthood with long-term conditions. Yet limited empirical evidence exists regarding transition experiences.
    DESIGN: Qualitative study fulfilling the completed consolidated criteria for reporting qualitative studies (COREQ) criteria (see supplementary File 1).
    METHODS: Semi-structured interviews with adolescents and young adults aged 14-25 years (n=47), parents (n=37) and health professionals (n=32) which was part of a larger mixed-methods study. Sample was recruited from two children's hospitals and four general hospitals in Ireland.
    RESULTS: Transfer occurred between the ages of 16 to early 20s years depending on the service. None of the hospitals had a transition policy and transition practices varied considerably. Adolescents worried about facing the unknown, communicating and trusting new staff and self-management. The transition process was smooth for some young adults, whilst others experienced a very abrupt transfer. Parents desired greater involvement in the transition process with some perceiving a lack of recognition of the importance of their role. In paediatric services, nurses reported following-up adolescents who struggled with treatment adherence and clinic attendance. Whereas after transfer, little effort was made to engage young adults if there were lapses in care, as this was generally considered the young adults' prerogative.
    CONCLUSIONS: The amount of preparation and the degree to which the shift in responsibility had occurred prior to transition appeared to influence successful transition for adolescents and young adults and their parents.
    RELEVANCE TO CLINICAL PRACTICE: Nurses in collaboration with the multidisciplinary team can help adolescents and young adults develop their self-management skills and guide parents on how to relinquish responsibility gradually prior to transition. This article is protected by copyright. All rights reserved.
    Keywords:  Transitional care; adolescents; chronic illness; experiences; parents; qualitative study; young adults
  13. Eur J Prev Cardiol. 2019 Jul 26. 2047487319865055
    Westhoff-Bleck M, Winter L, Aguirre Davila L, Herrmann-Lingen C, Treptau J, Bauersachs J, Bleich S, Kahl KG.
      OBJECTIVE: The purpose of this study was the diagnostic evaluation of the hospital anxiety and depression scale total score, its depression subscale and the Beck depression inventory II in adults with congenital heart disease.METHODS: This cross-sectional study evaluated 206 patients with congenital heart disease (mean age 35.3 ± 11.7 years; 58.3% men). Major depressive disorder was diagnosed by a structured clinical interview for the Diagnostic and Statistical Manual of Mental Disorders IV and disease severity with the Montgomery-Åsberg depression rating scale. Receiver operating characteristics provided assessment of diagnostic accuracy. Youden's J statistic identified optimal cut-off points.
    RESULTS: Fifty-three participants (25.7%) presented with major depressive disorder. Of these, 28 (52.8%) had mild and 25 (47.2%) had moderate to severe symptoms. In the total cohort, the optimal cut-off of values was >11 in the Beck depression inventory II, >11 in the hospital anxiety and depression scale and >5 in the depression subscale. Optimal cut-off points for moderate to severe major depressive disorder were similar. The cut-offs for mild major depressive disorder were lower (Beck depression inventory II >4; hospital anxiety and depression scale >8; >2 in its depression subscale). In the total cohort the calculated area under the curve varied between 0.906 (hospital anxiety and depression scale) and 0.93 (Beck depression inventory II). Detection of moderate to severe major depressive disorder (area under the curve 0.965-0.98) was excellent; detection of mild major depressive disorder (area under the curve 0.851-0.885) was limited. Patients with major depressive disorder had a significantly lower quality of life, even when they had mild symptoms.
    CONCLUSION: All scales were excellent for detecting moderate to severe major depressive disorder. Classification of mild major depressive disorder, representing 50% of cases, was limited. Therapy necessitating loss of quality of life is already present in major depressive disorder with mild symptoms. Established cut-off points may still be too high to identify patients with major depressive disorder requiring therapy. External validation is needed to confirm our data.
    Keywords:  Depression; adult congenital heart disease; self-rating scale; validation
  14. J Fam Nurs. 2019 Jul 25. 1074840719864255
    Burström Å, Acuña Mora M, Öjmyr-Joelsson M, Sparud-Lundin C, Rydberg A, Hanseus K, Frenckner B, Nisell M, Moons P, Bratt EL.
      Transfer to adult care for adolescents with chronic conditions ought to be determined by transition readiness. The aims of this study were (a) to describe the level of readiness for transition in adolescents with congenital heart disease, (b) to compare adolescents' assessment of transition readiness with their parents' assessments, and (c) to study potential correlates of transition readiness. A total of 157 triads of adolescents aged 14 to 18 years and their parents completed the Readiness for Transition Questionnaire. Adolescents scored higher on overall readiness than their parents. Multivariable analyses revealed that higher levels of adolescents' overall readiness were associated with a less threatening view of the illness, a higher level of empowerment, and with higher mothers' and fathers' overall readiness scores. Adolescents' responsibility scores were positively associated with age and parental adolescent responsibility scores. Parental involvement scores were negatively associated with adolescents' age and positively with the mothers' parental involvement scores. By using a triadic evaluation, the results of the present study significantly extend what is currently known about this population.
    Keywords:  adolescents; congenital heart disease; parents; readiness for transition
  15. J Midwifery Womens Health. 2019 Jul;64(4): 421-426
    Withrow E, Fussman C, Thompson K, Kleyn M.
      INTRODUCTION: In Michigan, pulse oximetry screening rates for critical congenital heart defects (CCHDs) are assessed for birthing hospitals but have not been assessed for the midwife-attended births that occur in the out-of-hospital birth community. This analysis was conducted to determine pulse oximetry screening rates among the midwife-attended out-of-hospital birth community in Michigan overall, and among midwives provided with loaned pulse oximeters from the Michigan Department of Health and Human Services (MDHHS).METHODS: Records for midwife-attended out-of-hospital births between April 1, 2014, and December 31, 2016, were linked via probabilistic matching with newborn screening records. Pulse oximetry screening rates were calculated for the midwife-attended out-of-hospital birth population overall, by midwife, and stratified by receipt of loaned pulse oximeters from MDHHS. Births from midwives who attended 5 or more nonhospital births during the study period were included in this analysis.
    RESULTS: Of the 3410 midwife-attended out-of-hospital births, 20.8% (n = 710) reported as having received a pulse oximetry screening for CCHDs. For births attended by midwives who received pulse oximeters from MDHHS, 50.5% had pulse oximetry screening results reported, compared with 12.7% among births attended by midwives without a loaned pulse oximeter. Of the 78 total midwives, 18% (n = 14) reported pulse oximetry screening results on more than half of the births they attended. Of the 14 midwives who received a pulse oximeter from MDHHS, 50.0% (n = 7) reported screening results for more than half of all births they attended.
    DISCUSSION: Our findings indicate that CCHD screening rates are low among midwife-attended out-of-hospital birth community. Screening rates were higher among midwives who received a pulse oximeter from MDHHS, but fewer than half of the attended births had a reported pulse oximetry screening. Further discussions with the midwife-attended out-of-hospital birth community to better understand screening barriers may be beneficial.
    Keywords:  congenital; heart defects; home childbirth; midwifery; oximetry
  16. J Cardiopulm Rehabil Prev. 2019 Jul 22.
    Li X, Chen N, Zhou X, Yang Y, Chen S, Song Y, Sun K, Du Q.
      BACKGROUND: Evidence from many studies demonstrates that regular exercise has a favorable effect on cardiovascular disease, but it is still unclear whether adults with congenital heart disease (ACHD) benefit from exercise training. The aim of this study was to assess the effects of exercise training on ACHD.METHODS: PubMed, EMBASE, the Cochrane Library, Cumulative Index to Nursing and Allied Health, and Web of Science databases were searched. Differences were expressed using mean difference (MD) with 95% CI. The statistical analysis was performed using Review Manager (RevMan 5.3). Outcomes, including cardiorespiratory fitness, neurohumoral activation (NT-proBNP levels), rating of perceived exertion (Borg scale), and safety status, were investigated.
    RESULTS: Nine trials with 403 ACHD were included. The following outcomes were statistically significant: peak (MD = 1.96, 95% CI, 0.70-3.23; P = .002); maximal workload (MD = 11.46, 95% CI, 7.06-15.87; P < .00001); and maximal exercise duration (MD = 2.04, 95% CI, 1.00-3.07; P = .0001). But no significant decrease was reported regarding neurohumoral activation or the rating of perceived exertion. Furthermore, no major adverse events were reported. The overall quality of evidence ranged from moderate to very low.
    CONCLUSIONS: Evidence was underpowered to suggest that exercise training is effective in the management of ACHD. Future studies with longer follow-up are needed.
  17. Curr Opin Pediatr. 2019 Jul 17.
    Kaufman BD, Cohen HJ.
      PURPOSE OF REVIEW: Advanced heart failure in children is characterized by dynamic clinical trajectories, uncertainty of prognosis, and intermittent need for difficult decision-making, often related to novel therapeutic interventions with uncertain impact on quality of life. This review will examine the current role of palliative care to support this unique population.RECENT FINDINGS: Pediatric heart failure patients commonly die in ICUs with high burden of invasive therapies together with end of life care needs. In addition, several studies advocate for integration of palliative care early in disease trajectory, not only focused on end of life care. Many advocate for the core tenets of palliative care (symptom management, communication of prognosis, and advanced care planning) to be provided by the primary cardiology team, with consultation by pediatric palliative care specialists. There is also a consensus that palliative care training should be incorporated into pediatric advanced heart disease training programs.
    SUMMARY: Palliative care is an important component of pediatric heart failure care. Research and quality improvement efforts are needed to determine the most effective palliative care interventions for children with advanced heart disease. Provision of palliative care is an essential component of training for pediatric heart failure and transplant specialists.
  18. PLoS One. 2019 ;14(7): e0220136
    Bellander T, Karlsson AM.
      In this article, patient activity in 8 audio recorded specialist consultations on fetal cardiology is investigated in order to explore how, why and when patients tend to participate in encounters in which the doctor dominates the interaction. The overall question is: How can the participation of patients in the consultations be connected to the development of higher levels of health literacy, i.e. to interactive literacy and to critical literacy? Patient participation is here understood as interactive action and is analyzed in terms of different interactive moves, which are related to different recurring topics. Despite the highly standardized format of the consultations, there is a large variation between the patients' participation: between 0.7 and 2.8 moves per minute. The patients participate most during the topics 'Prevalence' and 'Consultations' and least during the topic 'The normal heart'. Although most of the patients' moves are responses to what the doctor says, they remarkably often pose questions and use so called rejoinders. By posing questions, they take control of the information flow and sometimes even change the topics. By using rejoinders, they analyze the problems involved in the discussion e.g. by asking for clarifications or confirmation. Patients with a low over-all participation rate also use fewer moves that indicate higher literacy levels. The qualitative analysis problematizes the idea of a simple scale from basic literacy to critical literacy. Moves that indicate basic literacy skills are interactively important for the learning activity, led by the doctor. However, patients who mainly support the doctor's initiatives don't take the opportunity to influence the flow of information in ways that might favor their health literacy development.
  19. Heart. 2019 Jul 26. pii: heartjnl-2019-314977. [Epub ahead of print]
    Wang F, Harel-Sterling L, Cohen S, Liu A, Brophy JM, Paradis G, Marelli AJ.
      To summarise existing heart failure (HF) risk prediction models and describe the risk factors for HF-related adverse outcomes in adult patients with congenital heart disease (CHD). We performed a systematic search of MEDLINE, EMBASE and Cochrane databases from January 1996 to December 2018. Studies were eligible if they developed multivariable models for risk prediction of decompensated HF in adult patients with CHD (ACHD), death in patients with ACHD-HF or both, or if they reported corresponding predictors. A standardised form was used to extract information from selected studies. Twenty-five studies met the inclusion criteria and all studies were at moderate to high risk of bias. One study derived a model to predict the risk of a composite outcome (HF, death or arrhythmia) with a c-statistic of 0.85. Two studies applied an existing general HF model to patients with ACHD but did not report model performance. Twenty studies presented predictors of decompensated HF, and four examined patient characteristics associated with mortality (two reported predictors of both). A wide variation in population characteristics, outcome of interest and candidate risk factors was observed between studies. Although there were substantial inconsistencies regarding which patient characteristics were predictive of HF-related adverse outcomes, brain natriuretic peptide, New York Heart Association class and CHD lesion characteristics were shown to be important predictors. To date, evidence in the published literature is insufficient to accurately profile patients with ACHD. High-quality studies are required to develop a unique ACHD-HF prediction model and confirm the predictive roles of potential risk factors.
    Keywords:  cardiac risk factors and prevention; congenital heart disease; heart failure; systemic review