bims-hylehe Biomed News
on Hypoplastic left heart syndrome
Issue of 2019‒10‒06
nine papers selected by
Richard James
University of Pennsylvania


  1. Int J Cardiol. 2019 Sep 11. pii: S0167-5273(19)32432-5. [Epub ahead of print]
    Moroney E, Zannino D, Cordina R, Gentles T, d'Udekem Y, Zentner D.
      BACKGROUND: Pregnancy in women with a Fontan circulation is understood to have a significantly increased risk of maternal morbidity and mortality. Potential longer-term effects on health outcomes remain unknown.AIM: Ascertainment of adverse events, post-delivery, in women from the Australia and New Zealand (ANZ) Fontan Registry. Comparator data from women without pregnancy and men was utilised.
    METHODOLOGY/RESULTS: Living adults were identified (n = 263 women, 280 men) with Registry analysis ascertaining cardiac morphology, type of Fontan procedure, pregnancy and significant cardiac adverse events. Data are described descriptively for morphology and as median (inter quartile range) for other variables. Thirty women reported 45 pregnancies beyond 20 weeks' gestation. Despite being older, these women, pre-delivery, had the lowest cardiac morbidity burden, compared to both male and female controls (OR = 3.25 (95% CI 1.44-8.35, p = 0.008 and OR = 2.59 (95% CI 1.14-6.70), p = 0.03). These differences were not present post-delivery. Median follow-up time post-delivery was 3.6 (1.2-7.5) years. Thrombus documentation and thromboembolic events were more common post-delivery, although differences were not significant in a propensity analysis model (adjusting for post-Fontan follow-up time, age and type of Fontan). There were no differences in arrhythmia, cardioversion, heart transplant, Fontan conversion or mortality between women post-delivery and either male or female controls.
    CONCLUSION: The apparent increase in thromboembolic events post pregnancy requires further investigation in a larger group of women over a longer period of follow-up time. This may have potential implications for post-partum anticoagulation, and for future health outcomes.
    Keywords:  Embolism and thrombosis; Fontan procedure; Heart; Heart Defects, Congenital; Outcomes; Pregnancy
    DOI:  https://doi.org/10.1016/j.ijcard.2019.08.039
  2. Compr Child Adolesc Nurs. 2019 Oct 04. 1-20
    Jackson AC, Frydenberg E, Koey XM, Fernandez A, Higgins RO, Stanley T, Liang RP, Le Grande MR, Murphy BM.
      Families of children with congenital heart disease (CHD) can have difficulties coping with the stress of their child's condition and would benefit from assistance to cope better. To address the needs of these parents, the Australian Center for Heart Health/HeartKids Australia/Melbourne Graduate School of Education co-produced Family Coping Project was initiated. This project involved two systematic literature reviews, interviews with parents of children with CHD, and the development and piloting of a manualised parental coping program. The primary aims of the pilot study were to determine whether the program would: attract high needs families; enhance the coping self-efficacy of parents; and be acceptable to parents in terms of content and mode of delivery. The secondary aims were to investigate whether the program would impact on parental coping, parental stress and general stress. Parents completed pre-, post-program and 6-month follow up assessment measures, with parent stress scores being compared to stress scores reported for other chronic condition parent carer groups. Twenty-one parents participated and provided baseline data. They were found to be significantly more stressed than other parent carer groups. Eleven parents completed post-program data and 13 completed 6-month follow-up data. There was a significant increase in parents' coping self-efficacy from pre- to post-program, and from pre- to 6-months. Parents' use of productive coping styles increased significantly from pre- to post-program. The program was rated as highly acceptable in terms of content and delivery mode. The pilot provides strong evidence for upscaling the program in conjunction with individualized psychological support for parents to extend knowledge acquisition and attitude change into enhanced coping skills and demonstrated the benefits of a co-production process.
    Keywords:  Congenital heart disease; co-production; family coping; family stress
    DOI:  https://doi.org/10.1080/24694193.2019.1671915
  3. Can J Cardiol. 2019 Jun 26. pii: S0828-282X(19)30441-6. [Epub ahead of print]
    Keir M, Ebert P, Kovacs AH, Smith JMC, Kwan E, Field TS, Brossard-Racine M, Marelli A.
      Children born with congenital heart disease (CHD) are now living to adulthood in unprecedented numbers and many will eventually live to become senior citizens. As care goals shift from surviving to thriving, a new focus on quality of life has emerged. Neurocognition and the ability to participate fully in society, form meaningful relationships, and collaborate effectively with the health care system are important considerations. As adults with CHD age, research regarding their cognitive function becomes prescient. The focus is now shifting from defining neurocognitive deficits in children with CHD to preventing neurocognitive decline in adults living with CHD. In this review, we describe the possible etiologies and predictors of neurocognitive decline in adults with CHD. We performed a comprehensive literature review to identify all of the current data available on neurocognitive function in adults with CHD. We summarize the available evidence by describing common deficits in this patient population and the potential effects of these deficits on adult functioning, health care decision-making, and long-term relationships with care providers. We review potential modifiable etiologies for progressive neurocognitive decline and suggest strategies for surveillance and prevention of the potential decline. We conclude that the current information available regarding the aging brain of adults with CHD and the effect of neurocognitive decline on morbidity and mortality is woefully insufficient. This review, therefore, provides a roadmap for future research endeavours to study neurocognition in older adults with CHD.
    DOI:  https://doi.org/10.1016/j.cjca.2019.06.020
  4. J Child Health Care. 2019 Sep 30. 1367493519878550
    Dahlawi N, Milnes LJ, Swallow V.
      Congenital heart disease (CHD) is one of the common types of birth defects. Children and young people (CYP) with CHD might exhibit behavioural and emotional changes related to undergoing different medical treatments and hospitalization. Therefore, a literature review was conducted from January 2000 to June 2017 that aimed to understand and evaluate current international literature focusing on CYP's behavioural and emotional status as patients with CHD. A comprehensive search of Medline, PsycINFO and CINAHL databases was undertaken. Eight quantitative studies were reviewed following strict eligibility criteria. The Mixed Methods Appraisal Tool (MMAT) was used to assess the quality of the reviewed studies. Parents provided proxy reports on their children's behaviour and emotions in all studies. Half of the reviewed studies presented the self-perceptions of children who were over seven years old. CYP with CHD exhibited internalizing and externalizing behavioural problems, withdrawal, depression, social, and attention problems. CYP with more severe CHD reported greater behavioural and emotional problems than CYP with less severe CHD. Moreover, younger children developed more problems than older children. Future more depth research using qualitative designs is required to explore the personal views of children younger than seven years old on the impact of CHD on their behaviour and emotions.
    Keywords:  Adolescents; CHD; behaviour and emotions; children; parents; young people
    DOI:  https://doi.org/10.1177/1367493519878550
  5. Pediatr Crit Care Med. 2019 Sep 20.
    Spinner JA, Morris SA, Nandi D, Costarino AT, Marino BS, Rossano JW, Shamszad P.
      OBJECTIVE: There are scarce data about the prevalence and mortality of necrotizing enterocolitis in neonates with congenital heart disease. The purpose of this study is to provide a multi-institutional description and comparison of the overall prevalence and mortality of necrotizing enterocolitis in neonates with congenital heart disease.DESIGN: Retrospective multi-institutional study.
    SETTING: The Pediatric Health Information System database.
    PATIENTS: Neonates with congenital heart disease between 2004 and 2014.
    INTERVENTIONS: None.
    MEASUREMENTS AND MAIN RESULTS: The primary study measure is the prevalence of necrotizing enterocolitis. Secondary measures include in-hospital mortality, hospital charges, ICU length of stay, hospital length of stay, and 30-day readmission. The prevalence of necrotizing enterocolitis was 3.7% (1,448/38,770) and varied significantly among different congenital heart disease diagnoses. The lowest prevalence of necrotizing enterocolitis was in transposition of the great arteries (n = 104, 2.1%). Compared with transposition of the great arteries, necrotizing enterocolitis occurred more frequently in neonates with hypoplastic left heart syndrome (odds ratio, 2.7; 95% CI, 2.1-3.3), truncus arteriosus (odds ratio, 2.6; 95% CI, 1.9-3.5), common ventricle (odds ratio, 2.1; 95% CI, 1.5-2.8), and aortic arch obstruction (odds ratio, 1.4; 95% CI, 1.1-1.7). Prematurity is a significant risk factor for necrotizing enterocolitis and for mortality in neonates with necrotizing enterocolitis, conferring varying risk by cardiac diagnosis. Unadjusted mortality associated with necrotizing enterocolitis was 24.4% (vs 11.8% in neonates without necrotizing enterocolitis; p < 0.001), and necrotizing enterocolitis increased the adjusted mortality in neonates with transposition of the great arteries (odds ratio, 2.5; 95% CI, 1.5-4.4), aortic arch obstruction (odds ratio, 1.8; 95% CI, 1.3-2.6), and tetralogy of Fallot (odds ratio, 1.6; 95% CI, 1.1-2.4). Necrotizing enterocolitis was associated with increased hospital charges (p < 0.0001), ICU length of stay (p = 0.001), and length of stay (p = 0.001).
    CONCLUSIONS: The prevalence of necrotizing enterocolitis among neonates with congenital heart disease is 3.7% and is associated with increased in-hospital mortality, length of stay, and hospital charges. The prevalence and associated mortality of necrotizing enterocolitis in congenital heart disease vary among different heart defects.
    DOI:  https://doi.org/10.1097/PCC.0000000000002133
  6. J Am Heart Assoc. 2019 Oct 15. 8(20): e013450
    Agarwal A, Thombley R, Broberg CS, Harris IS, Foster E, Mahadevan VS, John A, Vittinghoff E, Marcus GM, Dudley RA.
      Background As patients with congenital heart disease (CHD) are living longer, understanding the comorbidities they develop as they age is increasingly important. However, there are no published population-based estimates of the comorbidity burden among the US adult patients with CHD. Methods and Results Using the IBM MarketScan commercial claims database from 2010 to 2016, we identified adults aged ≥18 years with CHD and 2 full years of continuous enrollment. These were frequency matched with adults without CHD within categories jointly defined by age, sex, and dates of enrollment in the database. A total of 40 127 patients with CHD met the inclusion criteria (mean [SD] age, 36.8 [14.6] years; and 48.2% were women). Adults with CHD were nearly twice as likely to have any comorbidity than those without CHD (P<0.001). After adjusting for covariates, patients with CHD had a higher prevalence risk ratio for "previously recognized to be common in CHD" (risk ratio, 9.41; 95% CI, 7.99-11.1), "other cardiovascular" (risk ratio, 1.73; 95% CI, 1.66-1.80), and "noncardiovascular" (risk ratio, 1.47; 95% CI, 1.41-1.52) comorbidities. After adjusting for covariates and considering interaction with age, patients with severe CHD had higher risks of previously recognized to be common in CHD and lower risks of other cardiovascular comorbidities than age-stratified patients with nonsevere CHD. For noncardiovascular comorbidities, the risk was higher among patients with severe than nonsevere CHD before, but not after, the age of 40 years. Conclusions Our data underscore the unique clinical needs of adults with CHD compared with their peers. Clinicians caring for CHD may want to use a multidisciplinary approach, including building close collaborations with internists and specialists, to help provide appropriate care for the highly prevalent noncardiovascular comorbidities.
    Keywords:  cohort study; comorbidities heart failure; congenital cardiac defect
    DOI:  https://doi.org/10.1161/JAHA.119.013450
  7. Pediatr Rev. 2019 Oct;40(10): 517-527
    Wertheimer F, Arcinue R, Niklas V.
      Necrotizing enterocolitis (NEC) has been recognized for well over 5 decades yet remains the most common life-threatening surgical emergency in the newborn. The incidence of NEC has decreased steadily in preterm and very-low-birthweight infants over several decades and is typically uncommon in term newborns and infants with a birthweight greater than 2,500 g. Evidence accumulating during the past decade, however, suggests that practitioners should consider NEC in this broader subset of term infants with chromosomal and congenital anomalies complicated by heart or gastrointestinal defects when signs and symptoms of feeding intolerance, abdominal illness, or sepsis are present. The short- and long-term consequences of NEC are devastating in all infants, and although early disease recognition and treatment are essential, promoting human milk feeding as a primary modality in prevention is critical. This article highlights our current understanding of the pathophysiology, the clinical presentation, the risk factors for NEC in term infants compared with premature infants, and the treatment of NEC and discusses strategies in the prevention of NEC. Finally, we review the long-term consequences of NEC and the importance of primary care practitioners in the long-term care of infants after hospitalization for NEC.
    DOI:  https://doi.org/10.1542/pir.2017-0338
  8. Pediatr Phys Ther. 2019 Oct;31(4): 315-322
    Hall CA, Donza C, McGinn S, Rimmer A, Skomial S, Todd E, Vaccaro F.
      PURPOSE: The purpose of this systematic review is to identify quality-of-life issues that affect participation in age-appropriate activities in chronically ill children, as reported by the children and their families.SUMMARY OF KEY POINTS: Social and emotional functioning scores on the Pediatric Quality of Life Inventory (PedsQL) 4.0 were found to have the greatest frequency of poor agreement between parents and children in 4 of the 6 studies included in this review, suggesting parents and children have wide variation in their assessment in these areas of psychosocial function.
    CONCLUSIONS: Cumulative evidence appears to indicate that parents of children with chronic illness perceive their children as having a poorer quality of life than the children report for themselves.
    RECOMMENDATIONS FOR CLINICAL PRACTICE: Identifying differences and commonalities between these reports can guide health care practitioners to specific activities that should be the focus of caring for children; specifically, functional goal development can become more personalized and appropriate.
    DOI:  https://doi.org/10.1097/PEP.0000000000000638
  9. J Pediatr. 2019 Sep 25. pii: S0022-3476(19)31094-7. [Epub ahead of print]
    Naef N, Wehrle F, Rousson V, Latal B.
      OBJECTIVE: To assess cohort and individual neurodevelopmental stability in children with congenital heart disease across childhood.STUDY DESIGN: The Reachout Study is a cohort study at the University Children's Hospital Zurich. Data from 148 children with congenital heart disease who underwent cardiopulmonary bypass surgery and 1-, 4-, and 6-year neurodevelopmental assessment were analyzed using mixed models.
    RESULTS: Cognitive and motor functions of the total cohort improved over time (cognitive: P = .01; motor: P <.001). The prevalence of children with cognitive impairment at age 6 years was 22.3%. Socioeconomic status showed a significant interaction with age on cognitive and motor development (cognitive: P <.001; motor: P = .001): higher socioeconomic status was associated with better neurodevelopmental outcome over time. Weight and head circumference at birth showed a significant interaction with age on motor development (weight: P = .048; head: P = .006). The correlation between test scores at different ages was weak to moderate (cognition: age 1-6 years: rho = 0.20, age 4-6 years: rho = 0.56, motor: age 1-6 years: rho = 0.23, age 4-6 years: rho = 0.50).
    CONCLUSIONS: Children with congenital heart disease show a mild improvement in cognitive and motor functions within the first 6 years of life, particularly those with higher socioeconomic status and larger head circumference and weight at birth. However, individual stability is moderate at best. Therefore, follow-up assessments are crucial to target therapeutic intervention effectively.
    Keywords:  cohort studies; congenital; developmental disabilities/classification; heart defects; neuropsychological test
    DOI:  https://doi.org/10.1016/j.jpeds.2019.08.036