bims-hylehe Biomed news
on Hypoplastic left heart syndrome
Issue of 2019‒02‒03
seven papers selected by
Richard James
University of Pennsylvania


  1. Cardiol Young. 2019 Jan 30. 1-5
    Gleason LP, Deng LX, Khan AM, Drajpuch D, Fuller S, Ludmir J, Mascio CE, Partington SL, Tobin L, Kim YY, Kovacs AH.
      BACKGROUND: Adults with congenital heart disease face psychological challenges although an understanding of depression vs. anxiety symptoms is unclear. We analyzed the prevalence of elevated symptoms of anxiety and depression and explored associations with demographic and medical factors as well as quality of life.METHODS: Adults with congenital heart disease enrolled from an outpatient clinic completed the Hospital Anxiety and Depression Scale and two measures of quality of life: the Linear Analogue Scale and the Satisfaction with Life Scale. Medical data were obtained by chart review.
    RESULTS: Of 130 patients (median age = 32 years; 55% female), 55 (42%) had elevated anxiety symptoms and 16 (12%) had elevated depression symptoms on subscales of the Hospital Anxiety and Depression Scale. Most patients with elevated depression symptoms also had elevated anxiety symptoms (15/16; 94%). Of 56 patients with at least one elevated subscale, 37 (66%) were not receiving mental health treatment. Compared to patients with 0 or 1 elevated subscales, patients with elevations in both (n=15) were less likely to be studying or working (47% vs. 81%; p=0.016) and reported lower scores on the Linear Analogue Scale (60 vs. 81, p<0.001) and the Satisfaction with Life Scale (14 vs. 28, p<0.001).
    CONCLUSIONS: Among adults with congenital heart disease, elevated anxiety symptoms are common and typically accompany elevated depressive symptoms. The combination is associated with unemployment and lower quality of life. Improved strategies to provide psychosocial care and support appropriate engagement in employment are required.
    Keywords:  Adult Congenital Heart Disease; Anxiety; Depression; Quality of Life
    DOI:  https://doi.org/10.1017/S1047951118002068
  2. Adv Neonatal Care. 2018 Jan 28.
    Davis JA, Spatz DL.
      BACKGROUND: Human milk is a life-saving medical intervention. Infants with congenital heart disease are at an increased risk for necrotizing enterocolitis, chylothorax, feeding difficulties, and growth failure. In the absence of evidence-based care, their mothers are also at risk for low milk supply and/or poor breastfeeding outcomes.PURPOSE: Summarize the role of human milk and clinical outcomes for infants with congenital heart disease (CHD). Summarize methods of ideal breastfeeding support.
    METHODS/SEARCH STRATEGY: PubMed, Cochrane Library, and CINAHL were the databases used. The terms used for the search related to CHD and necrotizing enterocolitis were "human milk" and "necrotizing enterocolitis" and "congenital heart disease." This resulted in a total of 17 publications for review.
    FINDINGS: Infants receiving exclusive human milk diet are at a lower risk for necrotizing enterocolitis and will have improved weight gain. Infants with chylothorax who receive skimmed human milk have higher weight-for-age scores than formula-fed infants. Maternal breastfeeding education correlates with decreased risk of poor breastfeeding outcomes.
    IMPLICATIONS FOR PRACTICE: Human milk is the ideal source of nutrition for infants with CHD and should be encouraged by the care team. Evidence-based lactation education and care must be provided to mothers and families prenatally and continue throughout the infant's hospitalization. If a mother's goal is to directly breastfeed, this should be facilitated during the infant's hospital stay.
    IMPLICATIONS FOR RESEARCH: Evaluate the role between human milk and the incidence of necrotizing enterocolitis, feeding difficulties, and clinical outcomes in the population of infants with CHD.
    DOI:  https://doi.org/10.1097/ANC.0000000000000582
  3. J Am Heart Assoc. 2019 Feb 05. 8(3): e010995
    Zhang W, Spero TL, Nolte CG, Garcia VC, Lin Z, Romitti PA, Shaw GM, Sheridan SC, Feldkamp ML, Woomert A, Hwang SA, Fisher SC, Browne ML, Hao Y, Lin S, .
      Background More intense and longer-lasting heat events are expected in the United States as a consequence of climate change. This study aimed to project the potential changes in maternal heat exposure during early pregnancy (3-8 weeks post conception) and the associated burden of congenital heart defects ( CHD s) in the future. Methods and Results This study expanded on a prior nationwide case-control study that evaluated the association between CHD s and maternal heat exposure during early pregnancy in summer and spring. We defined multiple indicators of heat exposure, and applied published odds ratios obtained for the matching season of the baseline (1995-2005) into the projection period (2025-2035) to estimate potential changes in CHD burden throughout the United States. Increases in maternal heat exposure were projected across the United States and to be larger in the summer. The Midwest will potentially have the highest increase in summer maternal exposure to excessively hot days (3.42; 95% CI, 2.99-3.88 per pregnancy), heat event frequency (0.52; 95% CI, 0.44-0.60) and heat event duration (1.73; 95% CI, 1.49-1.97). We also found large increases in specific CHD subtypes during spring, including a 34.0% (95% CI, 4.9%-70.8%) increase in conotruncal CHD in the South and a 38.6% (95% CI , 9.9%-75.1%) increase in atrial septal defect in the Northeast. Conclusions Projected increases in maternal heat exposure could result in an increased CHD burden in certain seasons and regions of the United States.
    Keywords:  climate change; congenital heart defects; maternal heat exposure; pregnancy; projection
    DOI:  https://doi.org/10.1161/JAHA.118.010995
  4. J Genet Couns. 2019 Jan 28.
    Kaulfus ME, Gardiner H, Hashmi SS, Mendez-Figueroa H, Miller VJ, Stevens B, Carter R.
      Trisomy 18 is an autosomal trisomy condition characterized by minor to major birth defects, severe disabilities, and high rates of pre- and postnatal mortality. Interventions for these infants have traditionally been withheld with focus instead on palliative support. The issues and attitudes surrounding corrective surgery of congenital heart defects, which is a birth defect that occurs in approximately 90% of infants with trisomy 18, is of our study's interest as recent literature has indicated that cardiac surgery is being performed and may lead to improved survival compared to palliative care. Thus, our study aimed to describe clinician attitudes toward cardiac surgery and trisomy 18. We surveyed 378 clinicians from multiple specialties, including genetic counselors, involved in the pre- and postnatal care of infants with trisomy 18. Descriptive statistics were performed to describe all clinicians' responses, and a secondary analysis with stratifications by clinician type was also performed. Forty-eight percent (n = 378) of clinicians felt it was appropriate to discuss the option of cardiac surgery. Ethical concerns and insufficient outcome data were the most agreed upon reasons for not offering cardiac surgery. Trisomy 18 not being uniformly lethal and expressed parental wishes were the most agreed upon justifications for offering surgery. Clinicians felt the discussion of the option of cardiac surgery is appropriate, however are hesitant due to ethical concerns and insufficient outcome data. Results from this study aim to promote discussion and collaboration among clinicians to improve consistency in patient care.
    Keywords:  aggressive interventions; congenital heart surgery; ethics; genetic counselor; trisomy 18
    DOI:  https://doi.org/10.1002/jgc4.1089
  5. Aging (Albany NY). 2019 Jan 28.
    Mandalenakis Z, Skoglund K, Dellborg M.
      
    Keywords:  aging; congenital heart disease
    DOI:  https://doi.org/10.18632/aging.101800
  6. Am J Cardiol. 2019 Jan 08. pii: S0002-9149(19)30055-4. [Epub ahead of print]
    Boukovala M, Müller J, Ewert P, Hager A.
      With rising survival rates of patients with congenital heart disease (CHD), functional health variables have become the key aspect in treatment evaluation. The effectiveness of various treatment options on the health-related quality of life (HRQoL) and the objectively measured exercise capacity as peak oxygen uptake (VO2 peak) remains rather unclear and hence, its investigation is the primary aim of this study. Data from 1014 patients (≥14-years-old, various CHD) were retrospectively reviewed. The patients had completed at least twice the SF-36 questionnaire on HRQoL prior to a cardiopulmonary exercise test. Each patient was assigned to 1 of 4 treatment groups (i.e., surgery, catheter intervention, drug therapy, and surveillance) according to the received treatment between the baseline and the follow-up examination. After 4.0 ± 2.2 years of follow-up, patients with surgery and catheter intervention showed an increase in the physical summary score of HRQoL as compared to the other treatment groups (p <0.001). This effect remained also significant in a multivariable model accounting for anthropometric and baseline data. No significant differences in the mental summary score of HRQoL and the VO2 peak were evident between the different treatment groups in the multivariable model. No significant correlation was found between the changes in HRQoL and VO2 peak over time. In conclusion, despite insignificant changes in aerobic capacity, adolescents and adults with CHD report better physical HRQoL following surgery and catheter intervention compared to the other treatment options. HRQoL and exercise capacity need to be considered concurrently in the evaluation of adolescents and adults with CHD.
    DOI:  https://doi.org/10.1016/j.amjcard.2018.12.048
  7. Paediatr Anaesth. 2019 Feb 01.
    Taylor D, Habre W.
      Database analysis has proved that peri-operative cardiac arrest occurs with increased frequency in children with congenital heart disease. Several case series and bigger data from ACS NSQIP, have elucidated subgroups at the highest risk. Consistently patients with single ventricle physiology (especially prior to cavo-pulmonary anastomosis), severe/supra-systemic pulmonary hypertension, complex lesions and cardiomyopathy with significantly reduced ventricular function have been shown to be of the greatest concern. Subsequently risk assessment algorithms have been proposed, some based on the national databases. How hospitals and health care systems apply these guidelines to provide safe care to this challenging patient groups requires application of modern quality improvement techniques. Each institution needs to develop a system which reflects local expertise and resources. This article is protected by copyright. All rights reserved.
    DOI:  https://doi.org/10.1111/pan.13595