bims-hylehe Biomed News
on Hypoplastic left heart syndrome
Issue of 2019‒04‒07
ten papers selected by
Richard James
University of Pennsylvania


  1. J Cardiol. 2019 Mar 27. pii: S0914-5087(19)30059-0. [Epub ahead of print]
    Gordon-Walker TT, Bove K, Veldtman G.
      The Fontan procedure has led to increased long-term survival of patients with single ventricle congenital heart disease. Hemodynamic changes associated with the Fontan circulation, including elevated central venous pressure and diminished cardiac output are responsible for the development of Fontan-associated liver disease (FALD). Liver fibrosis is a universal feature following the Fontan operation. The incidence of both liver cirrhosis and hepatocellular carcinoma (HCC) increases with the duration of the Fontan circulation. The staging of liver fibrosis in FALD requires a multi-modality approach involving clinical assessment, biochemical/hematological parameters, non-invasive fibrosis scores, radiological imaging, elastography, and liver histology. Patients with a failing Fontan circulation who have evidence of significant hepatic congestion require careful hemodynamic assessment to optimize the Fontan pathway and physiology. This may necessitate percutaneous or surgical intervention, or heart transplantation. Combined heart-liver transplantation may be required in patients with clinical, imaging, or biopsy evidence of advanced liver cirrhosis, particularly if there is evidence of hepatic decompensation or localized HCC. Patients with suspected liver cirrhosis should be enrolled into HCC surveillance and require endoscopic variceal assessment. There is a clear need to establish local/national registries for Fontan patients with standardized guidelines for the management of FALD, bringing together the expertise of professional bodies representing both cardiologists and hepatologists.
    Keywords:  Cirrhosis; Combined heart-liver transplant; Congenital heart disease; Fontan procedure; Hepatocellular carcinoma
    DOI:  https://doi.org/10.1016/j.jjcc.2019.02.016
  2. J Thorac Cardiovasc Surg. 2019 Feb 21. pii: S0022-5223(19)30479-9. [Epub ahead of print]
    Ruiz VM, Saenz L, Lopez-Magallon A, Shields A, Ogoe HA, Suresh S, Munoz R, Tsui FR.
      OBJECTIVE: Critical events are common and difficult to predict among infants with congenital heart disease and are associated with mortality and long-term sequelae. We aimed to achieve early prediction of critical events, that is, cardiopulmonary resuscitation, emergency endotracheal intubation, and extracorporeal membrane oxygenation in infants with single-ventricle physiology before second-stage surgery. We hypothesized that naïve Bayesian models learned from expert knowledge and clinical data can predict critical events early and accurately.METHODS: We collected 93 patients with single-ventricle physiology admitted to intensive care units in a single tertiary pediatric hospital between 2014 and 2017. Using knowledge elicited from experienced cardiac-intensive-care-unit providers and machine-learning techniques, we developed and evaluated the Cardiac-intensive-care Warning INdex (C-WIN) system, consisting of a set of naïve Bayesian models that leverage routinely collected data. We evaluated predictive performance using the area under the receiver operating characteristic curve, sensitivity, and specificity. We performed the evaluation at 5 different prediction horizons: 1, 2, 4, 6, and 8 hours before the onset of critical events.
    RESULTS: The area under the receiver operating characteristic curves of the C-WIN models ranged between 0.73 and 0.88 at different prediction horizons. At 1 hour before critical events, C-WIN was able to detect events with an area under the receiver operating characteristic curve of 0.88 (95% confidence interval, 0.84-0.92) and a sensitivity of 84% at the 81% specificity level.
    CONCLUSIONS: Predictive models may enhance clinicians' ability to identify infants with single-ventricle physiology at high risk of critical events. Early prediction of critical events may indicate the need to perform timely interventions, potentially reducing morbidity, mortality, and healthcare costs.
    Keywords:  cardiopulmonary resuscitation; congenital heart defects; endotracheal intubation; extracorporeal membrane oxygenation; hypoplastic left heart syndrome; risk assessment
    DOI:  https://doi.org/10.1016/j.jtcvs.2019.01.130
  3. J Pediatr Nurs. 2019 Mar 27. pii: S0882-5963(18)30415-9. [Epub ahead of print]
    Chen YJ, Wang JK, Liu CY, Yang CY, Chiang YT, Chen CW.
      PURPOSE: The aims of this study were to examine the relationship between healthcare needs, health-promoting behaviors and quality of life (QoL) of youths with congenital heart disease (CHD), and to determine whether health-promoting behaviors were a mediating variable.DESIGN AND METHODS: This cross-sectional descriptive study was conducted recruiting 205 youths aged 15 to 24 with either ventricular septal defect (VSD) or tetralogy of Fallot (TOF). A self-reported questionnaire was used to collect demographic information; furthermore, the thickness of the triceps skinfold was measured. The Healthcare Needs Scale for Youth with CHD, Adolescent Health Promotion-short form scale, and World Health Organization QoL-BREF scale were administered, and Pearson's correlation, multiple regressions, and the Sobel test were conducted.
    RESULTS: The triceps skinfold of participants with VSD was significantly thicker than that of participants with TOF (p < 0.05). Healthcare needs, health-promoting behaviors, and QoL were positively correlated. The overall health-promoting behaviors served as a partial mediator between overall healthcare needs and QoL, accounting for 40.1% of the total variation.
    CONCLUSIONS: The study illustrates that health-promoting behaviors have an impact on healthcare needs and QoL of youths with CHD. The development of tailored health consulting interventions to fulfill healthcare needs is required to improve physical and psychosocial wellness among youths with CHD.
    PRACTICE IMPLICATIONS: Targeting individual and public health strategies for enhancing health-promoting behaviors should be integrated in CHD care planning.
    Keywords:  Congenital heart disease; Health care transition; Health-promoting behaviors; Healthcare needs; Quality of life
    DOI:  https://doi.org/10.1016/j.pedn.2019.03.011
  4. Congenit Heart Dis. 2019 Apr 04.
    Ezzeddine FM, Moe T, Ephrem G, Kay WA.
      BACKGROUND: Delivery of care to the adult congenital heart disease (ACHD) population has been limited by a shortage in the ACHD physician resources. There is limited data regarding the adequacy of the ACHD physician resources in the United States and our population estimates are extrapolated from Canadian data. Therefore, we proposed to evaluate the adequacy of ACHD physician: patient ratios in the United States at both national and regional levels.METHODS: Data from the Adult Congenital Heart Association (ACHA) website along with metropolitan area and statewide population data from 2016 US Census Bureau estimates were analyzed. Physicians listed on the ACHA website were cross-referenced with ABIM to verify ACHD board certification status.
    RESULTS: There are 115 self-identified ACHD programs and 418 self-identified ACHD physicians listed in the ACHA website. There are 320 board-certified ACHD cardiologists in the United States today, including 161 not listed in the ACHA website. Regarding ratios of ACHD-certified physicians to patients, the best served metropolitan statistical area (MSA) is Raleigh-Cary, NC, and the worst served MSA is Riverside-San Bernardino-Ontario, CA. The best served State is Washington, DC, and the worst served State is Indiana.
    CONCLUSIONS: The ACHD population continues to grow, and the looming national physician shortage is likely to greatly affect the ability to meet the complex needs of this growing population. In order to bring the ACHD patient: physician ratio to 1000:1, a minimum of 170 additional ACHD board-certified physicians are needed now.
    Keywords:  United States; congenital heart disease; physician; program
    DOI:  https://doi.org/10.1111/chd.12771
  5. J Clin Psychol Med Settings. 2019 Apr 02.
    Lakatos PP, Matic T, Carson M, Williams ME.
      Hospitalization in the Neonatal Intensive Care Unit (NICU) is a stressful and potentially traumatic experience for infants as well as their parents. The highly specialized medical environment can threaten the development of a nurturing and secure caregiving relationship and potentially derail an infant's development. Well-timed, dose-specific interventions that include an infant mental health approach can buffer the impact of medical traumatic stress and separations and support the attachment relationship. Many psychological interventions in the NICU setting focus on either the parent's mental health or the infant's neurodevelopmental functioning. An alternative approach is to implement a relationship-based, dyadic intervention model that focuses on the developing parent-infant relationship. Child-parent psychotherapy (CPP) is an evidence-based trauma-informed dyadic intervention model for infants and young children who have experienced a traumatic event. This article describes the adaptation of CPP for the NICU environment.
    Keywords:  Child–parent psychotherapy; Infant mental health; Neonatal intensive care unit; Pediatric medical traumatic stress; Pediatric psychology; Trauma-informed care
    DOI:  https://doi.org/10.1007/s10880-019-09614-6
  6. J Card Surg. 2019 Apr 03.
    Parker DM, Everett AD, Stabler ME, Vricella L, Jacobs ML, Jacobs JP, Thiessen-Philbrook H, Parikh CR, Brown JR.
      OBJECTIVES: Novel cardiac biomarkers serum (suppression of tumorigenicity [ST2]) and Galectin-3 may be associated with an increased likelihood of important events after cardiac surgery. Our objective was to explore the association between pre- and postoperative serum biomarker levels and 30-day readmission or mortality for pediatric patients.METHODS: We prospectively enrolled pediatric patients <18 years of age who underwent at least one cardiac surgical operation at Johns Hopkins Children's Center from 2010 to 2014 (N = 162). Blood samples were collected immediately before surgery and at the end of bypass. We evaluated the association between pre- and postoperative Galectin-3 and ST2 with 30-day readmission or mortality, using backward stepwise logistic regression, adjusting for covariates based on the Society of Thoracic Surgeons (STS) Congenital Heart Surgery Mortality Risk Model.
    RESULTS: In our cohort, 21 (12.9%) patients experienced readmission or mortality 30-days from discharge. Before adjustment, preoperative ST2 terciles demonstrated a strong association with readmission and/or mortality after surgery (OR: 2.58; 95% CI: 1.17-3.66 and OR: 4.37; 95% CI: 1.31-14.57). After adjustment for covariates based on the STS congenital risk model, Galectin-3 postoperative mid-tercile was significantly associated with 30-day readmission or mortality (OR: 6.17; 95% CI: 1.50-0.43) as was the highest tercile of postoperative ST2 (OR: 4.98; 95% CI: 1.06-23.32).
    CONCLUSIONS: Elevated pre-and postoperative levels of ST2 and Galectin-3 are associated with increased risk of readmission or mortality after pediatric heart surgery. These clinically available biomarkers can be used for improved risk stratification and may guide improved patient care management.
    Keywords:  biomarkers; pediatric congenital heart disease; prediction; readmission
    DOI:  https://doi.org/10.1111/jocs.14038
  7. Ann Am Thorac Soc. 2019 Apr 05.
    Johnson CC, Suchyta MR, Darowski ES, Collar EM, Kiehl AL, Van J, Jackson JC, Hopkins RO.
      RATIONALE: Family members of critically-ill patients hospitalized in the intensive care unit (ICU) often become caregivers and they are at risk to develop adverse psychological outcomes. There is a need to understand the psychological impact of critical illness on family caregivers.OBJECTIVES: The aim of this systematic review is to document the prevalence of depression, anxiety, and posttraumatic stress disorder (PTSD) in family caregivers of critically-ill patients and identify potential risk factors for psychological outcomes to inform clinical and future research recommendations.
    METHODS: A literature search for psychological outcomes for family caregivers of critically-ill patients was conducted. A total of 1,148 studies from PsycINFO, CINAHL, Web of Science, SCOPUS and Medline were identified.
    RESULTS: Forty studies met inclusion criteria and were included in the review. The prevalence of psychological outcomes in family caregivers ranged from 4% to 94% for depression, 2% to 80% for anxiety, and 3% to 62% for PTSD. Caregiver depression, anxiety, and PTSD decreased in most studies that assessed longitudinal outcomes. Common risk factors identified for adverse psychological outcomes included younger caregiver age, caregiver relationship to the patient, lower socioeconomic status, and female sex.
    CONCLUSIONS: The prevalence of depression, anxiety, and PTSD varies greatly across studies of family caregivers of critically-ill patients. This finding highlights the need for more systematic investigations of psychological outcomes and the implementation of clinical interventions to prevent or reduce depression, anxiety, and PTSD in family caregivers of critically-ill patients.
    DOI:  https://doi.org/10.1513/AnnalsATS.201808-540SR
  8. J Clin Nurs. 2019 Apr 02.
    So SCY, Li WHC, Ho KY.
      AIMS AND OBJECTIVES: This study examined the psychological well-being and quality of life of Hong Kong Chinese adolescents with congenital heart disease.BACKGROUND: Congenital heart disease (CHD) in adolescents is associated with having negative psychological impact and impairment to quality of life. A literature review revealed that most studies on the impact of CHD on the psychological well-being and quality of life of adolescents to date have been conducted in Western populations.
    METHOD: A cross-sectional design was employed. Adolescents aged 12-18 with CHD attending an outpatient clinic in an acute public hospital were invited to participate. Another similar age group of healthy Chinese adolescents were also invited to participate for comparison purposes. Subjects were asked to respond to the Chinese version of the Pediatric Quality of Life Inventory 4.0 Generic Core Scales, Center for Epidemiologic Studies Depression Scale for Children, Rosenberg Self-Esteem Scale, and a demographic sheet. A STROBE checklist was completed.
    RESULTS: Adolescents with CHD exhibited more depressive symptoms, lower self-esteem, and poorer quality of life than their healthy counterparts. Disease severity might affect the self-esteem, depressive symptoms and quality of life of adolescents with CHD. Disease severity, depressive symptoms, self-esteem level, and types of treatment received, were associated with the quality of life of adolescents with CHD.
    CONCLUSIONS: Hong Kong Chinese adolescents suffering from CHD experience negative impacts on their psychological well-being and quality of life. In this group, disease severity may affect psychological well-being and quality of life. Specifically, quality of life of adolescents with CHD was associated with disease severity, depressive symptoms, self-esteem level and the type of treatment received.
    RELEVANCE TO CLINICAL PRACTICE: The findings contribute to clinical care guidelines and serve as a reference in developing nursing intervention to adolescents with CHD so as to uphold quality of care. This article is protected by copyright. All rights reserved.
    Keywords:  Adolescents; Chinese; Congenital heart disease; Hong Kong; Psychological well-being; Quality of life
    DOI:  https://doi.org/10.1111/jocn.14864
  9. Congenit Heart Dis. 2019 Mar;14(2): 138-139
    Akintoye E, Veldtman GR, Miranda WR, Connolly HM, Egbe AC.
      The purpose of this study was to determine the optimal age for performing Fontan operation using data from the National Inpatient Sample. Our results showed that although the Fontan operation was most commonly performed at age 2 in the United States, age 3 is the optimum age for this procedure as evident by lower rate of in-hospital mortality, procedure-related complications, and rate of nonroutine home discharge when procedure is performed at age 3 years.
    DOI:  https://doi.org/10.1111/chd.12690