bims-hylehe Biomed News
on Hypoplastic left heart syndrome
Issue of 2020‒03‒15
seven papers selected by
Richard James
University of Pennsylvania


  1. J Pediatr. 2020 Mar 05. pii: S0022-3476(20)30114-1. [Epub ahead of print]
    Miller TA, Lisanti AJ, Witte MK, Elhoff JJ, Mahle WT, Uzark KC, Alexander N, Butler SC.
      OBJECTIVE: Assess differences in approaches to and provision of developmental care for infants undergoing surgery for congenital heart disease.STUDY DESIGN: A collaborative learning approach was used to stratify, assess, and compare individualized developmental care practices among multidisciplinary teams at 6 pediatric heart centers. Round robin site visits were completed with structured site visit goals and postvisit reporting. Practices of the hosting site were assessed by the visiting team and reviewed along with center self-assessments across specific domains including pain management, environment, cue-based care, and family based care coordination.
    RESULTS: Developmental care for infants in the cardiac intensive care unit (CICU) varies at both a center and individual level. Differences in care are primarily driven by variations in infrastructure and resources, composition of multidisciplinary teams, education of team members, and use of developmental care champions. Management of pain follows a protocol in most cardiac intensive care units, but the environment varies across centers, and the provision of cue-based infant care and family-based care coordination varies widely both within and across centers. The project led to proposed changes in clinical care and center infrastructure at each participating site.
    CONCLUSIONS: A collaborative learning design fostered rapid dissemination, comparison, and sharing of strategies to approach a complex multidisciplinary care paradigm. Our assessment of experiences revealed marked variability across and within centers. The collaborative findings were a first step toward strategies to quantify and measure developmental care practices in the cardiac intensive care unit to assess the association of complex inpatient practices with long-term neurodevelopmental outcomes.
    Keywords:  NIDCAP; cardiac intensive care unit; collaborative learning design; congenital heart disease; individualized developmental care
    DOI:  https://doi.org/10.1016/j.jpeds.2020.01.043
  2. Cardiol Young. 2020 Mar;30(3): 377-382
    Lowenstein S, Macauley R, Perko K, Ronai C.
      OBJECTIVE: Hypoplastic left heart syndrome is a single ventricle defect. While staged surgical palliative treatments have revolutionised care, patients with hypoplastic left heart syndrome continue to have significant morbidity and mortality. In 2017, the National Pediatric Cardiology Quality Improvement Collaborative recommended all single ventricle patients to receive a prenatal palliative care consult. This study aimed to elucidate provider perspectives on the implementation of prenatal palliative care consults for families expecting a child with hypoplastic left heart syndrome.METHODS: An online survey was administered to obstetric and paediatric providers of relevant disciplines to assess their experience with palliative care involvement in hypoplastic left heart syndrome cases.
    RESULTS: Nearly, all physicians (97%) and most registered nurses (79%) agreed that the initial palliative care consult for patients with hypoplastic left heart syndrome should occur during the prenatal period. Respondents also indicated that prenatal palliative care consults should also be offered in a variety of other CHD conditions. Participants believed positive aspects of this new referral protocol included an expanded support network for families, decreased family stress during the postnatal period, increased patient education about what to expect during the postnatal period, and continuity of care.
    CONCLUSION: Multidisciplinary healthcare professionals believe that prenatal palliative care consults provide a variety of benefits for patients and families with hypoplastic left heart syndrome. Additional, multi-centre research is necessary to evaluate whether prenatal palliative care consults should become standard of care for families expecting a child with a single ventricle defect.
    Keywords:  Hypoplastic left heart syndrome; goals of care; palliative care; prenatal diagnosis; single ventricle defect
    DOI:  https://doi.org/10.1017/S1047951120000128
  3. Dev Med Child Neurol. 2020 Mar 09.
    Huisenga D, La Bastide-Van Gemert S, Van Bergen A, Sweeney J, Hadders-Algra M.
      AIM: (1) To systematically review the literature on developmental outcomes from infancy to adolescence of children with complex congenital heart disease (CHD) who underwent early surgery; (2) to run a meta-regression analysis on the Bayley Scales of Infant Development, Second Edition Mental Developmental Index and Psychomotor Developmental Index (PDI) of infants up to 24 months and IQs of preschool-aged children to adolescents; (3) to assess associations between perioperative risk factors and outcomes.METHOD: We searched pertinent literature (January 1990 to January 2019) in PubMed, Embase, CINAHL, and PsycINFO. Selection criteria included infants with complex CHD who had primary surgery within the first 9 weeks of life. Methodological quality, including risk of bias and internal validity, were assessed.
    RESULTS: In total, 185 papers met the inclusion criteria; the 100 with high to moderate methodological quality were analysed in detail. Substantial heterogeneity in the group with CHD and in methodology existed. The outcome of infants with single-ventricle CHD was inferior to those with two-ventricle CHD (respectively: average scores for PDI 77 and 88; intelligence scores 92 and 98). Perioperative risk factors were inconsistently associated with developmental outcomes.
    INTERPRETATION: The literature on children undergoing surgery in early infancy suggests that infants with a single ventricle are at highest risk of adverse developmental outcomes.
    WHAT THIS PAPER ADDS: Children with complex congenital heart disease (CHD) are at increased risk of impaired developmental outcome. Children with single-ventricle CHD have worse outcomes than children with two-ventricle CHD. Children with two-ventricle CHD gradually grow out of their initial developmental impairment. Perioperative factors are inconsistently associated with outcome.
    DOI:  https://doi.org/10.1111/dmcn.14512
  4. Ann Thorac Surg. 2020 Mar 05. pii: S0003-4975(20)30340-4. [Epub ahead of print]
    Stokes JR, Muscara F, Zannino D, McCarthy MC, Anderson VA, Nicholson JM, d'Udekem Y.
      BACKGROUND: This study investigated factors associated with long-term mental health outcomes of parents of children with a cardiac illness. The objective of the study was to investigate the hypothesis that acute mental health status and psychosocial risk factors (e.g., acute stress reactions, quality of life) would be more strongly associated with long-term mental health outcomes than demographic, diagnostic, morphology or procedure-related factors.METHODS: Participants were 31 parents of children that underwent cardiac surgery within a pediatric hospital. Acute mental health status, psychosocial risk, demographic information, and morphology and procedure-related data were collected within the first 4 weeks of the child's hospital admission. Mental health outcomes, including symptoms of posttraumatic stress, depression, anxiety and general stress, were collected at a 2-year follow-up.
    RESULTS: Acute mental health status and psychosocial risk, specifically acute stress reactions, contributed significantly to parent mental health, explaining 44% of the variance in parent post-traumatic stress score (p<0.001); and, 40% in depression scores (p<0.001). Morphology and procedure-related factors (e.g. prolonged mechanical ventilation) explained a further 12% of the variance in parent post-traumatic stress score (p=0.015); and a further 13% in depression score (p=0.014). No associations were found with demographic factors.
    CONCLUSIONS: Results suggest that acute mental health status is more strongly related to parent mental health outcomes than morphology and procedure-related variables in children undergoing neonatal cardiac surgery, and that demographic variables are not associated with mental health outcomes.
    Keywords:  Cardiac; Congenital Heart Disease (CHD); Pediatric
    DOI:  https://doi.org/10.1016/j.athoracsur.2020.01.072
  5. J Clin Psychol. 2020 Mar 09.
    Martínez-Quintana E, Girolimetti A, Jiménez-Rodríguez S, Fraguela-Medina C, Rodríguez-González F, Tugores A.
      OBJECTIVE: To determine psychological distress in congenital heart disease (CHD) patients.METHODS: Cross-sectional study among consecutive CHD patients recruited from a single hospital outpatient clinic to determine anxiety and depression according to the Hospital Anxiety and Depression Scale (HADS) questionnaire.
    RESULTS: One hundred and sixty-nine CHD patients [29 (19-39) years old, 100 (59%) males] were studied. A total of 25% and 9% of CHD patients showed anxiety and depression symptoms, respectively. Patients with an HADS score ≥ 8 had a significantly worse New York Heart Association (NYHA) functional class, needed more psychological support, had more mental health history, and took more anxiolytic/antidepressant medication than the CHD patients with an HADS score below 8. A worse NYHA functional class [OR, 1.88 (1.01-3.52)] proved to be a predictor of a borderline/abnormal HADS score.
    CONCLUSION: Psychological distress has a high prevalence among CHD patients and having an NYHA Class II and III is a significant predictor of an HADS score ≥ 8.
    Keywords:  anxiety; congenital heart disease; depression; observational descriptive study; quality of life
    DOI:  https://doi.org/10.1002/jclp.22948
  6. Indian J Pediatr. 2020 Mar 12.
    Rohit M, Budakoty S.
      Pediatric heart failure (PHF) is an important cause of mortality and morbidity. Whereas ischemic heart disease is the most important cause of heart failure in adults, congenital heart diseases (CHD) and cardiomyopathies are important etiologies of PHF. Management of PHF also differs from that of adults. Here authors have reviewed the literature on PHF with respect to etiology, symptoms, investigations and treatment strategies.
    Keywords:  Cardiomyopathy; Congenital heart diseases (CHD); Heart failure (HF); Pediatric
    DOI:  https://doi.org/10.1007/s12098-020-03255-6
  7. Ann Thorac Surg. 2020 Mar 05. pii: S0003-4975(20)30338-6. [Epub ahead of print]
    Haapanen H, Tsang V, Kempny A, Neijenhuis R, Kennedy F, Cullen S, Walker F, Kostolny M, Hsia TY, Van Doorn C.
      BACKGROUND: Surgery in grown-ups with congenital heart disease (GUCH) is characterized by complex anatomy, comorbidities, reoperations, and technical challenges. While 30-day postoperative mortality is low, this measure might be insufficient to reflect adverse outcome monitoring. Our study aimed to establish whether prolonged intensive care unit (ICU) stay (≥7 days) and 6-month mortality were more clinically meaningful measures than 30-day mortality and to identify predictors of adverse outcome.METHODS: All consecutive GUCH patients from 1998 to 2015 were identified. Perioperative characteristics, diagnoses, and postoperative data were collected retrospectively. Predictors of 30-day, 6-month mortality, and prolonged ICU stay were determined with logistic regression. Era effect was tested for quality assurances by dividing the cohort into four time intervals.
    RESULTS: Within 17 years, 1093 consecutive cardiac surgical procedures were identified in 1026 GUCH patients. Thirty-day mortality improved significantly over the study period, with an overall 30-day mortality of 1.5%. Six-month mortality and prolonged ICU stay were 2.4% and 6.7% respectively. Despite a decreased number of preoperative patients in NYHA class ≥3, prolonged ICU stay increased over the eras. Predictors of adverse outcome were; NYHA class ≥3, preoperative renal failure, disease of great complexity, preoperative ventilator support, cardiopulmonary bypass time, and concomitant procedures.
    CONCLUSIONS: In the current era of low 30-day mortality, extended 6-month mortality and prolonged ICU stay reporting may be more realistic measures of adverse outcomes for counseling GUCH patients at risk.
    DOI:  https://doi.org/10.1016/j.athoracsur.2020.01.071