bims-hylehe Biomed News
on Hypoplastic left heart syndrome
Issue of 2019‒12‒01
seven papers selected by
Richard James
University of Pennsylvania


  1. J Am Coll Cardiol. 2019 Dec 03. pii: S0735-1097(19)37851-9. [Epub ahead of print]74(22): 2786-2795
    Gaies M, Pasquali SK, Banerjee M, Dimick JB, Birkmeyer JD, Zhang W, Alten JA, Chanani N, Cooper DS, Costello JM, Gaynor JW, Ghanayem N, Jacobs JP, Mayer JE, Ohye RG, Scheurer MA, Schwartz SM, Tabbutt S, Charpie JR.
      BACKGROUND: Patients undergoing complex pediatric cardiac surgery remain at considerable risk of mortality and morbidity, and variation in outcomes exists across hospitals. The Pediatric Cardiac Critical Care Consortium (PC4) was formed to improve the quality of care for these patients through transparent data sharing and collaborative learning between participants.OBJECTIVES: The purpose of this study was to determine whether outcomes improved over time within PC4.
    METHODS: The study analyzed 19,600 hospitalizations (18 hospitals) in the PC4 clinical registry that included cardiovascular surgery from August 2014 to June 2018. The primary exposure was 2 years of PC4 participation; this provided adequate time for hospitals to accrue data and engage in collaborative learning. Aggregate case mix-adjusted outcomes were compared between the first 2 years of participation (baseline) and all months post-exposure. We also evaluated outcomes from the same era in a cohort of similar, non-PC4 hospitals.
    RESULTS: During the baseline period, there was no evidence of improvement. We observed significant improvement in the post-exposure period versus baseline for post-operative intensive care unit mortality (2.1% vs. 2.7%; 22% relative reduction [RR]; p = 0.001), in-hospital mortality (2.5% vs. 3.3%; 24% RR; p = 0.001), major complications (10.1% vs. 11.5%; 12% RR; p < 0.001), intensive care unit length of stay (7.3 days vs. 7.7 days; 5% RR; p < 0.001), and duration of ventilation (61.3 h vs. 70.6 h; 13% RR; p = 0.01). Non-PC4 hospitals showed no significant improvement in mortality, complications, or hospital length of stay.
    CONCLUSIONS: This analysis demonstrates improving cardiac surgical outcomes at children's hospitals participating in PC4. This change appears unrelated to secular improvement trends, and likely reflects PC4's commitment to transparency and collaboration.
    Keywords:  cardiac surgery; collaborative learning; congenital; outcomes; pediatric; quality
    DOI:  https://doi.org/10.1016/j.jacc.2019.09.046
  2. J Am Coll Cardiol. 2019 Dec 03. pii: S0735-1097(19)38162-8. [Epub ahead of print]74(22): 2796-2798
    Bhatt AB, Krishnamurthy Y.
      
    Keywords:  congenital heart disease; quality reporting; surgical outcomes
    DOI:  https://doi.org/10.1016/j.jacc.2019.10.023
  3. Pediatr Cardiol. 2019 Nov 25.
    Silva JA, Neves AL, Flor-de-Lima F, Soares P, Guimarães H.
      Tetralogy of Fallot (ToF) is the most prevalent cyanotic congenital heart disease. Genetic syndromes are present in up to one quarter of patients with this condition, leading to increased morbidity and mortality. Our aim in this work is to characterize our population, evaluate ToF based on the presence of genotype anomalies, and investigate early intervention predictors and outcomes. A retrospective study was performed on neonates with ToF born between August 1, 2008, and August 31, 2018, and admitted to a level III neonatal intensive care unit (NICU). Patients were categorized based on the presence of genotype anomalies and timing of intervention. Thirty-nine neonates were included. The overall mortality during the follow-up period was 5.1% (n = 2). Threatened preterm labor/preterm labor was more prevalent in patients with associated genotype anomalies (p = 0.015). Multivariate analysis showed an association between an abnormal amount of amniotic fluid and ToF with altered genotype, adjusted for smoking, maternal age, gestational age and birth weight [OR = 29.92, 95% CI (1.35-662.44), p = 0.032]. We also found an association between cesarean delivery and neonatal procedures (p = 0.006). Mortality was significantly higher in neonates who underwent early intervention (p = 0.038). Our results indicate that an abnormal amount of amniotic fluid is an independent predictive factor for ToF with genotype alterations. This finding could ultimately have an impact on both prenatal and neonatal counseling and management.
    Keywords:  Genotype anomalies; Neonatal intensive care; Neonatal intervention; Neonates; Tetralogy of Fallot
    DOI:  https://doi.org/10.1007/s00246-019-02239-4
  4. Cardiol Young. 2019 Nov 26. 1-7
    Eckhauser AW, Van Rompay MI, Ravishankar C, Newburger JW, Ram Kumar S, Pizarro C, Ghanayem N, Trachtenberg FL, Burns KM, Hill GD, Atz AM, Hamstra MS, Mazwi M, Park P, Richmond ME, Wolf M, Zampi JD, Jacobs JP, Minich LL, .
      BACKGROUND: The Single Ventricle Reconstruction Trial randomised neonates with hypoplastic left heart syndrome to a shunt strategy but otherwise retained standard of care. We aimed to describe centre-level practice variation at Fontan completion.METHODS: Centre-level data are reported as median or median frequency across all centres and range of medians or frequencies across centres. Classification and regression tree analysis assessed the association of centre-level factors with length of stay and percentage of patients with prolonged pleural effusion (>7 days).
    RESULTS: The median Fontan age (14 centres, 320 patients) was 3.1 years (range from 1.7 to 3.9), and the weight-for-age z-score was -0.56 (-1.35 + 0.44). Extra-cardiac Fontans were performed in 79% (4-100%) of patients at the 13 centres performing this procedure; lateral tunnels were performed in 32% (3-100%) at the 11 centres performing it. Deep hypothermic circulatory arrest (nine centres) ranged from 6 to 100%. Major complications occurred in 17% (7-33%). The length of stay was 9.5 days (9-12); 15% (6-33%) had prolonged pleural effusion. Centres with fewer patients (<6%) with prolonged pleural effusion and fewer (<41%) complications had a shorter length of stay (<10 days; sensitivity 1.0; specificity 0.71; area under the curve 0.96). Avoiding deep hypothermic circulatory arrest and higher weight-for-age z-score were associated with a lower percentage of patients with prolonged effusions (<9.5%; sensitivity 1.0; specificity = 0.86; area under the curve 0.98).
    CONCLUSIONS: Fontan perioperative practices varied widely among study centres. Strategies to decrease the duration of pleural effusion and minimise complications may decrease the length of stay. Further research regarding deep hypothermic circulatory arrest is needed to understand its association with prolonged pleural effusion.
    Keywords:  CHD; Fontan; hypoplastic left heart syndrome; management; perioperative care; quality care
    DOI:  https://doi.org/10.1017/S1047951119002658
  5. J Clin Psychol Med Settings. 2019 Nov 27.
    Saxton SN, Dempsey AG, Willis T, Baughcum AE, Chavis L, Hoffman C, Fulco CJ, Milford CA, Steinberg Z.
      A training and competencies workgroup was created with the goal of identifying guidelines for essential knowledge and skills of psychologists working in neonatal intensive care unit (NICU) settings. This manuscript reviews the aspirational model of the knowledge and skills of psychologists working in NICUs across six clusters: Science, Systems, Professionalism, Relationships, Application, and Education. The purpose of these guidelines is to identify key competencies that direct the practice of neonatal psychologists, with the goal of informing the training of future neonatal psychologists. Neonatal psychologists need specialized training that goes beyond the basic competencies of a psychologist and includes a wide range of learning across multiple domains, such as perinatal mental health, family-centered care, and infant development. Achieving competency will enable the novice neonatal psychologist to successfully transition into a highly complex, medical, fast-paced, often changing environment, and ultimately provide the best care for their young patients and families.
    Keywords:  Competence; Education; NICU; Psychologist; Training
    DOI:  https://doi.org/10.1007/s10880-019-09682-8
  6. Expert Rev Med Devices. 2019 Nov 28.
    Hetzer R, Javier MFDM, Javier Delmo EM.
      Introduction: The development of ventricular assist devices (VADs) have enabled myocardial recovery and improved patient survival until heart transplantation. However, device options remain limited for children and lag in development.Areas covered: This review focuses on the evolution of pediatric VADs in becoming an accepted treatment option in advanced heart failure, discusses the classification of VADs available for children, i.e. types of pumps and duration of support, and defines implantation indications and explantation criteria with attendant complications, discussing its long-term outcome. Furthermore, we emphasize key considerations in the application of these devices in infants, children and adolescents.Expert opinion: Increasing use of VADs has facilitated a leading edge in management of advanced heart failure either as a bridge to transplantation or as a bridge to myocardial recovery. In newborns and small children, the EXCOR Pediatric VAD remains the only reliable option. In some patients ventricular unloading may lead to complete myocardial recovery. VAD development should be aimed at: (1) smaller, more effective durable system components, (2) an energy supply without risk of infection, (3) minimalization of thrombus formation by optimal interior pump design and new antithrombotic medications (4) pulse modulation of continuous flow (5) adaptation of pump activity according to demand (6) a safe automatic system monitoring with telemonitoring (7) a system with simple and safe handling for the patients/parents. There may be future need for pumps that are fully implantable, suitable for single ventricle physiology, such as the right ventricle.
    Keywords:  anticoagulation; cardiomyopathy; congenital heart diseases; heart failure; heart transplantation; myocardial recovery; myocarditis; thrombosis; ventricular assist devices
    DOI:  https://doi.org/10.1080/17434440.2020.1699404
  7. Early Hum Dev. 2019 Nov 20. pii: S0378-3782(19)30367-6. [Epub ahead of print]140 104930
    Malin KJ, Johnson TS, McAndrew S, Westerdahl J, Leuthner J, Lagatta J.
      BACKGROUND: Risk factors for perinatal posttraumatic stress disorder (PTSD) among parents of an infant in the NICU have varied in previous literature. The relationships between perception of illness severity and objective measures of illness severity with PTSD are not well understood.AIMS: To determine if PTSD among parents after an infant NICU discharge can be predicted by 1) objective measures of infant illness severity or 2) perceptions of infant illness severity.
    STUDY DESIGN: A prospective, observational study.
    SUBJECTS: Parent/infant dyads who were in the NICU for ≥14 days.
    OUTCOME MEASURES: Objective measures of illness severity were obtained from the electronic health record. Perceptions of illness were measured by the response to the question, "How sick is your child/patient?" on a 5-point Likert scale. The Perinatal Post-Traumatic Stress Disorder Questionnaire (PPQ) was completed by parents three months after discharge.
    RESULTS: One hundred ninety-four dyads participated in the study, 86% of parents completed follow up screening. 25% of parents screened positive for PTSD. Parents perceived infants to be sick more often than hospital caregivers. In bivariate analysis many objective measures of illness severity were associated with PTSD. Parent perceptions of illness were also associated with PTSD after adjusting for objective measures of illness (OR 3.2, 95% CI 1.1-6.1, p = 0.008).
    CONCLUSIONS: PTSD in parents after NICU discharge is multifactorial. Objective illness risk factors can be used to screen parents at risk. Hospital caregivers should strive to understand parents' perception of illness and improve communication to potentially decrease PTSD after discharge.
    Keywords:  Infant illness; NICU; Perceptions of illness; Perinatal PTSD
    DOI:  https://doi.org/10.1016/j.earlhumdev.2019.104930