bims-hylehe Biomed News
on Hypoplastic left heart syndrome
Issue of 2020–07–12
nine papers selected by
Richard James, University of Pennsylvania
  1. The Fontan outcomes network: first steps towards building a lifespan registry for individuals with Fontan circulation in the United States.
  2. Characteristics of Adults With Congenital Heart Defects in the United States.
  3. Emerging 3D technologies and applications within congenital heart disease: teach, predict, plan and guide.
  4. State of Cardiac Implantable Electronic Devices in Congenital Heart Disease: A 2019 Update.
  5. Associations Between Postoperative Management in the Critical Care Unit and Adolescent Developmental Outcomes Following Cardiac Surgery in Infancy: An Exploratory Study.
  6. An Analysis of Hospital Mortality After Cardiac Operations in Children with Down Syndrome.
  7. Genetics in congenital heart disease. Are we ready for it?
  8. Fathers of Children With Congenital Heart Disease: Sources of Stress and Opportunities for Intervention.
  9. Influence of Early Extubation Location on Outcomes Following Pediatric Cardiac Surgery.
  1. Cardiol Young. 2020 Jul 08. 1-6
    The Fontan outcomes network: first steps towards building a lifespan registry for individuals with Fontan circulation in the United States.
    Tarek Alsaied, Kiona Y Allen, Jeffrey B Anderson, Julia S Anixt, David W Brown, Frank Cetta, Rachael Cordina, Yves D'udekem, Meghan Didier, Salil Ginde, Michael V Di Maria, Michelle Eversole, David Goldberg, Bryan H Goldstein, Erin Hoffmann, Adrienne H Kovacs, Carole Lannon, Stacey Lihn, Adam M Lubert, Bradley S Marino, Emily Mullen, Diane Pickles, Rahul H Rathod, Jack Rychik, James S Tweddell, Sharyl Wooton, Gail Wright, Adel Younoszai, Kurt Schumacher.
      The Fontan Outcomes Network was created to improve outcomes for children and adults with single ventricle CHD living with Fontan circulation. The network mission is to optimise longevity and quality of life by improving physical health, neurodevelopmental outcomes, resilience, and emotional health for these individuals and their families. This manuscript describes the systematic design of this new learning health network, including the initial steps in development of a national, lifespan registry, and pilot testing of data collection forms at 10 congenital heart centres.
    Keywords:  Fontan; improving outcomes; registry; single ventricle
    DOI:  https://doi.org/10.1017/S1047951120001869
  2. J Am Coll Cardiol. 2020 Jul 14. pii: S0735-1097(20)35313-4. [Epub ahead of print]76(2): 175-182
    Characteristics of Adults With Congenital Heart Defects in the United States.
    Michelle Gurvitz, Julie E Dunn, Ami Bhatt, Wendy M Book, Jill Glidewell, Carol Hogue, Angela E Lin, George Lui, Claire McGarry, Cheryl Raskind-Hood, Alissa Van Zutphen, Ali Zaidi, Kathy Jenkins, Tiffany Riehle-Colarusso.
       BACKGROUND: In the United States, >1 million adults are living with congenital heart defects (CHDs), but gaps exist in understanding the health care needs of this growing population.
    OBJECTIVES: This study assessed the demographics, comorbidities, and health care use of adults ages 20 to 64 years with CHDs.
    METHODS: Adults with International Classification of Disease-9th Revision-Clinical Modification CHD-coded health care encounters between January 1, 2008 (January 1, 2009 for Massachusetts) and December 31, 2010 were identified from multiple data sources at 3 U.S. sites: Emory University (EU) in Atlanta, Georgia (5 counties), Massachusetts Department of Public Health (statewide), and New York State Department of Health (11 counties). Demographics, insurance type, comorbidities, and encounter data were collected. CHDs were categorized as severe or not severe, excluding cases with isolated atrial septal defect and/or patent foramen ovale.
    RESULTS: CHD severity and comorbidities varied across sites, with up to 20% of adults having severe CHD and >50% having ≥1 additional cardiovascular comorbidity. Most adults had ≥1 outpatient encounters (80% EU, 90% Massachusetts, and 53% New York). Insurance type differed across sites, with Massachusetts having a large proportion of Medicaid (75%) and EU and New York having large proportions of private insurance (44% EU, 67% New York). Estimated proportions of adults with CHD-coded health care encounters varied greatly by location, with 1.2 (EU), 10 (Massachusetts), and 0.6 (New York) per 1,000 adults based on 2010 census data.
    CONCLUSIONS: This was the first surveillance effort of adults with CHD-coded inpatient and outpatient health care encounters in 3 U.S. geographic locations using both administrative and clinical data sources. This information will provide a clearer understanding of health care use in this growing population.
    Keywords:  adult congenital heart disease; epidemiology; surveillance
    DOI:  https://doi.org/10.1016/j.jacc.2020.05.025
  3. Future Cardiol. 2020 Jul 06.
    Emerging 3D technologies and applications within congenital heart disease: teach, predict, plan and guide.
    Arash Salavitabar, C Alberto Figueroa, Jimmy C Lu, Sonal T Owens, David M Axelrod, Jeffrey D Zampi.
      3D visualization technologies have evolved to become a mainstay in the management of congenital heart disease (CHD) with a growing presence within multiple facets. Printed and virtual 3D models allow for a more comprehensive approach to educating trainees and care team members. Computational fluid dynamics can take 3D modeling to the next level, by predicting post-procedural outcomes and helping to determine surgical approach. 3D printing and extended reality are developing resources for pre-procedural planning and intra-procedural guidance with the potential to revolutionize decision-making and procedural success. Challenges still remain within existing technologies and their applications to the CHD field. Addressing these gaps, both by those within and outside of CHD, will transform education and patient care within our field.
    Keywords:  3D printing; 3D rotational angiography; 3D visualization; augmented reality; congenital heart disease; virtual reality
    DOI:  https://doi.org/10.2217/fca-2020-0004
  4. J Innov Card Rhythm Manag. 2019 Dec;10(12): 3940-3943
    State of Cardiac Implantable Electronic Devices in Congenital Heart Disease: A 2019 Update.
    Johannes C von Alvensleben, Kathryn K Collins.
      
    Keywords:  Congenital heart disease; defibrillation; pacing; pediatrics
    DOI:  https://doi.org/10.19102/icrm.2019.101207
  5. Pediatr Crit Care Med. 2020 Jun 26.
    Associations Between Postoperative Management in the Critical Care Unit and Adolescent Developmental Outcomes Following Cardiac Surgery in Infancy: An Exploratory Study.
    Allison H Shevell, Sossy K Sahakian, Quynh Nguyen, Patricia Fontela, Charles Rohlicek, Annette Majnemer.
       OBJECTIVES: Children with congenital heart disease are at high risk for developmental sequelae. Most studies focus on preoperative and intraoperative predictors of developmental impairment, with less attention to the postoperative period. The relationship between patient-related factors specific to the postoperative course in the PICU following cardiac surgery with long-term neurodevelopmental outcomes in adolescence was examined.
    DESIGN: Retrospective chart review of patients previously recruited to a study describing their developmental outcomes in adolescence.
    SETTING: Single tertiary care pediatric hospital in Canada.
    PATIENTS: Eighty adolescents, born between 1991 and 1999, with congenital heart disease who required open-heart surgery before 2 years old.
    MEASUREMENTS AND MAIN RESULTS: Several variables related to acuity of illness and complexity of postoperative course in the PICU were collected. Outcome measures included the Movement-Assessment Battery for Children-2 (motor), Leiter Brief Intelligence Quotient (cognition), and Strength and Difficulties Questionnaire (behavior). Analyses examined associations between PICU variables and long-term outcomes. Longer mechanical ventilation (β = -0.49; p = 0.013) and dopamine use (β = -14.41; p = 0.012) were associated with lower motor scores. Dopamine use was associated with lower cognitive scores (β = -14.02; p = 0.027). Longer PICU stay (β = 0.18; p = 0.002), having an open chest postoperatively (β = 3.83; p = 0.017), longer mechanical ventilation (β = 0.20; p = 0.001), longer inotrope support (β = 0.27; p = 0.002), hours on dopamine (β = 0.01; p = 0.007), days to enteral feeding (β = 0.22; p = 0.012), lower hemoglobin (β = -0.11; p = 0.004), and higher creatinine (β = 0.05; p = 0.014) were all associated with behavioral difficulties.
    CONCLUSIONS: Several important developmental outcomes in adolescents were associated with factors related to their postoperative course in the PICU as infants. Findings may highlight those children at highest risk for neurodevelopmental sequelae and suggest new approaches to critical care management following open-heart surgery, with the aim of mitigating or preventing adverse long-term outcomes.
    DOI:  https://doi.org/10.1097/PCC.0000000000002398
  6. Semin Thorac Cardiovasc Surg. 2020 Jul 01. pii: S1043-0679(20)30228-8. [Epub ahead of print]
    An Analysis of Hospital Mortality After Cardiac Operations in Children with Down Syndrome.
    Gurpreet S Dhillon, Nancy S Ghanayem, Christopher R Broda, Seema R Lalani, Carlos M Mery, Lara S Shekerdemian, Steven J Staffa, Shaine A Morris.
      Children with Down syndrome (DS) have lower mortality compared to non-syndromic (NS) children after atrioventricular septal defect (AVSD) repair. Limited data exist regarding hospital mortality and utilization after other congenital heart disease (CHD) operations in DS. We compared hospital mortality and utilization after CHD operations in both populations and hypothesized that the survival benefit in children with DS is not consistent across CHD lesions. The Texas Inpatient Public Use Datafile was queried for all patients <18 years old undergoing operations for CHD between 1999-2016. Hospital mortality, length-of-stay and charges were compared between DS and NS groups, stratified by CHD operation using mixed-effects multivariable analyses and propensity score matching (PSM) analyses adjusting for prematurity, low birth weight, age, and sex. Over the 18-year period, 2841 cases with DS underwent CHD operations compared to 25063 NS cases. The most common types of interventions performed in DS were AVSD repair, isolated ventricular septal defect (VSD) repair and tetralogy of Fallot (TOF) repair. By multivariable analyses, DS was associated with lower mortality after isolated AVSD repair (RR 0.40[IQR 0.20-0.79]), and higher hospital mortality after bidirectional Glenn anastomosis (BDG) (RR 5.17[IQR2.10-12.77]) and TOF/pulmonary atresia repair (RR 9.71[IQR2.16-43.68]) compared to NS children. Similar results were noted using PSM. Children with DS had lower mortality after AVSD repair than NS children, but higher mortality after operations for BDG and TOF/pulmonary atresia. Further study is needed to determine if the presence of pulmonary hypertension in DS modifies the association between DS and mortality depending on cardiac lesion.
    DOI:  https://doi.org/10.1053/j.semtcvs.2020.06.037
  7. Rev Esp Cardiol (Engl Ed). 2020 Jul 06. pii: S1885-5857(20)30226-7. [Epub ahead of print]
    Genetics in congenital heart disease. Are we ready for it?
    Julie De Backer, Bert Callewaert, Laura Muiño Mosquera.
      Genetics has rightly acquired an important place in almost all medical disciplines in recent years and this is certainly the case in the field of congenital cardiology. Not only has this led to greater insight into the pathophysiology of congenital heart defects but it also has a beneficial impact on patient management. Integration of clinical genetics in multidisciplinary centers of expertise for CHD is therefore a clear recommendation. Adult and pediatric cardiologists play a crucial role in the process of genetic evaluation of patients and families and should have be familiar with red flags for referral for further clinical genetic elaboration, counseling, and eventual testing. Some basic knowledge is also important for the correct interpretation of genetic testing results. In this review article, we provide a practical overview of what genetic evaluation entails, which type of genetic tests are possible today, and how this can be used in practice for the individual patient.
    Keywords:  Análisis genético; Cardiopatías congénitas; Congenital heart disease; Consejo genético; Genetic counseling; Genetic testing
    DOI:  https://doi.org/10.1016/j.rec.2020.05.019
  8. Pediatr Crit Care Med. 2020 Jun 26.
    Fathers of Children With Congenital Heart Disease: Sources of Stress and Opportunities for Intervention.
    Michael F Hoffman, Allison Karpyn, Jennifer Christofferson, Trent Neely, Linda G McWhorter, Abigail C Demianczyk, Richard James Mslis, Jason Hafer, Anne E Kazak, Erica Sood.
       OBJECTIVES: To examine sources of stress for fathers of children with congenital heart disease and opportunities for intervention to prevent or reduce paternal mental health problems.
    DESIGN: Qualitative study using online crowdsourcing, an innovative research methodology to create an online community to serve as a research sample.
    SETTING: Yammer, an online social networking site.
    SUBJECTS: Geographically diverse sample of 70 parents (25 fathers and 45 mothers) of young children with congenital heart disease.
    INTERVENTIONS: Participants joined a private group on Yammer and responded to 37 open-ended study questions over a 6-month period. Qualitative data were coded and analyzed using an iterative process, and themes regarding sources of stress for fathers of children with congenital heart disease and opportunities for intervention were identified.
    MEASUREMENTS AND MAIN RESULTS: Four broad themes regarding sources of stress for fathers of children with congenital heart disease emerged from the qualitative data from both mothers and fathers: societal expectations for fatherhood and standards of masculinity, balancing work and family responsibilities, feeling overlooked as a partner in care, and lack of father supports. To begin to address these sources of stress, participants recommended that care teams acknowledge and normalize the impact of congenital heart disease on fathers, provide support for balancing work and family responsibilities, recognize and promote father knowledge and engagement, and provide formal and informal supports for fathers of children with congential heart disease.
    CONCLUSIONS: Fathers of children with congenital heart disease experience unique sources of stress in the absence of targeted interventions to meet their needs. Care teams play an important role in acknowledging the experiences of fathers and including and engaging fathers in care.
    DOI:  https://doi.org/10.1097/PCC.0000000000002388
  9. Pediatr Crit Care Med. 2020 Jun 26.
    Influence of Early Extubation Location on Outcomes Following Pediatric Cardiac Surgery.
    Sydney R Rooney, Christopher W Mastropietro, Brian Benneyworth, Eric M Graham, Darren Klugman, John Costello, Nancy Ghanayem, Wenying Zhang, Mousumi Banerjee, Michael Gaies.
       OBJECTIVES: Early extubation following pediatric cardiac surgery is common, but debate exists whether location affects outcome, with some centers performing routine early extubations in the operating room (odds ratio) and others in the cardiac ICU. We aimed to define early extubation practice variation across hospitals and assess impact of location on hospital length-of-stay and other outcomes.
    DESIGN: Secondary analysis of the Pediatric Cardiac Critical Care Consortium registry.
    SETTING: Twenty-eight Pediatric Cardiac Critical Care Consortium hospitals.
    PATIENTS: Patients undergoing Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery mortality category 1-3 operations between August 2014 and February 2018.
    INTERVENTIONS: None.
    MEASUREMENTS AND MAIN RESULTS: We defined early extubation as extubation less than 6 hours after postoperative admission. Hospitals were categorized based on the proportion of their early extubation patients who underwent an odds ratio extubation. Categories included low- (< 50% of early extubation, n = 12), medium- (50%-90%, n = 8), or high- (> 90%, n = 8) frequency odds ratio early extubation centers. The primary outcome of interest was postoperative hospital length-of-stay. We analyzed 16,594 operations (9,143 early extubation, 55%). Rates of early extubation ranged from 16% to 100% across hospitals. Odds ratio early extubation rates varied from 16% to 99%. Patient characteristics were similar across hospital odds ratio early extubation categories. Early extubation rates paralleled the hospital odds ratio early extubation rates-77% patients underwent early extubation at high-frequency odds ratio extubation centers compared with 39% at low-frequency odds ratio extubation centers (p < 0.001). High- and low-frequency odds ratio early extubation hospitals had similar length-of-stay, cardiac arrest rates, and low mortality. However, high-frequency odds ratio early extubation hospitals used more noninvasive ventilation than low-frequency hospitals (15% vs. 9%; p < 0.01), but had fewer extubation failures (3.6% vs. 4.5%; p = 0.02).
    CONCLUSIONS: Considerable variability exists in early extubation practices after low- and moderate-complexity pediatric cardiac surgery. In this patient population, hospital length-of-stay did not differ significantly between centers with different early extubation strategies based on location or frequency.
    DOI:  https://doi.org/10.1097/PCC.0000000000002452
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