bims-hylehe Biomed News
on Hypoplastic left heart syndrome
Issue of 2020–04–05
eight papers selected by
Richard James, University of Pennsylvania
  1. Promoting Parent Partnership in Developmentally Supportive Care for Infants in the Pediatric Cardiac Intensive Care Unit.
  2. Heart University: a new online educational forum in paediatric and adult congenital cardiac care. The future of virtual learning in a post-pandemic world?
  3. The Creation of a Pediatric Health Care Learning Network: The ACTION Quality Improvement Collaborative.
  4. Palliation but not cure - Meeting the lifetime needs of Fontan patients.
  5. Does routine fenestration improve early and late postoperative outcomes in patients undergoing Fontan palliation?
  6. In-hospital interstage improves interstage survival after the Norwood stage 1 operation.
  7. Prevalence and Cause of Early Fontan Complications: Does the Lymphatic Circulation Play a Role?
  8. Impacting child health outcomes in congenital heart disease: Cluster randomized controlled trial protocol of in-clinic physical activity counselling.
  1. Adv Neonatal Care. 2020 Apr;20(2): 161-170
    Promoting Parent Partnership in Developmentally Supportive Care for Infants in the Pediatric Cardiac Intensive Care Unit.
    Janie Klug, Christine Hall, Emily A Delaplane, Caitlin Meehan, Karli Negrin, Dana Mieczkowski, Sarah K Russell, Bridy O Hamilton, David A Hehir, Erica Sood.
       BACKGROUND: Limited opportunities for parents to care for their critically ill infant after cardiac surgery can lead to parental unpreparedness and distress.
    PURPOSE: This project aimed to create and test a bedside visual tool to increase parent partnership in developmentally supportive infant care after cardiac surgery.
    METHODS: The Care Partnership Pyramid was created by a multidisciplinary team and incorporated feedback from nurses and parents. Three Plan-Do-Study-Act (PDSA) cycles tested its impact on parent partnership in care. Information about developmentally supportive care provided by parents during each 12-hour shift was extracted from nursing documentation. A staff survey evaluated perceptions of the tool and informed modifications.
    RESULTS: Changes in parent partnership during PDSA 1 did not reach statistical significance. Staff perceived that the tool was generally useful for the patient/family but was sometimes overlooked, prompting its inclusion in the daily goals checklist. For PDSA 2 and 3, parents were more often observed participating in rounds, asking appropriate questions, providing environmental comfort, assisting with the daily care routine, and changing diapers.
    IMPLICATIONS FOR PRACTICE: Use of a bedside visual tool may lead to increased parent partnership in care for infants after cardiac surgery.
    IMPLICATIONS FOR RESEARCH: Future projects are needed to examine the impact of bedside care partnership interventions on parent preparedness, family well-being, and infant outcomes.
    DOI:  https://doi.org/10.1097/ANC.0000000000000679
  2. Cardiol Young. 2020 Mar 31. 1-21
    Heart University: a new online educational forum in paediatric and adult congenital cardiac care. The future of virtual learning in a post-pandemic world?
    Justin T Tretter, Jonathan Windram, Theresa Faulkner, Michelle Hudgens, Skaiste Sendzikaite, Nico A Blom, Katarina Hanseus, Rohit S Loomba, Colin J McMahon, Bistra Zheleva, R Krishna Kumar, Jeffrey P Jacobs, Erwin N Oechslin, Gary D Webb, Andrew N Redington.
      Online learning has become an increasingly expected and popular component for education of the modern-day adult learner, including the medical provider. In light of the recent coronavirus pandemic, there has never been more urgency to establish opportunities for supplemental online learning. Heart University aims to be 'the go-to online resource' for e-learning in congenital heart disease and paediatric acquired heart disease. It is a carefully-curated open access library of pedagogical material for all providers of care to children and adults with congenital heart disease or children with acquired heart disease, whether a trainee or a practicing provider. In this manuscript, we review the aims, development, current offerings and standing, and future goals of Heart University.
    Keywords:  adult congenital heart disease; congenital heart disease; medical education; online learning
    DOI:  https://doi.org/10.1017/S1047951120000852
  3. ASAIO J. 2020 Apr;66(4): 441-446
    The Creation of a Pediatric Health Care Learning Network: The ACTION Quality Improvement Collaborative.
    Angela Lorts, Lauren Smyth, Robert J Gajarski, Christina J VanderPluym, Mary Mehegan, Chet R Villa, Jenna M Murray, Robert A Niebler, Christopher S Almond, Philip Thrush, Matthew J O'Connor, Jennifer Conway, David L Sutcliffe, Jodi E Lantz, Farhan Zafar, David L S Morales, David M Peng, David N Rosenthal.
      Improving the outcomes of pediatric patients with congenital heart disease with end-stage heart failure depends on the collaboration of all stakeholders; this includes providers, patients and families, and industry representatives. Because of the rarity of this condition and the heterogeneity of heart failure etiologies that occur at pediatric centers, learnings must be shared between institutions and all disciplines to move the field forward. To foster collaboration, excel discovery, and bring data to the bedside, a new, collaborative quality improvement science network-ACTION (Advanced Cardiac Therapies Improving Outcomes Network)-was developed to meet the needs of the field. Existing gaps in care and the methods of improvement that will be used are described, along with the mission and vision, utility of real-world data for regulatory purposes, and the organizational structure of ACTION is described.
    DOI:  https://doi.org/10.1097/MAT.0000000000001133
  4. Ann Thorac Surg. 2020 Mar 27. pii: S0003-4975(20)30423-9. [Epub ahead of print]
    Palliation but not cure - Meeting the lifetime needs of Fontan patients.
    Kurt R Schumacher.
      
    DOI:  https://doi.org/10.1016/j.athoracsur.2020.02.049
  5. Interact Cardiovasc Thorac Surg. 2020 Apr 03. pii: ivaa002. [Epub ahead of print]
    Does routine fenestration improve early and late postoperative outcomes in patients undergoing Fontan palliation?
    Alexandra Toncu, Cristina Ramona Rădulescu, Dan Dorobanţu, Șerban Stoica.
      A best evidence topic in cardiac surgery was written according to a structured protocol. The question addressed was: 'In [patients undergoing Fontan palliation] does [fenestration] affect [early and late postoperative outcomes]?' Altogether 509 papers were found using the reported search, of which 11 papers represented the best evidence to answer the clinical question. The authors, journal, date and country of publication, patient group studied, study type, relevant outcomes and results of these papers are tabulated. Current data suggest that the use of fenestration has advantages in the immediate postoperative course, with fewer complications such as pleural effusions, shorter hospitalization and decreased early Fontan failure, but comparable long-term outcomes to a non-fenestrated approach. Fenestration should be used in high-risk patients or based on the haemodynamic parameters measured before weaning from cardiopulmonary bypass. Routine use may potentially lead to additional late fenestration closure procedures in some patients, without improving long-term outcomes.
    Keywords:  Fenestration; Fontan operation; Total cavopulmonary connection
    DOI:  https://doi.org/10.1093/icvts/ivaa002
  6. Eur J Cardiothorac Surg. 2020 Apr 01. pii: ezaa074. [Epub ahead of print]
    In-hospital interstage improves interstage survival after the Norwood stage 1 operation.
    Guido Michielon, Giovanni DiSalvo, Alain Fraisse, Julene S Carvalho, Sylvia Krupickova, Zdenek Slavik, Margarita Bartsota, Pierce Daubeney, Carles Bautista, Ajay Desai, Margarita Burmester, Duncan Macrae.
       OBJECTIVES: The interstage mortality rate after a Norwood stage 1 operation remains 12-20% in current series. In-hospital interstage facilitates escalation of care, possibly improving outcome.
    METHODS: A retrospective study was designed for hypoplastic left heart syndrome (HLHS) and HLHS variants, offering an in-hospital stay after the Norwood operation until the completion of stage 2. Daily and weekly examinations were conducted systematically, including two-dimensional and speckle-tracking echocardiography. Primary end points included aggregate survival until the completion of stage 2 and interstage freedom from escalation of care. Moreover, we calculated the sensitivity and specificity of speckle-tracking echocardiographic myocardial deformation in predicting death/transplant after the Norwood procedure.
    RESULTS: Between 2015 and 2019, 33 neonates with HLHS (24) or HLHS variants (9) underwent Norwood stage 1 (31) or hybrid palliation followed by a comprehensive stage 2 operation (2). Stage 1 Norwood-Sano was preferred in 18 (54.5%) neonates; the classic Norwood with Blalock-Taussig shunt was performed in 13 (39.4%) neonates. The Norwood stage 1 30-day mortality rate was 6.2%. The in-hospital interstage strategy was implemented after Norwood stage 1 with a 3.4% interstage mortality rate. The aggregate Norwood stage 1 and interstage Kaplan-Meier survival rate was 90.6 ± 5.2%. Escalation of care was necessary for 5 (17.2%) patients at 2.5 ± 1.2 months during the interstage for compromising atrial arrhythmias (2), Sano-shunt stenosis (1) and pneumonia requiring a high-frequency oscillator (2); there were no deaths. A bidirectional Glenn (25) or a comprehensive-Norwood stage 2 (2) was completed in 27 patients at 4.7 ± 1.2 months with a 92.6% survival rate. The overall Kaplan-Meier survival rate is 80.9 ± 7.0% at 4.3 years (mean 25.3 ± 15.7 months). An 8.7% Δ longitudinal strain 30 days after Norwood stage 1 had 100% sensitivity and 81% specificity for death/transplant.
    CONCLUSIONS: In-hospital interstage facilitates escalation of care, which seems efficacious in reducing interstage Norwood deaths. A significant reduction of longitudinal strain after Norwood stage 1 is a strong predictor of poor outcome.
    Keywords:  Congenital; Hypoplastic left heart syndrome; Interstage
    DOI:  https://doi.org/10.1093/ejcts/ezaa074
  7. J Am Heart Assoc. 2020 Apr 07. 9(7): e015318
    Prevalence and Cause of Early Fontan Complications: Does the Lymphatic Circulation Play a Role?
    Reena M Ghosh, Heather M Griffis, Andrew C Glatz, Jonathan J Rome, Christopher L Smith, Matthew J Gillespie, Kevin K Whitehead, Michael L O'Byrne, David M Biko, Chitra Ravishankar, Aaron G Dewitt, Yoav Dori.
      Background Recent studies suggest that lymphatic congestion plays a role in development of late Fontan complications, such as protein-losing enteropathy. However, the role of the lymphatic circulation in early post-Fontan outcomes is not well defined. Methods and Results This was a retrospective, single-center study of patients undergoing first-time Fontan completion from 2012 to 2017. The primary outcome was early Fontan complication ≤6 months after surgery, a composite of death, Fontan takedown, extracorporeal membrane oxygenation, chest tube drainage >14 days, cardiac catheterization, readmission, or transplant. Complication causes were assigned to 1 of 4 groups: (1) Fontan circuit obstruction, (2) ventricular dysfunction or atrioventricular valve regurgitation, (3) persistent pleural effusions in the absence of Fontan obstruction or ventricular dysfunction, and (4) chylothorax or plastic bronchitis. T2-weighted magnetic resonance imaging sequences were used to assess for lymphatic perfusion abnormality. The cohort consisted of 238 patients. Fifty-eight (24%) developed early complications: 20 of 58 (34.5%) in group 1, 8 of 58 (14%) in group 2, 18 of 58 (31%) in group 3, and 12 of 58 (20%) in group 4. Preoperative T2 imaging was available for 126 (53%) patients. Patients with high-grade lymphatic abnormalities had 6 times greater odds of developing early complications (P=0.001). Conclusions There is substantial morbidity in the early post-Fontan period. Half of those who developed early complications had lymphatic failure or persistent effusions unrelated to structural or functional abnormalities. Preoperative T2 imaging demonstrated that patients with higher-grade lymphatic perfusion abnormalities were significantly more likely to develop early complications. This has implications for risk stratification and optimization of patients before Fontan palliation.
    Keywords:  Fontan procedure; cardiovascular magnetic resonance imaging; congenital heart disease; lymph; morbidity/mortality
    DOI:  https://doi.org/10.1161/JAHA.119.015318
  8. Contemp Clin Trials. 2020 Mar 25. pii: S1551-7144(20)30072-0. [Epub ahead of print] 105994
    Impacting child health outcomes in congenital heart disease: Cluster randomized controlled trial protocol of in-clinic physical activity counselling.
    Olivia Lemire, Jenna Yaraskavitch, Jane Lougheed, Andrew S Mackie, Kambiz Norozi, Patricia E Longmuir.
       BACKGROUND: Most (>90%) children with congenital health defects are not active enough for optimal health. Proactively promoting physical activity during every clinic visit is recommended, but rarely implemented due to a lack of appropriate resources.
    METHODS: This cluster randomized controlled trial will implement an evidence-based, multi-faceted physical activity intervention. All eligible patients at small (London, ON), medium (Ottawa, ON) and large (Edmonton, AB) pediatric cardiac clinics will be approached, with randomization to intervention/control by clinic and week. Intervention patients will be counselled with 5 key physical activity messages, have questions about physical activity answered, and have access to a custom web site with personalized activity suggestions and support from a Registered Kinesiologist. The primary outcome is daily physical activity (number of steps, minutes of moderate-to-vigorous activity) assessed via pedometer one week per month for 6-months. Standardized questionnaires assess activity motivation and quality of life at baseline and end of study. Healthcare outcomes will be clinic visit time and contacts for physical activity concerns. Repeated measures ANCOVA will compare control/intervention pedometer outcomes, adjusting for covariates (alpha = 0.05).
    CONCLUSIONS: This trial aims to determine whether providing resources and protocols enables clinicians to counsel about physical activity as part of every pediatric cardiology appointment. Evaluations of healthcare system impact and intervention delivery in small, medium and large clinics will assess applicability for implementation in all pediatric cardiac clinics. The impact on physical activity motivation and participation will evaluate the effectiveness of this standardized approach for increasing physical activity in children with congenital health defects.
    Keywords:  Adolescent; Exercise; Health promotion; Innocent heart murmur; Pragmatic trial
    DOI:  https://doi.org/10.1016/j.cct.2020.105994
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