bims-hylehe Biomed news
on Hypoplastic left heart syndrome
Issue of 2019‒01‒06
six papers selected by
Richard James
University of Pennsylvania


  1. J Matern Fetal Neonatal Med. 2019 Jan 04. 1-6
    Tsintoni A, Dimitriou G, Karatza AA.
      INTRODUCTION: Congenital heart disease is one of the most of the groups of congenital anomalies with an incidence of about 1 per 100 live births. Almost one-third of these infants require some type of intervention, usually in the first year of life and increasingly often in the neonatal period. Innovative reparative and palliative surgical procedures and advanced medical support in the Neonatal Intensive Care Unit have significantly reduced the mortality related to congenital heart disease. Achieving survival is not the only target of clinicians for these patients. Appropriate growth, development, and improved quality of life are also very important. Growth failure is a very common problem of these children and nutritional support and management are a challenge for health care providers. Early intervention and identification of at-risk patients have the potential to decrease morbidity and mortality related to malnutrition.AIM/METHODS: The purpose of this article is to analyze the existing evidence and common concerns about perioperative and postdischarge nutritional management of neonates with congenital heart disease based on the special issues or complications that may arise. Furthermore, we reviewed the recent literature about current practices and proposed policies that could prevent malnutrition and improve the outcomes of neonates with congenital heart disease.
    RESULTS/CONCLUSION: A standardized institutional protocol and clear guidelines referring to feeding initiation, prompt estimation of caloric needs and provision of adequate and appropriate nutrient intake is likely to benefit these patients. Clear definitions for the nutritional approach in the setting of medical complications and close assessment of growth by pediatricians and specialized nutritionists are crucial for the long-term outlook and quality of life of these infants.
    Keywords:  Congenital; feedings; infants
    DOI:  https://doi.org/10.1080/14767058.2018.1548602
  2. Congenit Heart Dis. 2019 Jan 03.
    Hoerst A, Bakar A, Cassidy SC, Clabby M, Grippo ED, Graupe M, Harahsheh AS, Hlavacek AM, Hart SA, Kipps AK, Madsen NL, O'Neil DD, Patel SS, Strohacker CM, Tanel RE, .
      BACKGROUND: The Pediatric Acute Care Cardiology Collaborative (PAC3 ) was established in 2014 to improve the quality, value, and experience of hospital-based cardiac acute care outside of the intensive care unit. An initial PAC3 project was a comprehensive survey to understand unit structure, practices, and resource utilization across the collaborative. This report aims to describe the previously unknown degree of practice variation across member institutions.METHODS: A 126-stem question survey was developed with a total of 412 possible response fields across nine domains including demographics, staffing, available resources and therapies, and standard care practices. Five supplemental questions addressed surgical case volume and number of cardiac acute care unit (CACU) admissions. Responses were recorded and stored in Research Electronic Data Capture (REDCap).
    RESULTS: Surveys were completed by 31 out of 34 centers (91%) with minimal incomplete fields. A majority (61%) of centers have a single dedicated CACU, which is contiguous or adjacent to the intensive care unit in 48%. A nurse staffing ratio of 3:1 is most common (71%) and most (84%) centers employed a resource nurse. Centralized wireless rhythm monitoring is used in 84% of centers with 54% staffed continuously. There was significant variation in the use of noninvasive respiratory support, vasoactive infusions, and ventricular assist devices across the collaborative. Approximately half of the surveyed centers had lesion-specific postoperative pathways and approximately two-thirds had protocols for single-ventricle patients.
    CONCLUSIONS: The PAC3 hospital survey is the most comprehensive description of systems and care practices unique to CACUs to date. There exists considerable heterogeneity among unit composition and variation in care practices. These variations may allow for identification of best practices and improved quality of care for patients.
    Keywords:  cardiovascular care unit; inpatient cardiology; pediatric cardiology; quality improvement
    DOI:  https://doi.org/10.1111/chd.12739
  3. Pediatr Transplant. 2019 Jan 01. e13349
    Kirk R, Butts RJ, Dipchand AI.
      BACKGROUND: The first pediatric heart transplant died 5 hours after transplant and the case was published by Kantrowitz. There is no report of the first successful case in the medical literature, nor indeed the outcome of children transplanted prior to 1982. However, we recently discovered that children from this period were entered retrospectively into the ISHLT Registry when it began and they form the basis of this report.METHODS: A retrospective review of the ISHLT Thoracic Registry was undertaken for pediatric heart transplants prior to 1982. Demographic and descriptive data, and patient and graft survival were analyzed.
    RESULTS: Thirty children (24 male) had a median age of 13 years (IQR 12-16) at the time of primary transplant. The underlying cardiac diagnosis was cardiomyopathy (18), congenital heart disease (7), not reported (5). The median follow-up was 2.63 years (IQR 0.1-7.2). Twenty-two patients are known to have died, and eight underwent retransplantation. Median patient survival was 3.5 years. The first patient to survive for more than one year was transplanted in 1968 and survived 6 years.
    CONCLUSION: The definition of a successful transplant is debatable; however, the first child reported to the registry to survive beyond one year was transplanted in 1968. Survival for these early patients was considerably less than the subsequent eras and retransplantation more common; however, the experience in managing these children laid the foundation for the future.
    Keywords:  first; heart transplant; outcomes; pediatric
    DOI:  https://doi.org/10.1111/petr.13349
  4. Ann Thorac Surg. 2018 Dec 31. pii: S0003-4975(18)31867-8. [Epub ahead of print]
    Huygens SA, Rutten-van Mölken MPMH, Noruzi A, Etnel JRG, Ramos IC, Bouten CVC, Kluin J, Takkenberg JJM.
      BACKGROUND: As a living heart valve substitute with growth potential and improved durability, tissue-engineered heart valves (TEHV) may prevent re-interventions that are currently often needed in children with congenital heart disease. We performed early Health Technology Assessment to assess the potential cost-effectiveness of TEHV in children requiring right ventricular outflow tract reconstruction (RVOTR).METHODS: A systematic review and meta-analysis was conducted of studies reporting clinical outcome after RVOTR with existing heart valve substitutes in children (mean age ≤12 and/or maximum age≤21 years) published between 1/1/2000-2/5/2018. Using a patient-level simulation model, costs and effects of RVOTR with TEHV compared to existing heart valve substitutes were assessed from a healthcare perspective applying a 10-year time horizon. Improvements in performance of TEHV, divided in durability, thrombogenicity, and infection resistance, were explored to estimate quality-adjusted life years (QALY) gain, cost reduction, headroom, and budget impact associated with TEHV.
    RESULTS: Five-year freedom from re-intervention after RVOTR with existing heart valve substitutes was 46.1% in patients ≤2 years old and 81.1% in patients >2 years old. Improvements in durability had the highest impact on QALYs and costs. In the 'improved TEHV performance' scenario (durability≥5 years and -50% other valve-related events), QALY gain was 0.074 and cost reduction was €10,378 per patient, translating to maximum additional costs of €11,856 per TEHV compared to existing heart valve substitutes.
    CONCLUSIONS: This study showed that there is room for improvement in clinical outcomes in children requiring RVOTR. If TEHV result in improved clinical outcomes, they are expected to be cost-effective compared to existing heart valve substitutes.
    DOI:  https://doi.org/10.1016/j.athoracsur.2018.11.066
  5. Curr Opin Obstet Gynecol. 2019 Jan 02.
    Qiao F, Hu P, Xu Z.
      PURPOSE OF REVIEW: Congenital heart defects (CHDs) are the most common type of birth defects, and are thought to result from genetic-environmental interactions. Currently, karyotype and chromosomal microarray analyses are the primary methods used to detect chromosomal abnormalities and copy number variations in fetuses with CHD. Recently, with the introduction of next-generation sequencing (NGS) in prenatal diagnosis, gene mutations have been identified in cases of CHD. The purpose of this review is to summarize current studies about the genetic cause of fetal CHD, paying particular attention to the application of NGS for fetuses with CHD.RECENT FINDINGS: In addition to chromosomal abnormalities, gene mutations are an important genetic cause of fetal CHD. Furthermore, incidences of pathogenic mutations in fetuses with CHD are associated with the presence of other structural anomalies, but are irrelevant to the categories of CHD.
    SUMMARY: Gene mutations are important causes of fetal CHD and NGS should be applied to all fetuses with normal karyotype and copy number variations, regardless of whether the CHD is isolated or syndromic.
    DOI:  https://doi.org/10.1097/GCO.0000000000000520
  6. Cardiol Young. 2019 Jan 02. 1-7
    Heye KN, Knirsch W, Scheer I, Beck I, Wetterling K, Hahn A, Hofmann K, Latal B, Reich B, Landolt MA.
      BACKGROUND: Little is known about health-related quality of life in young children undergoing staged palliation for single-ventricle CHD. The aim of this study was to assess the impact of CHD on daily life in pre-schoolers with single-ventricle CHD and to identify determinants of health-related quality of life.METHOD: Prospective two-centre cohort study assessing health-related quality of life using the Preschool Paediatric Cardiac Quality of Life Inventory in 46 children at a mean age of 38 months and 3 weeks. Children with genetic anomalies were excluded. Scores were compared with reference data of children with biventricular CHD. Multiple linear regression analysis was used to identify determinants of health-related quality of life.
    RESULTS: Health-related quality of life in pre-schoolers with single-ventricle CHD was comparable to children with biventricular CHD. Preterm birth and perioperative variables were significant predictors of low health-related quality of life. Notably, pre-Fontan brain MRI findings and neurodevelopmental status were not associated with health-related quality of life. Overall, perioperative variables explained 24% of the variability of the total health-related quality of life score.InterpretationDespite substantial health-related burden, pre-schoolers with single-ventricle CHD showed good health-related quality of life. Less-modifiable treatment-related risk factors and preterm birth had the highest impact on health-related quality of life. Long-term follow-up assessment of self-reported health-related quality of life is needed to identify patients with poorer health-related quality of life and to initiate supportive care.
    Keywords:  CHD; Health-related quality of life; risk factors; single-ventricle
    DOI:  https://doi.org/10.1017/S1047951118001993