bims-hylehe Biomed News
on Hypoplastic left heart syndrome
Issue of 2019‒08‒04
eight papers selected by
Richard James
University of Pennsylvania


  1. Pediatr Cardiol. 2019 Jul 31.
    Fernandes SM, Verstappen A, Clair M, Rummell M, Barber D, Ackerman K, Dummer K, Mares JC, Cannobio MM, Reardon LC, Long J, Crumb S, Bhatt A, Takahashi M, Khairy P, Williams R, Landzberg MJ, Moe T, Pearson D, .
      National management guidelines recommend that patients with moderate and complex congenital heart disease (CHD) receive life-long cardiac care (LLCC), guided in adulthood by an adult congenital heart disease (ACHD) specialist. However, the percentage of adult CHD patients who receive such care is quite low. Inadequate knowledge regarding LLCC may contribute to care interruption. We, therefore, sought to determine the knowledge of adolescents and young adults regarding LLCC. In this multi-center study, we administered a survey to patients 13-20 years of age with surgically repaired congenital heart disease. We assessed the understanding of both their need for LLCC and awareness of the type of recommended care providers. A total of 290/302 (96%) patients approached in the outpatient clinic setting (10 centers) agreed to study participation; mean age was 16.3 ± 2.3 years; patients were 62% male. While the need for LLCC was recognized by 78% of subjects, only 37% understood that an ACHD specialist in adulthood should guide this care. Only 37% of respondents stated that their current cardiology team had spoken to them about LLCC, but 90% wished to learn more. A substantial number of adolescents and young adults with moderate and complex CHD lack adequate knowledge about LLCC, but most have a desire to learn more about the type of care they will require in adulthood. Transition education/assessment ensuring successful transfer to adult-oriented care for this population should emphasize the importance of LLCC.
    Keywords:  Adult congenital heart disease; Congenital heart disease; Health care transition; Pediatric cardiology
    DOI:  https://doi.org/10.1007/s00246-019-02154-8
  2. Open Heart. 2019 ;6(2): e000998
    Verrall CE, Blue GM, Loughran-Fowlds A, Kasparian N, Gecz J, Walker K, Dunwoodie SL, Cordina R, Sholler G, Badawi N, Winlaw D.
      It is established that neurodevelopmental disability (NDD) is common in neonates undergoing complex surgery for congenital heart disease (CHD); however, the trajectory of disability over the lifetime of individuals with CHD is unknown. Several 'big issues' remain undetermined and further research is needed in order to optimise patient care and service delivery, to assess the efficacy of intervention strategies and to promote best outcomes in individuals of all ages with CHD. This review article discusses 'gaps' in our knowledge of NDD in CHD and proposes future directions.
    Keywords:  congenital heart disease; genetics; paediatric cardiology; quality of care and outcomes
    DOI:  https://doi.org/10.1136/openhrt-2018-000998
  3. Congenit Heart Dis. 2019 Aug 02.
    Holst LM, Serrano F, Shekerdemian L, Ravn HB, Guffey D, Ghanayem NS, Monteiro S.
      OBJECTIVE: To investigate the impact of feeding mode on neurodevelopmental outcomes in children with congenital heart defects.DESIGN: A retrospective cohort study of 208 children with congenital heart disease (CHD), who had surgery from 1 January 2013 until 31 December 2016 at Texas Children's Hospital, Houston, TX, US.
    SETTINGS: University Hospital, Developmental Outcome Clinic.
    OUTCOMES MEASURES: Standardized cognitive scores were assessed with Capute Scales and motor development with Revised Gesell Developmental Schedules. We analyzed anthropometrics, mode of feeding, surgical complexity, syndrome, and gender as predictors of developmental outcomes at four time points: hospital discharge, and 6, 12, and 24 months of age.
    RESULTS: Mode of feeding is associated with neurodevelopmental outcome in children with CHD. Children on enteral feeding tubes had significantly lower developmental quotient (DQ) scores in cognition, communication, and motor function at 12 and 24 months compared to orally fed children. There were greater proportions of developmental delays (DQ < 70) in enteral tube fed children at the 6, 12, and 24 months visits. Further, there was a strong association between presence of enteral feeding tube, syndrome, and developmental outcome. Greater surgical complexity, weight gain and ethnicity were not associated with the developmental outcomes.
    CONCLUSIONS: Our findings suggest that the presence of an enteral feeding tube following corrective congenital heart surgery are at increased risk of neurodevelopmental delays at 12 and 24 months.
    Keywords:  cardiac; congenital heart defect; developmental impairment; feeding mode; heart surgery; infants; neurodevelopmental outcome
    DOI:  https://doi.org/10.1111/chd.12827
  4. F1000Res. 2019 ;8 242
    Aranguren Bello HC, Londoño Trujillo D, Troncoso Moreno GA, Dominguez Torres MT, Taborda Restrepo A, Fonseca A, Sandoval Reyes N, Chamorro CL, Dennis Verano RJ.
      Background: Undiagnosed congenital heart disease in the prenatal stage can occur in approximately 5 to 15 out of 1000 live births; more than a quarter of these will have critical congenital heart disease (CCHD). Late postnatal diagnosis is associated with a worse prognosis during childhood, and there is evidence that a standardized measurement of oxygen saturation in the newborn by cutaneous oximetry is an optimal method for the detection of CCHD. We conducted a systematic review of the literature and meta-analysis comparing the operational characteristics of oximetry and physical examination for the detection of CCHD. Methods: A systematic review of the literature was conducted on the following databases including published studies between 2002 and 2017, with no language restrictions: Pubmed, Science Direct, Ovid, Scopus and EBSCO, with the following keywords: oximetry screening, critical congenital heart disease, newborn OR oximetry screening heart defects, congenital, specificity, sensitivity, physical examination. Results: A total of 419 articles were found, from which 69 were selected based on their titles and abstracts. After quality assessment, five articles were chosen for extraction of data according to inclusion criteria; data were analyzed on a sample of 404,735 newborns in the five included studies. The following values were found, corresponding to the operational characteristics of oximetry in combination with the physical examination: sensitivity: 0.92 (CI 95%, 0.87-0.95), specificity: 0.98 (CI 95%, 0.89-1.00), for physical examination alone sensitivity: 0.53 (CI 95%, 0.28-0.78) and specificity: 0.99 (CI 95%, 0.97-1.00). Conclusions: Evidence found in different articles suggests that pulse oximetry in addition to neonatal physical examination presents optimal operative characteristics that make it an adequate screening test for detection of CCHD in newborns, above all this is essential in low and middle-income settings where technology medical support is not entirely available.
    Keywords:  Oximetry; critical congenital heart disease; newborn; physical examination; screening; sensitivity; specificity
    DOI:  https://doi.org/10.12688/f1000research.17989.1
  5. Congenit Heart Dis. 2019 Jul 30.
    Salciccioli KB, Oluyomi A, Lupo PJ, Ermis PR, Lopez KN.
      BACKGROUND: Follow-up at a regional adult congenital heart disease (ACHD) center is recommended for all ACHD patients at least once per the 2018 ACC/AHA guidelines. Other specialties have demonstrated poorer follow-up and outcomes correlating with increased distance from health care providers, but driving time to regional ACHD centers has not been examined in the US population.OBJECTIVE: To identify and characterize potential disparities in access to ACHD care in the US based on drive time to ACHD centers and compounding sociodemographic factors.
    METHODS: Mid- to high-volume ACHD centers with ≥500 outpatient ACHD visits and ≥20 ACHD surgeries annually were included based on self-reported, public data. Geographic Information System mapping was used to delineate drive times to ACHD centers. Sociodemographic data from the 2012-2016 American Community Survey (US Census) and the Environmental Systems Research Institute were analyzed based on drive time to nearest ACHD center. Previously established CHD prevalence estimates were used to estimate the similarly located US ACHD population.
    RESULTS: Nearly half of the continental US population (45.1%) lives >1 hour drive to an ACHD center. Overall, 39.7% live 1-4 hours away, 3.4% live 4-6 hours away, and 2.0% live >6 hours away. Hispanics were disproportionately likely to live a >6 hour drive to a center (p < .001). Compared to people with <1 hour drive, those living >6 hours away have higher proportions of uninsured adults (29% vs. 18%; p < .001), households below the federal poverty level (19% vs. 13%; p < .001), and adults with less than college education (18% vs. 12%; p < .001).
    CONCLUSIONS: We estimate that ~45% of the continental US population lives >1 hour to an ACHD center, with 5.4% living >4 hours away. Compounding barriers exist for Hispanic, uninsured, lower socioeconomic status, and less-educated patients. These results may help drive future policy changes to improve access to ACHD care.
    Keywords:  access to care; adult congenital heart disease; disparities
    DOI:  https://doi.org/10.1111/chd.12819
  6. Nurs Open. 2019 Jul;6(3): 1143-1149
    Hjorth-Johansen E, Hofoss D, Kynø NM.
      Aim: This project aimed to create, implement and evaluate an e-learning course on nursing infants with congenital heart disease (CHD) and to measure its efficacy compared with classroom learning.Design: This is a comparative interventional study with two groups.
    Methods: The study involved 15 postgraduate students and 13 newly employed nurses. The learning outcome was computed as the difference between pre-test and post-test knowledge scores and analysed using t tests and multiple regression.
    Results: Both learning groups scored significantly higher 1 week after training. The improvement did not differ significantly between the groups when controlling for the years of experience in CHD nursing and the baseline knowledge score. Participants with higher baseline knowledge scores improved their scores less. Neither learning method was proven more effective than the other. Participants reported experiencing traditional classroom teaching as more positive, but e-learning was more time effective.
    Keywords:  congenital heart disease; education; e‐learning; haemodynamics; neonatal intensive care
    DOI:  https://doi.org/10.1002/nop2.317
  7. J Perinatol. 2019 Aug 01.
    Pyle AK, Shabanova V, Cleary MA, Ozgediz D, Cummings CL, Kamin DS, Mercurio MR.
      BACKGROUND/OBJECTIVES: Necrotizing enterocolitis (NEC) is a serious disease linked to prematurity. A variant, NEC totalis, is associated with nearly 100% mortality. There is wide variation in counseling practices for NEC totalis. Our objectives are to determine what treatment options, if any, are offered to families, and which factors influence these decisions.METHODS: An anonymous survey was distributed to members of the AAP Sections on Neonatal-Perinatal Medicine and Pediatric Surgery. Data were analyzed utilizing chi-square tests and Spearman correlations, where applicable.
    RESULTS: In the setting of NEC totalis, 90% of the 378 respondents viewed offering life-sustaining interventions (LSI) as ethically permissible and 87% felt that transfer to another center willing to provide LSI should be considered; however, only 43% reported offering LSI to families.
    CONCLUSIONS: Management of NEC totalis remains challenging and significant practice variability persists. Most respondents do not offer ongoing medical/surgical management, despite believing it is an ethically permissible option.
    DOI:  https://doi.org/10.1038/s41372-019-0448-0
  8. J Thorac Cardiovasc Surg. 2019 Jul 10. pii: S0022-5223(19)31356-X. [Epub ahead of print]
    Huang SC.
      
    DOI:  https://doi.org/10.1016/j.jtcvs.2019.06.059