bims-hylehe Biomed news
on Hypoplastic Left Heart Syndrome
Issue of 2018‒05‒20
seven papers selected by
Richard James
University of Pennsylvania


  1. Pediatr Cardiol. 2018 May 12.
    Essaid L, Strassle PD, Jernigan EG, Nelson JS.
      Hypoplastic left heart syndrome (HLHS) is a highly resource-intensive diagnosis. Geographic variation in cost and length of stay (LOS) in HLHS is not well described. Neonates diagnosed with HLHS between 2000 and 2012 were identified using the Kids' Inpatient Database. Hospitalizations were stratified into two groups: (1) birth and (2) secondary. United States regional differences in hospital charges and LOS were compared using adjusted linear regression. Of 2431 birth hospitalizations, 449 neonates (18.5%) died while inpatient and mortality rates differed by region (p = 0.02). After birth, 40.5% (n = 985) of neonates were transferred; transfers were most common in the Midwest (p < 0.0001). Adjusted average LOS was shortest in the West and longest in the South (26.1 days; 95% CI 24.0, 35.1 vs. 34.9 days; 95% CI 31.8, 38.1). Average adjusted charges were lowest in the Northeast ($324,600; 95% CI $271,400, $377,900) and highest in the West ($400,500; 95% CI $346,700, $454,300, p = 0.05). Among 1895 secondary hospitalizations, 24.9% of neonates died as inpatients, and the average adjusted LOS was shortest in the West (26.8 days; 95% CI 23.9, 29.7) and longest in the South (38.5 days; 95% CI 34.4, 42.4). Average adjusted charges were lowest in the Northeast ($326,900; 95% CI $270,700, $383,100) and highest in the South ($505,900; 95% CI $450,200, $561,500, p < 0.0001). Significant geographic variations in mortality, LOS, and hospital charges exist in care of US HLHS neonates. Reducing variation in care should remain a priority in national quality efforts in congenital heart disease.
    Keywords:  Congenital heart disease; Cost analysis; Hypoplastic left heart syndrome; Outcomes
    DOI:  https://doi.org/10.1007/s00246-018-1887-7
  2. Eur J Med Genet. 2018 May 09. pii: S1769-7212(18)30229-5. [Epub ahead of print]
    Santoro M, Coi A, Spadoni I, Bianchi F, Pierini A.
      BACKGROUND: Down syndrome (DS) is the most common autosomal chromosomal anomaly in liveborn infants. About 40% of infants with DS have a major congenital heart defect (CHD). Among them, atrioventricular septal defects (AVSD), atrial septal defects (ASD), ventricular septal defect (VSD) and Tetralogy of Fallot (ToF) are the most common. The aim of this study was to estimate the sex difference in the occurrence of CHD in infants with DS comparing it with non-DS infants.METHOD: Live birth cases of DS diagnosed by the first year of life were extracted from the Registry of Congenital Anomalies of Tuscany (2003-2015 period). CHDs associated with DS were detected both from the registry and the hospital discharge data. Sex differences in total CHDs and ASD, VSD, AVSD, severe CHDs, ToF subgroups were investigated. Relative Risks between males and females (RRMF) with p-values and 95% confidence interval (95% CI) were estimated. RRMF of CHD in infants with DS was compared to RRMF in infants without DS. The ratio between relative risks (RRR) was estimated.
    RESULTS: A total of 230 live birth cases of DS were analyzed, with a prevalence of 5.70 per 10,000 births. Sex ratioMF was 1.3. One hundred and one DS cases (43.9%) were associated with at least one CHD. Among them, CHDs are more frequent in females (total CHD: RRMF = 0.62; 95% CI: 0.46-0.83, ASD: RRMF = 0.40; 95% CI: 0.21-0.77, severe CHD: RRMF = 0.58; 95% CI: 0.35-0.95, AVSD: RRMF = 0.57; 95% CI: 0.32-1.00, VSD: RRMF = 0.59; 95% CI: 0.35-1.00). Four cases of ToF were observed (all males). Sex difference was more evident in DS than in non-DS infants (RRR = 0.63; 95% CI: 0.52-0.77), in particular for severe CHDs (RRR = 0.38; 95% CI: 0.25-0.57).
    CONCLUSION: The increased sex difference for CHDs in DS suggests a possible role of sex as effect modifier in the association between DS and CHD. The results enforce the evidence on sex differences for CHDs in DS and can stimulate future genetic research activities.
    Keywords:  Congenital heart defects; Down syndrome; Population-based registry; Sex difference; Trisomy 21
    DOI:  https://doi.org/10.1016/j.ejmg.2018.05.013
  3. J Am Heart Assoc. 2018 May 12. pii: e008094. [Epub ahead of print]7(10):
    Ubeda Tikkanen A, Nathan M, Sleeper LA, Flavin M, Lewis A, Nimec D, Mayer JE, Del Nido P.
      BACKGROUND: Patients with congenital heart disease are at risk of motor, cognitive, speech, and feeding difficulties after cardiac surgery. Rehabilitation therapy could improve functional outcomes in this population if applied in the acute postcardiac surgical in-hospital stay. However, information on the types of acute postcardiac surgery therapy needs in children is scarce. Our goal was to describe rehabilitation therapy following congenital heart surgery and pre/intraoperative factors associated with need for therapy.METHODS AND RESULTS: This is a retrospective cohort study of patients <18 years undergoing heart surgery at our center from January 1, 2013 to January 31, 2015. Demographic, and pre-, intra-, and postoperative clinical and rehabilitation therapy (physical, occupational, speech, feeding therapy, and neurodevelopment intervention) data were collected. Need for rehabilitation therapy in the acute postoperative period, particularly following palliative repair, was the outcome variable in a multivariable logistic regression model to identify independent pre- and intraoperative factors associated with therapy. A total of 586 out of 1415 (41%) subjects received rehabilitation therapy postsurgery. Certain subgroups had increased rehabilitation therapy use such as neonates (80%). On multivariable analysis, palliative repair, prematurity, genetic syndrome, presurgical hospital stay of more than 1 day, and prolonged cardiopulmonary bypass time were independently associated with rehabilitation therapy.
    CONCLUSIONS: Nearly half of patients who underwent post-congenital heart surgery received rehabilitation therapy. Frequency of use and types of therapy vary according to patient characteristics; however, certain pre- and intraoperative factors are associated with need for rehabilitation therapy, and may aid decision-making for appropriate resource allocation.
    Keywords:  congenital heart disease; function; pediatrics; rehabilitation; surgery
    DOI:  https://doi.org/10.1161/JAHA.117.008094
  4. Pediatr Cardiol. 2018 May 12.
    Duignan S, Ryan A, O'Keeffe D, Kenny D, McMahon CJ.
      The complexity and potential biases involved in decision making have long been recognised and examined in both the aviation and business industries. More recently, the medical community have started to explore this concept and its particular importance in our field. Paediatric cardiology is a rapidly expanding field and for many of the conditions we treat, there is limited evidence available to support our decision-making. Variability exists within decision-making in paediatric cardiology and this may influence outcomes. There are no validated tools available to support and examine consistent decision-making for various treatment strategies in children with congenital heart disease in a multidisciplinary cardiology and cardiothoracic institution. Our primary objective was to analyse the complexity of decision-making for children with cardiac conditions in the context of our joint cardiology and cardiothoracic conference (JCC). Two paediatric cardiologists acted as investigators by observing the weekly joint cardiology-cardiothoracic surgery conference and prospectively evaluating the degree of complexity of decision-making in the management of 107 sequential children with congenital heart disease discussed. Additionally, the group consensus on the same patients was prospectively assessed to compare this to the independent observers. Of 107 consecutive children discussed at our JCC conference 32 (27%) went on to receive surgical intervention, 20 (17%) underwent catheterisation and 65 (56%) received medical treatment. There were 53 (50%) cases rated as simple by one senior observer, while 54 (50%) were rated as complex to some degree. There was high inter-observer agreement with a Krippendorff's alpha of ≥ 0.8 between 2 observers and between 2 observers and the group consensus as a whole for grading of the complexity of decision-making. Different decisions were occasionally made on patients with the same data set. Discussions revisiting the same patient, in complex cases, resulted in different management decisions being reached in this series. Anchoring of decision-making was witnessed in certain cases. Potential application of decision making algorithms is discussed in making decisions in paediatric cardiology patients. Decision-making in our institution's joint cardiology-cardiothoracic conference proved to be complex in approximately half of our patients. Inconsistency in decision-making for patients with the same diagnosis, and different decisions made for the same complex patient at different time points confounds the reliability of the decision-making process. These novel data highlight the absence of evidence-based medicine for many decisions, occasional lack of consistency and the impact of anchoring, heuristics and other biases in complex cases. Validated decision-making algorithms may assist in providing consistency to decision-making in this setting.
    Keywords:  Bias; Conference; Congenital heart disease; Decision making; Heuristics; Multidisciplinary
    DOI:  https://doi.org/10.1007/s00246-018-1899-3
  5. Ann Thorac Surg. 2018 May 10. pii: S0003-4975(18)30648-9. [Epub ahead of print]
    Jones S, McCracken C, Alsoufi B, Mahle WT, Oster ME.
      BACKGROUND: We examined the association of pre-operative cell count abnormalities, which have been shown to be associated with outcomes in adult cardiac patients, with morbidity and mortality following surgery for congenital heart disease (CHD) in children.METHODS: We performed a retrospective cohort study on 4865 children undergoing cardiac surgery from 2004- 2014. Our exposures of interest were presence of pre-operative lymphopenia (lymphocyte count ≤ 3000 cells/μL), thrombocytopenia (platelet count < 150 x103/ μL), and neutrophilia (neutrophil count ≥ 7000 cells/ μL). Our outcomes of interest were mortality status, post-operative length of stay (LOS), and occurrence of post-operative complications. We performed logistic and linear regressions to determine the associations of pre-operative cell counts with mortality, LOS, and complications, adjusting for age, gender, race/ethnicity, presence of a genetic syndrome, and STS-EACTS Congenital Heart Surgery Mortality Category.
    RESULTS: Overall mortality was 2.8%, median LOS was 6 days, and 7.6% of patients had post-operative complications. Lymphopenia was associated with increased odds of post-operative mortality (OR 1.67, 95% CI 1.15-2.43, p = 0.007). Lymphopenia, thrombocytopenia, and neutrophilia were all associated with longer post-operative LOS. Lymphopenia and thrombocytopenia were associated with increased occurrence of post-operative sepsis, and neutrophilia was associated with need for post-operative mechanical circulatory support.
    CONCLUSIONS: In children undergoing CHD surgery, pre-operative lymphopenia is associated with increased in-hospital mortality post-operatively. Pre-operative lymphopenia, neutrophilia and thrombocytopenia are associated with longer post-operative LOS and with development of post-operative complications. Pre-operative cell counts may serve as important prognostic markers in pre-operative planning for CHD patients.
    DOI:  https://doi.org/10.1016/j.athoracsur.2018.04.022
  6. J Pediatr. 2018 May 09. pii: S0022-3476(18)30489-X. [Epub ahead of print]
    Heo JH, Rascati KL, Lopez KN, Moffett BS.
      OBJECTIVES: To determine the association of furosemide therapy with the incidence of bone fractures in children with congenital heart disease.STUDY DESIGN: We conducted a retrospective cohort study with data extracted from the 2008-2014 Texas Medicaid databases. Pediatric patients aged <12 years diagnosed with congenital heart disease, cardiomyopathy, or heart failure were included. Patients taking furosemide were categorized into a furosemide-adherent group (medication possession ratio of ≥70%), and a furosemide-nonadherent group (medication possession ratio of <70%). A third group of patients was matched to the furosemide user groups by using propensity score matching. A multivariate logistic regression and Cox proportional hazard model with a Kaplan-Meier plot (time-to-fracture) were used to compare the 3 groups, controlling for baseline demographics and clinical characteristics.
    RESULTS: After matching, 3912 patients (furosemide adherent, n = 254; furosemide nonadherent, n = 724; no furosemide, n = 2934) were identified. The incidence of fractures was highest for the furosemide-adherent group (9.1%; 23 of 254), followed by the furosemide-nonadherent group (7.2%; 52 of 724), which were both higher than for patients who did not receive furosemide (5.0%; 148 of 2934) (P < .001). Using logistic regression, both furosemide groups were more likely to have fractures than the no furosemide group: furosemide-adherent OR of 1.9 (95% CI, 1.17-2.98; P = .009); furosemide nonadherent OR of 1.5 (95% CI, 1.10-2.14; P = .01). In the Cox proportional hazard model, the risk of fractures for the furosemide-adherent group was significantly higher compared with the no furosemide group (HR, 1.6; 95% CI, 1.00-2.42; P = .04).
    CONCLUSIONS: Furosemide therapy, even with nonconsistent dosing, was associated with an increased risk of bone fractures in children with congenital heart disease.
    Keywords:  congenital heart disease; fractures; furosemide; loop diuretic
    DOI:  https://doi.org/10.1016/j.jpeds.2018.03.077
  7. J Pediatr. 2018 May 10. pii: S0022-3476(18)30493-1. [Epub ahead of print]
    Cummings CL, Geis GM, Feldman HA, Berson ER, Kesselheim JC.
      OBJECTIVE: To develop and validate the Test of Ethics Knowledge in Neonatology (TEK-Neo) with good internal consistency reliability, item performance, and construct validity that reliably assesses interprofessional staff and trainee knowledge of neonatal ethics.STUDY DESIGN: We adapted a published test of ethics knowledge for use in neonatology. The novel instrument had 46 true/false questions distributed among 7 domains of neonatal ethics: ethical principles, professionalism, genetic testing, beginning of life/viability, end of life, informed permission/decision making, and research ethics. Content and correct answers were derived from published statements and guidelines. We administered the voluntary, anonymous test via e-mailed link to 103 participants, including medical students, neonatology fellows, neonatologists, neonatology nurses, and pediatric ethicists. After item reduction, we examined psychometric properties of the resulting 36-item test and assessed overall sample performance.
    RESULTS: The overall response rate was 27% (103 of 380). The test demonstrated good internal reliability (Cronbach α = 0.66), with a mean score of 28.5 ± 3.4 out of the maximum 36. Participants with formal ethics training performed better than those without (30.3 ± 2.9 vs 28.1 ± 3.5; P = .01). Performance improved significantly with higher levels of medical/ethical training among the 5 groups: medical students, 25.9 ± 3.7; neonatal nurses/practitioners, 27.7 ± 2.7; neonatologists, 28.8 ± 3.7; neonatology fellows, 29.8 ± 2.9; and clinical ethicists, 33.0 ± 1.9 (P < .0001).
    CONCLUSIONS: The TEK-Neo reliably assesses knowledge of neonatal ethics among interprofessional staff and trainees in neonatology. This novel tool discriminates between learners with different levels of expertise and can be used interprofessionally to assess individual and group performance, track milestone progression, and address curricular gaps in neonatal ethics.
    Keywords:  assessment; medical education; medical ethics; milestones; professionalism
    DOI:  https://doi.org/10.1016/j.jpeds.2018.04.004