bims-hylehe Biomed news
on Hypoplastic Left Heart Syndrome
Issue of 2018‒05‒06
eight papers selected by
Richard James
University of Pennsylvania


  1. J Thorac Cardiovasc Surg. 2018 Apr 07. pii: S0022-5223(18)30930-9. [Epub ahead of print]
    Nair AG, Oladunjoye OO, Trenor CC, LaRonde M, van den Bosch SJ, Sleeper LA, VanderPluym C, Emani SM, Kheir JN.
      BACKGROUND: Patients undergoing surgery for congenital heart disease are at high risk for bleeding as well as thrombosis in the postoperative period. The objective of the study was to describe the design and effects of implementing a standardized unfractionated heparin anticoagulation protocol for children after congenital heart surgery.METHODS: We created a tiered guideline for the postoperative management of bleeding and thrombosis. In patients treated with unfractionated heparin, anti-factor Xa activity level as well as activated partial thromboplastin time were used for dose titration. Clinical outcomes, including bleeding and thrombosis events, were prospectively collected for 5 months before and after protocol implementation and adjudicated as either minor, clinically relevant nonmajor, or major.
    RESULTS: Among 792 surgical patients followed during the study period, a total of 203 patients (87 preimplementation, 116 postimplementation) were treated with therapeutic unfractionated heparin over a total of 1481 patient days. Of these, 28% were neonates and 35% were infants (29 days to 1 year), with a trend toward fewer neonates and lower Risk Adjustment for Congenital Heart Surgery (RACHS) scores after protocol implementation. Among 1321 time-matched pairs, activated partial thromboplastin time and antifactor Xa activity levels were poorly correlated (r2 = 0.33). Clinically relevant bleeding events, which required increased medical care, including blood transfusion, decreased after protocol implementation (4.14 vs 1.62 bleeding events per 100 patient-days; risk ratio, 0.39 [0.20-0.75]; P = .005), even after correcting for differences in age and RACHS scores (P = .006). This finding was primarily found after RACHS category 1 to 3 procedures (risk ratio, 0.27 [0.10-0.73]; P = .0099) and in noninfants (risk ratio, 0.25 [0.09-0.65]; P = .005). There were no significant differences in the incidences of major bleeding (P = .88) or any thrombosis (P = .55).
    CONCLUSIONS: The use of a standardized anticoagulation protocol is feasible and might reduce the incidence of bleeding and thrombosis events in postcardiotomy patients.
    Keywords:  anticoagulation; bleeding; congenital heart disease; heparin; thrombosis
    DOI:  https://doi.org/10.1016/j.jtcvs.2018.02.106
  2. Cardiol Young. 2018 Apr 29. 1-11
    Cohen S, Gilutz H, Marelli AJ, Iserin L, Benis A, Bonnet D, Burgun A.
      The need for population-based studies of adults with CHD has motivated the growing use of secondary analyses of administrative health data in a variety of jurisdictions worldwide. We aimed at systematically reviewing all studies using administrative health data sources for adult CHD research from 2006 to 2016. Using PubMed and Embase (1 January, 2006 to 1 January, 2016), we identified 2217 abstracts, from which 59 studies were included in this review. These comprised 12 different data sources from six countries. Of these, 55% originated in the United States of America, 28% in Canada, and 17% in Europe and Asia. No study was published before 2007, after which the number of publications grew exponentially. In all, 41% of the studies were cross-sectional and 25% were retrospective cohort studies with a wide variation in the availability of patient-level compared with hospitalisation-level episodes of care; 58% of studies from eight different data sources linked administrative data at a patient level; and 37% of studies reported validation procedures. Assessing resource utilisation and temporal trends of relevant epidemiological and outcome end points were the most reported objectives. The median impact factor of publication journals was 4.04, with an interquartile range of 3.15, 7.44. Although not designed for research purposes, administrative health databases have become powerful data sources for studying adult CHD populations because of their large sample sizes, comprehensive records, and long observation periods, providing a useful tool to further develop quality of care improvement programmes. Data linkage with electronic records will become important in obtaining more granular life-long adult CHD data. The health services nature of the data optimises the impact on policy and public health.
    Keywords:  Adult with CHD; administrative health database; epidemiology; outcomes
    DOI:  https://doi.org/10.1017/S1047951118000446
  3. Cardiol Young. 2018 Apr 29. 1-11
    Marino LV, Johnson MJ, Hall NJ, Davies NJ, Kidd CS, Daniels ML, Robinson JE, Richens T, Bharucha T, Darlington AE, .
      IntroductionDespite improvements in the medical and surgical management of infants with CHD, growth failure before surgery in many infants continues to be a significant concern. A nutritional pathway was developed, the aim of which was to provide a structured approach to nutritional care for infants with CHD awaiting surgery.Materials and methodsThe modified Delphi process was development of a nutritional pathway; initial stakeholder meeting to finalise draft guidelines and develop questions; round 1 anonymous online survey; round 2 online survey; regional cardiac conference and pathway revision; and final expert meeting and pathway finalisation.RESULTS: Paediatric Dietitians from all 11 of the paediatric cardiology surgical centres in the United Kingdom contributed to the guideline development. In all, 33% of participants had 9 or more years of experience working with infants with CHD. By the end of rounds 1 and 2, 76 and 96% of participants, respectively, were in agreement with the statements. Three statements where consensus was not achieved by the end of round 2 were discussed and agreed at the final expert group meeting.
    CONCLUSIONS: Nutrition guidelines were developed for infants with CHD awaiting surgery, using a modified Delphi process, incorporating the best available evidence and expert opinion with regard to nutritional support in this group.
    Keywords:  CHD; Delphi; growth; infants; nutrition
    DOI:  https://doi.org/10.1017/S1047951118000549
  4. Rev Port Cardiol. 2018 Apr 25. pii: S0870-2551(16)30294-3. [Epub ahead of print]
    Epifânio P, Amaral ME, Noronha N, Martins P, Rodrigues D, Pires A, Castela E.
      Infective endocarditis is a microbial infection of the endocardium and it is rare in the pediatric population. In children, congenital heart disease is one of the most important risk factors for developing infective endocarditis and can involve other structures in addition to cardiac valves. The prognosis is generally better than in other forms of endocarditis, although the average mortality rate in the pediatric population is 15-25%. Clinical manifestations can mimic other diseases such as meningitis and collagen-vascular disease or vasculitis. Therefore, a high degree of suspicion is required to make an early diagnosis. Gram-positive bacteria, specifically alpha-hemolytic streptococci, Staphylococcus aureus and coagulase-negative staphylococci, are the most commonly involved bacteria. Diagnosis is based on the modified Duke criteria, which rely mostly on clinical assessment, echocardiography and blood cultures. Antibacterial treatment should ideally be targeted. However, if no specific bacteria have been identified, patients should promptly be treated empirically with multiple drug regimens based on local resistance and the most common etiologies. The authors describe a case of a seven-year-old girl with classic clinical signs of endocarditis, with a clinical twist.
    Keywords:  Coartação da aorta nativa; Endarterite infecciosa; Enfartes tromboembólicos; Infective endarteritis; Native aortic coarctation; Staphylococcus schleiferi; Thromboembolic infarcts
    DOI:  https://doi.org/10.1016/j.repc.2017.06.023
  5. Heart Lung Circ. 2018 Apr 05. pii: S1443-9506(18)30142-2. [Epub ahead of print]
    Sweeting J, Semsarian C.
      Sudden cardiac death (SCD) in athletes is a rare but tragic complication of a number of cardiovascular diseases. Inherited causes such as the structural and arrhythmogenic genetic heart conditions are often found or suspected to be the underlying cause of death at post mortem examination. Physical activity and intense exercise may trigger cardiac arrhythmias in individuals with these conditions leading to SCD. Prevention and treatment strategies include individual athlete management strategies, coupled with public health measures such as universal cardiopulmonary resuscitation (CPR) training and availability of automatic external defibrillators (AEDs) in public places, thereby preventing SCD in both athletes and the general population. Where an athlete is known to have a cardiac condition, some restrictions from participation may be prudent, however, new evidence is emerging that perhaps current restrictions are too strict and could be relaxed in some cases. An athlete-centred model of care is essential to ensure the clinical implications and athlete preferences are balanced providing the best outcome for all concerned.
    Keywords:  Athletes; Genetics; Heart; Prevention; Sudden cardiac death
    DOI:  https://doi.org/10.1016/j.hlc.2018.03.026
  6. Complement Ther Clin Pract. 2018 May;pii: S1744-3881(18)30064-1. [Epub ahead of print]31 47-52
    Şenel E, Demir E.
      Apitherapy is the medical use and the application of honey bee products and in recent years there has been a growing interest in studies of this field. We aimed to perform a bibliometric study in the apitherapy literature. We used Web of Science database in this study and our search retrieved a total of 6917 documents of which great majority (82.4%) was original articles. Brazil was found to ranked first on the publication number with 889 papers followed by the USA, China, Japan and Turkey. We measured a productivity score for each country and the most productive countries in apitherapy field were Switzerland (2.978), Croatia (2.074), and Bulgaria (1.840). Propolis was the most used keyword followed by bee venom, flavonoids, apis mellifera and apoptosis. A moderate correlation was detected between number of publications and GDP. To the best of our knowledge our study was the first in this area and we proposed that further studies should be supported in this field.
    Keywords:  Apitherapy; Bee venom; Bibliometrics; Propolis; Publication trend analysis; Royal jelly; Scientometrics
    DOI:  https://doi.org/10.1016/j.ctcp.2018.02.003
  7. Heart Lung Circ. 2018 Apr 04. pii: S1443-9506(18)30139-2. [Epub ahead of print]
    Oomen AWGJ, Semsarian C, Puranik R, Sy RW.
      Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited cardiomyopathy that predominantly affects the right ventricle. With a prevalence in the range of 1:5000 to 1:2000 persons, ARVC is one of the leading causes of sudden cardiac death in young people and in athletes. Although early detection and treatment is important, the diagnosis of ARVC remains challenging. There is no single pathognomonic diagnostic finding in ARVC; rather, current international task force criteria specify diagnostic major and minor criteria in six categories: right ventricular imaging (including echocardiography and cardiac magnetic resonance imaging (MRI)), histology, repolarisation abnormalities, depolarisation and conduction abnormalities, arrhythmias and family history (including genetic testing). Combining findings from differing diagnostic modalities can establish a "definite", "borderline" or "possible" diagnosis of ARVC. However, there are limitations inherent in the current task force criteria, including the lack of specificity for ARVC; future iterations may be improved, for example, by enhanced imaging protocols able to detect subtle changes in the structure and function of the right ventricle, incorporation of electro-anatomical data, response to adrenergic challenge, and validated criteria for interpreting genetic variants.
    Keywords:  Arrhythmogenic ventricular cardiomyopathy; Criteria; Diagnosis
    DOI:  https://doi.org/10.1016/j.hlc.2018.03.023
  8. Acad Pediatr. 2018 Apr 25. pii: S1876-2859(18)30175-X. [Epub ahead of print]
    Arthur KC, Mangione-Smith R, Burkhart Q, Parast L, Liu H, Elliott MN, McGlynn EA, Schneider EC.
      OBJECTIVE: To examine the relationship between continuity of care for children with medical complexity (CMC) and emergency department (ED) utilization, care coordination quality, and family impact related to care coordination.METHODS: We measured ED utilization and primary care continuity with the Bice-Boxerman continuity of care index for 1477 CMC using administrative data from Minnesota and Washington state Medicaid agencies. For a subset of 186 of these CMC a caregiver survey was used to measure care coordination quality (using items adapted from the Consumer Assessment of Healthcare Providers and System (CAHPS©) Adult Health Plan Survey) and family impact (using items adapted from the National Survey of Children with Special Health Care Needs). Multivariable regression was used to examine the relationship between continuity, entered as a continuous variable ranging from 0 to 1, and the outcomes.
    RESULTS: The median continuity was 0.27 (interquartile range [IQR] 0.12, 0.48) in the administrative data cohort and 0.27 (IQR 0.14, 0.43) in the survey cohort. Compared to children with a continuity score of 0, children with a score of 1 had lower odds of having ≥1 ED visit (OR=0.65, 95% confidence interval [CI], 0.46, 0.93; p=0.017) and their caregivers reported higher scores for the measure of receipt of care coordination (β=35.2 on a 0-100 scale, 95% CI, 11.5, 58.9, p=0.004). There was no association between continuity and family impact.
    CONCLUSIONS: Continuity of care holds promise as a quality measure for CMC given its association with lower ED utilization and more frequent receipt of care coordination.
    Keywords:  Children with Medical Complexity; Continuity of Care; Quality Measurement
    DOI:  https://doi.org/10.1016/j.acap.2018.04.009