bims-hylehe Biomed news
on Hypoplastic left heart syndrome
Issue of 2019‒02‒24
thirteen papers selected by
Richard James
University of Pennsylvania


  1. Pediatr Crit Care Med. 2018 Nov 14.
    Dhillon GS, Lasa JJ, Aggarwal V, Checchia PA, Bavare AC.
      OBJECTIVES: Children with medical cardiac disease experience poorer survival to hospital discharge after cardiopulmonary arrest compared with children with surgical cardiac disease. Limited literature exists describing epidemiology and factors associated with mortality in this heterogeneous population. We aim to evaluate the clinical characteristics and outcomes after cardiopulmonary arrest in medical cardiac patients.DESIGN: We performed a retrospective review of pediatric cardiac patients who underwent cardiopulmonary resuscitation in a tertiary care cardiac ICU. Surgical cardiac patients underwent cardiac surgery immediately prior to ICU admission. Nonsurgical cardiac patients were divided into two groups based on the presence of congenital heart disease: congenital heart disease medical or noncongenital heart disease medical. Clinical and outcome variables were collected. Primary outcome was survival to hospital discharge.
    SETTINGS: Texas Children's Hospital cardiac ICU.
    PATIENTS: Patients admitted to Texas Children's Hospital cardiac ICU between January 2011 and December 2016.
    INTERVENTIONS: None.
    MEASUREMENTS AND MAIN RESULTS: Of 150 cardiopulmonary arrest events reviewed, 90 index events were included (46 surgical, 26 congenital heart disease medical, and 18 noncongenital heart disease medical). There was no difference in primary outcome among the three groups. The absence of an epinephrine infusion precardiopulmonary arrest was associated with increased odds of survival in the congenital heart disease medical group (p = 0.03). Noncongenital heart disease medical patients experienced pulseless ventricular tachycardia/ventricular fibrillation more frequently than congenital heart disease medical patients (p = 0.02). Congenital heart disease medical patients had trends toward longer cardiac arrest durations, higher prevalence of neurologic sequelae postcardiopulmonary arrest, and higher mortality when extracorporeal support at cardiopulmonary resuscitation was employed.
    CONCLUSIONS: Although trends in first documented rhythm, neurologic sequelae, and inotropic support prior to cardiopulmonary arrest were noted between groups, no significant differences in survival after cardiac arrest were seen. Larger scale studies are needed to better describe factors associated with cardiopulmonary arrest as well as survival in heterogeneous medical cardiac populations.
    DOI:  https://doi.org/10.1097/PCC.0000000000001810
  2. Gen Thorac Cardiovasc Surg. 2019 Feb 21.
    Savluk OF, Guzelmeric F, Yavuz Y, Ukil F, Yilmaz A, Cevirme D, Tuncer E, Hatemi AC, Ceyran H.
      BACKGROUND: Hypoplastic left heart syndrome is a lethal congenital heart malformation when untreated resulting in a 95% mortality in the first month of life. In this study, we aimed to investigate the newly introduced inflammatory biomarker, neutrophil-lymphocyte ratio, as a mortality predictor in postoperative hypoplastic left heart syndrome patients.METHODS: Patients were divided into two groups; Group 1 consisted of 33 patients who were discharged and Group 2 including 20 patients who were deceased following surgery. Patients' preoperative demographic characteristics, total white blood cell counts, neutrophil counts, lymphocyte counts, neutrophil-lymphocyte ratio, C-reactive proteins, alanine aminotransferase, aspartate transaminase, urea, and creatinine levels were recorded. Studys' primary endpoint was all-cause patient mortality following surgery.
    RESULTS: The preoperative neutrophil-lymphocyte ratio was found to be significantly different between the groups (p = 0.001). High neutrophil-lymphocyte ratio was found to be associated with an increased risk of death. The ROC curves of neutrophil-lymphocyte ratio were found to be associated with mortality. The area under curve for the preoperative neutrophil-lymphocyte ratio was 0.74. Neutrophil-lymphocyte ratio predicted mortality with a sensitivity of 78% and a specificity of 65%.
    CONCLUSION: Neutrophil-lymphocyte ratio can contribute to the early identification of patients at high risk for complications. In addition, through the use of NLR, clinicians could implement measures for the optimal therapeutic approach of cardiac surgery patients and the elimination of adverse patient outcomes.
    Keywords:  Biomarker; Congenital heart; Hypoplastic left heart syndrome; Mortality; Neutrophil–lymphocyte ratio
    DOI:  https://doi.org/10.1007/s11748-019-01081-y
  3. Pol Arch Intern Med. 2019 Feb 19.
    Smaś M, Skubera M, Wilkosz T, Weryński P, Kołcz J, Olszowska M, Podolec P, Tomkiewicz-Pająk L.
      INTRODUCTION A hemodynamic derangement in Fontan circulation causes liver pathology identified as Fontan associated liver disease. Although liver biopsy in a standard for recognition of liver fibrosis, noninvasive methods are being developed, including shear wave elastography (SWE). Objectives: The aim of the study was to evaluate the degree of liver stiffness (LS) using SWE among Fontan patients in a long-term follow-up and to investigate the relationship between patient characteristics and LS. PATIENTS AND METHODS 59 Fontan patients were included in the study. Clinical examinations and laboratory tests were performed. According to the stage of LS patients were divided to two subgroups: Group I (F1, F2) and Group II (F3, F4). Furthermore AST to ALT ratio, APRI, Fib-4 score, Forns score were assessed. RESULTS The median LS was 9.1 (3.9-18.5) kPa. 5 of patients (9%) demonstrated LS in stage F1, 14 (26%) - F2, 28 (52%) - F3 and 7 (13%) - F4. Patients in Group II had a statistically significant higher AST, GGTP, APRI, FIB-4 and Forns scores and lower level of platelet count comparing to Group I. Canonic correlation analysis indicated that LS and thrombocytopenia were related to time from Fontan operation, age at procedure and single ventricle ejection fraction (SVEF). CONCLUSIONS Our study showed that adult Fontan patients develop liver dysfunction. Time from surgery, age at procedure and SVEF are related to the degree of LS assessed by SWE. SWE and APRI, Forns index, and FIB-4 score may be helpful in assessing of liver fibrosis degree.
    DOI:  https://doi.org/10.20452/pamw.4452
  4. J Cardiovasc Nurs. 2019 Feb 16.
    Hadler RA, Goldstein NE, Bekelman DB, Riegel B, Allen LA, Arnold RM, Harinstein ME, Kavalieratos D.
      BACKGROUND: Although cardiology organizations recommend early introduction of palliative care for patients with heart failure (HF), integration has remained challenging, particularly in patients with cardiac devices such as cardiac implantable electronic devices and left ventricular assist devices. Study authors suggest that patients often have limited and erroneous understanding of these devices and their implications for future care.OBJECTIVE: The aim of this study was to assess perceptions of cardiac devices in patients with HF and how these perceptions impacted advance care planning and future expectations.
    METHODS: This study used qualitative semistructured interviews with 18 community-dwelling patients with New York Heart Association stage II to IV HF.
    RESULTS: We interviewed 18 patients (mean ejection fraction, 38%; mean age, 64 years; 33% female; 83% white; 39% New York Heart Association class II, 39% class III, and 22% class IV). All had a cardiac implantable electronic device (6% permanent pacemaker, 56% implantable cardioverter-defibrillator, 28% biventricular implantable cardioverter-defibrillator); 11% had left ventricular assist devices. Patients with devices frequently misunderstood the impact of their device on cardiac function. A majority expressed the belief that the device would forestall further deterioration, regardless of whether this was the case. This anticipation of stability was often accompanied by the expectation that emerging technologies would continue to preempt decline. Citing this faith in technology, these patients frequently saw limited value in advance care planning.
    CONCLUSIONS: In our sample, patients with cardiac devices overestimated the impact of their devices on preventing disease progression and death and deprioritized advance care planning as a result.
    DOI:  https://doi.org/10.1097/JCN.0000000000000565
  5. J Pediatr Psychol. 2019 Feb 19. pii: jsz003. [Epub ahead of print]
    Rea KE, Quast LF, Stolz MG, Blount RL.
      OBJECTIVE: Parents and siblings of children with chronic illnesses are at increased risk for experiencing psychosocial difficulties. Therapeutic recreation camps have become increasingly popular among these families. The current systematic literature review provides a synthesis of research on how these camps impact the parents and siblings of children facing a variety of chronic health conditions.METHODS: Databases searched: PubMed, PsycInfo, SportDISCUS, and Health Source Nursing/Academic Edition. Inclusion criteria included publication in a peer-reviewed journal between January 2000 and May 2018, written in the English language, and assessment of parent, sibling, or family outcomes.
    RESULTS: Twenty-one studies were included. Results indicated that camp attendance relates to positive changes in parent and sibling psychosocial outcomes. Additionally, parents report camp to be a place of social support and respite from daily life, and siblings find camp to be enjoyable and a place of belonging. Given the limited number of methodologically sound studies examining family functioning, it is not yet clear the extent to which camp influences family-level outcomes.
    CONCLUSION: Overall, camp appears to have a positive impact on parents and siblings across chronic illness populations. Future research should specifically assess family outcomes, including communication and family functioning, and the impact of incorporating well-defined interventions and education programming into the traditional therapeutic recreation camp experience.
    Keywords:  chronic illness; family functioning; parent stress; psychosocial functioning; systematic review
    DOI:  https://doi.org/10.1093/jpepsy/jsz003
  6. Interact Cardiovasc Thorac Surg. 2019 Feb 20. pii: ivz022. [Epub ahead of print]
    Van Puyvelde J, Rega F, Minami T, Claus P, Cools B, Gewillig M, Meyns B.
      OBJECTIVES: Patients with a single ventricle survive thanks to the Fontan palliation. Nevertheless, there is a growing number of Fontan patients with progressive heart failure. To validate therapeutic options in these patients, we developed a chronic Fontan large animal model.METHODS: A Fontan circulation was surgically created in 15 sheep. The superior vena cava was anastomosed end-to-side to the pulmonary artery. The inferior vena cava was connected to the pulmonary artery by an ePTFE conduit, and the inferior vena cava-right atrium junction was ligated.
    RESULTS: Total cavopulmonary connection was successfully performed in all 15 animals. After creation of the Fontan circulation, central venous pressure increased from 4 [interquartile range (IQR) 3-6] mmHg to 16 (IQR 14-17) mmHg, mean arterial blood pressure decreased from 68 (IQR 54-75) mmHg to 52 (IQR 50-61) mmHg and cardiac output decreased from 5.1 (IQR 4.6-6.8) l/min to 1.7 (IQR 1.3-2.7) l/min. Five animals were electively sacrificed after a follow-up period of 21 weeks.
    CONCLUSIONS: These results demonstrate that it is feasible to create a chronic animal model with unsupported Fontan circulation. This animal model not only opens perspectives to investigate the pathophysiology of the failing Fontan circulation, but also provides the possibility to study therapeutic options such as the effect of mechanical circulatory support in the failing Fontan physiology.
    Keywords:  Congenital heart disease; Experimental study; Failing Fontan circulation; Fontan procedure; Total cavopulmonary connection
    DOI:  https://doi.org/10.1093/icvts/ivz022
  7. Int J Epidemiol. 2019 Feb 19. pii: dyz009. [Epub ahead of print]
    Liu Y, Chen S, Zühlke L, Black GC, Choy MK, Li N, Keavney BD.
      BACKGROUND: Globally, access to healthcare and diagnostic technologies are known to substantially impact the reported birth prevalence of congenital heart disease (CHD). Previous studies have shown marked heterogeneity between different regions, with a suggestion that CHD prevalence is rising globally, but the degree to which this reflects differences due to environmental or genetic risk factors, as opposed to improved detection, is uncertain. We performed an updated systematic review to address these issues.METHODS: Studies reporting the birth prevalence of CHD between the years 1970-2017 were identified from searches of PubMed, EMBASE, Web of Science and Google Scholar. Data on the prevalence of total CHD and 27 anatomical subtypes of CHD were collected. Data were combined using random-effect models. Subgroup and meta-regression analyses were conducted, focused on geographical regions and levels of national income.
    RESULTS: Two hundred and sixty studies met the inclusion criteria, encompassing 130 758 851 live births. The birth prevalence of CHD from 1970-2017 progressively increased to a maximum in the period 2010-17 of 9.410/1000 [95% CI (confidence interval) 8.602-10.253]. This represented a significant increase over the fifteen prior years (P = 0.031). The change in prevalence of mild CHD lesions (ventricular septal defect, atrial septal defect and patent ductus arteriosus) together explained 93.4% of the increased overall prevalence, consistent with a major role of improved postnatal detection of less severe lesions. In contrast the prevalence of lesions grouped together as left ventricular outflow tract obstruction (which includes hypoplastic left heart syndrome) decreased from 0.689/1000 (95% CI 0.607-0.776) in 1995-99, to 0.475/1000 (95% CI 0.392-0.565; P = 0.004) in 2010-17, which would be consistent with improved prenatal detection and consequent termination of pregnancy when these very severe lesions are discovered. There was marked heterogeneity among geographical regions, with Africa reporting the lowest prevalence [2.315/1000 (95% CI 0.429-5.696)] and Asia the highest [9.342/1000 (95% CI 8.072-10.704)].
    CONCLUSIONS: The reported prevalence of CHD globally continues to increase, with evidence of severe unmet diagnostic need in Africa. The recent prevalence of CHD in Asia for the first time appears higher than in Europe and America, where disease ascertainment is likely to be near-complete, suggesting higher genetic or environmental susceptibility to CHD among Asian people.
    Keywords:  Congenital heart disease; geographical region; meta-analysis; national income; prevalence; systematic review
    DOI:  https://doi.org/10.1093/ije/dyz009
  8. JACC Clin Electrophysiol. 2019 Feb;pii: S2405-500X(18)30802-8. [Epub ahead of print]5(2): 245-251
    Bezzerides VJ, Walsh A, Martuscello M, Escudero CA, Gauvreau K, Lam G, Abrams DJ, Triedman JK, Alexander ME, Bevilacqua L, Mah DY.
      OBJECTIVES: This study sought to determine the practical use of the recently introduced LINQ implantable loop recorder (LINQ-ILR) in a cohort of pediatric and adult congenital arrhythmia patients.BACKGROUND: Correlating symptoms to a causative arrhythmia is a key aspect of diagnosis and management in clinical electrophysiology.
    METHODS: Retrospective review of clinical data, implantation indications, findings, and therapeutic decisions in patients who underwent LINQ-ILR implantation from April 1st, 2014 to January 30th, 2017 at Boston Children's Hospital.
    RESULTS: A total of 133 patients were included, of which 76 (57%) were male. The mean age at implantation was 15.7 ± 9.1 years with a duration of follow-up of 11.8 months. Congenital heart disease was present in 34 patients (26%), a confirmed genetic diagnosis in 50 (38%), and cardiomyopathy in 22 (26%), and the remainder were without a previous diagnosis. Syncope was the most common indication for LINQ-ILR implantation, occurring in 59 patients (44%). The median time to diagnosis was 4.5 months, occurring in 78 patients (59%). Cardiac device placement occurred in 17 patients (22%), a medication change in 9 (12%), electrophysiology study/ablation in 5 (6%), or LINQ-ILR explantation in 42 (54%). Infection or erosion occurred in 5 patients. Syncope was correlated with a diagnostic transmission (54% vs. 31%, p = 0.01).
    CONCLUSIONS: The LINQ-ILR is an important diagnostic tool, providing useful data in more than one-half of patients in <6 months. Adverse events are low. Patient selection is critical and undiagnosed syncope represents an important presenting indication for which a LINQ-ILR implant should be considered.
    Keywords:  adult congenital; arrhythmias; loop recorder; pediatric
    DOI:  https://doi.org/10.1016/j.jacep.2018.09.016
  9. World J Pediatr. 2019 Feb 22.
    Xu JJ, Luo YJ, Wang JH, Xu WZ, Shi Z, Fu JZ, Shu Q.
      BACKGROUND: Preoperative planning for children with congenital heart diseases remains crucial and challenging. This study aimed to investigate the roles of three-dimensional printed patient-specific heart models in the presurgical planning for complex congenital heart disease.METHODS: From May 2017 to January 2018, 15 children diagnosed with complex congenital heart disease were included in this study. Heart models were printed based on computed tomography (CT) imaging reconstruction by a 3D printer with photosensitive resin using the stereolithography apparatus technology. Surgery options were first evaluated by a sophisticated cardiac surgery group using CT images only, and then surgical plans were also set up based on heart models.
    RESULTS: Fifteen 3D printed heart models were successfully generated. According to the decisions based on CT, 13 cases were consistent with real options, while the other 2 were not. According to 3D printed heart models, all the 15 cases were consistent with real options. Unfortunately, one child diagnosed with complete transposition of great arteries combined with interruption of aortic arch (type A) died 5 days after operation due to postoperative low cardiac output syndrome. The cardiologists, especially the younger ones, considered that these 3D printed heart models with tangible, physical and comprehensive illustrations were beneficial for preoperative planning of complex congenital heart diseases.
    CONCLUSION: 3D printed heart models are beneficial and promising in preoperative planning for complex congenital heart diseases, and are able to help conform or even improve the surgery options.
    Keywords:  Computed tomography; Congenital heart disease; Surgery; Three-dimensional printing
    DOI:  https://doi.org/10.1007/s12519-019-00228-4
  10. Quant Imaging Med Surg. 2019 Jan;9(1): 115-126
    Kiraly L, Kiraly B, Szigeti K, Tamas CZ, Daranyi S.
      Education and training of morphology for medical students, and professionals specializing in pediatric cardiology and surgery has traditionally been based on hands-on encounter with congenitally malformed cardiac specimens. Large international archives are no longer widely available due to stricter data protection rules, a reduced number of autopsies, attrition rate of existing specimens, and most importantly due to a higher survival rate of patients. Our Cardiac Archive houses about 400 cardiac specimens with congenital heart disease. The collection spans almost 60 years and thus goes back to pre-surgical era. Unfortunately, attrition rate due to desiccation has led to an increased natural decay in recent years. The present multi-institutional project focuses on saving the collection by digitization. Specimens are scanned by high-resolution micro-CT/MRI. Virtual 3D-models are segmented and a comprehensive database is built. We now report an initial feasibility study with six test specimens that provided promising results, however, adequate presentation of the intracardiac anatomy, including septa and cardiac valves requires further refinements. Computer assisted design methods are necessary to overcome consequences of pathological examination, shrinkage and/or distortion of the specimens. For a next step, we anticipate an expandable web-based virtual museum with interactive reference and training tools. Web access for professional third parties will be provided by registration/subscription. In a future phase, segmental wall motion data could be added to virtual models. 3D-printed models may replace actual specimens and serve as hands-on surgical training to elucidate complex morphologies, promote surgical emulation, and extract more accurate procedural knowledge based on such a collection.
    Keywords:  Congenital abnormalities; anatomic; imaging, three-dimensional; interactive learning; models; multimodal imaging
    DOI:  https://doi.org/10.21037/qims.2018.12.05
  11. Heart Asia. 2019 ;11(1): e011125
    Vervoort D, Park KB, Swain JD.
      
    Keywords:  congenital heart disease; education; public health; quality of care and outcomes
    DOI:  https://doi.org/10.1136/heartasia-2018-011125
  12. Quant Imaging Med Surg. 2019 Jan;9(1): 107-114
    Lau I, Wong YH, Yeong CH, Abdul Aziz YF, Md Sari NA, Hashim SA, Sun Z.
      Current visualization techniques of complex congenital heart disease (CHD) are unable to provide comprehensive visualization of the anomalous cardiac anatomy as the medical datasets can essentially only be viewed from a flat, two-dimensional (2D) screen. Three-dimensional (3D) printing has therefore been used to replicate patient-specific hearts in 3D views based on medical imaging datasets. This technique has been shown to have a positive impact on the preoperative planning of corrective surgery, patient-doctor communication, and the learning experience of medical students. However, 3D printing is often costly, and this impedes the routine application of this technology in clinical practice. This technical note aims to investigate whether reducing 3D printing costs can have any impact on the clinical value of the 3D-printed heart models. Low-cost and a high-cost 3D-printed models based on a selected case of CHD were generated with materials of differing cost. Quantitative assessment of dimensional accuracy of the cardiac anatomy and pathology was compared between the 3D-printed models and the original cardiac computed tomography (CT) images with excellent correlation (r=0.99). Qualitative evaluation of model usefulness showed no difference between the two models in medical applications.
    Keywords:  Accuracy; assessment; cost; heart disease; three-dimensional printing (3D printing)
    DOI:  https://doi.org/10.21037/qims.2019.01.02