Publisher: Cambridge University Press   (Total: 387 journals)

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Showing 1 - 200 of 387 Journals sorted alphabetically
Acta Neuropsychiatrica     Hybrid Journal   (Followers: 6, SJR: 0.733, CiteScore: 2)
Acta Numerica     Hybrid Journal   (Followers: 5, SJR: 6.709, CiteScore: 10)
Advances in Animal Biosciences     Full-text available via subscription   (Followers: 14)
Advances in Applied Mathematics and Mechanics     Full-text available via subscription   (Followers: 2, SJR: 0.441, CiteScore: 1)
Aeronautical J., The     Hybrid Journal   (Followers: 7)
Africa     Hybrid Journal   (Followers: 22, SJR: 0.582, CiteScore: 1)
African Studies Review     Full-text available via subscription   (Followers: 21, SJR: 0.437, CiteScore: 1)
Ageing & Society     Hybrid Journal   (Followers: 44, SJR: 0.756, CiteScore: 2)
Agricultural and Resource Economics Review     Open Access   (Followers: 7, SJR: 0.414, CiteScore: 1)
AI EDAM     Hybrid Journal   (Followers: 2, SJR: 0.375, CiteScore: 1)
AJIL Unbound     Open Access  
AJS Review     Full-text available via subscription   (Followers: 4, SJR: 0.128, CiteScore: 0)
American Political Science Review     Hybrid Journal   (Followers: 322, SJR: 5.587, CiteScore: 4)
Anatolian Studies     Full-text available via subscription   (Followers: 5, SJR: 0.528, CiteScore: 1)
Ancient Mesoamerica     Hybrid Journal   (Followers: 12, SJR: 0.478, CiteScore: 1)
Anglo-Saxon England     Hybrid Journal   (Followers: 36, SJR: 0.1, CiteScore: 0)
animal     Hybrid Journal   (Followers: 4, SJR: 0.842, CiteScore: 2)
Animal Health Research Reviews     Hybrid Journal   (Followers: 3, SJR: 0.69, CiteScore: 2)
Animal Science     Full-text available via subscription   (Followers: 10)
Annals of Actuarial Science     Full-text available via subscription   (Followers: 1)
Annual of the British School at Athens     Full-text available via subscription   (Followers: 17, SJR: 0.177, CiteScore: 0)
Annual Review of Applied Linguistics     Hybrid Journal   (Followers: 44, SJR: 3.223, CiteScore: 4)
Antarctic Science     Hybrid Journal   (Followers: 1, SJR: 0.643, CiteScore: 1)
Antichthon     Full-text available via subscription   (Followers: 3, SJR: 0.101, CiteScore: 0)
Antiquaries J., The     Full-text available via subscription   (Followers: 12, SJR: 0.106, CiteScore: 0)
Antiquity     Hybrid Journal   (Followers: 33)
ANZIAM J.     Open Access   (Followers: 1, SJR: 0.216, CiteScore: 0)
Applied Psycholinguistics     Hybrid Journal   (Followers: 25, SJR: 0.945, CiteScore: 2)
APSIPA Transactions on Signal and Information Processing     Open Access   (Followers: 9, SJR: 0.404, CiteScore: 2)
Arabic Sciences and Philosophy     Hybrid Journal   (Followers: 9, SJR: 0.101, CiteScore: 0)
Arbor Clinical Nutrition Updates     Full-text available via subscription   (Followers: 5)
Archaeological Dialogues     Hybrid Journal   (Followers: 36, SJR: 0.898, CiteScore: 1)
Archaeological Reports     Full-text available via subscription   (Followers: 5, SJR: 0.128, CiteScore: 0)
Architectural History     Full-text available via subscription  
arq: Architectural Research Quarterly     Hybrid Journal   (Followers: 7, SJR: 0.123, CiteScore: 0)
Art Libraries J.     Full-text available via subscription   (Followers: 1)
Asian J. of Comparative Law     Hybrid Journal   (Followers: 11, SJR: 0.129, CiteScore: 0)
Asian J. of Intl. Law     Hybrid Journal   (Followers: 18, SJR: 0.135, CiteScore: 0)
Asian J. of Law and Society     Hybrid Journal   (Followers: 7, SJR: 0.195, CiteScore: 0)
Astin Bulletin     Full-text available via subscription   (Followers: 1, SJR: 0.878, CiteScore: 1)
Australasian J. of Organisational Psychology     Hybrid Journal   (Followers: 9, SJR: 0.154, CiteScore: 1)
Australian J. of Environmental Education     Full-text available via subscription   (Followers: 9, SJR: 0.403, CiteScore: 1)
Australian J. of Indigenous Education, The     Full-text available via subscription   (Followers: 11, SJR: 0.26, CiteScore: 1)
Australian J. of Rehabilitation Counseling     Full-text available via subscription   (Followers: 6, SJR: 0.144, CiteScore: 0)
Austrian History Yearbook     Full-text available via subscription   (Followers: 11, SJR: 0.161, CiteScore: 0)
Behavioral and Brain Sciences     Hybrid Journal   (Followers: 39, SJR: 0.595, CiteScore: 1)
Behaviour Change     Full-text available via subscription   (Followers: 13, SJR: 0.508, CiteScore: 1)
Behavioural and Cognitive Psychotherapy     Hybrid Journal   (Followers: 183, SJR: 0.976, CiteScore: 2)
Bilingualism: Language and Cognition     Hybrid Journal   (Followers: 44, SJR: 1.446, CiteScore: 2)
Biofilms     Full-text available via subscription   (Followers: 2)
Bird Conservation Intl.     Hybrid Journal   (Followers: 25, SJR: 0.581, CiteScore: 1)
BJPsych Advances     Full-text available via subscription   (Followers: 57, SJR: 0.275, CiteScore: 0)
BJPsych Intl.     Open Access   (Followers: 2)
BJPsych Open     Open Access   (Followers: 3)
Brain Impairment     Full-text available via subscription   (Followers: 2, SJR: 0.321, CiteScore: 1)
Breast Cancer Online     Full-text available via subscription   (Followers: 5)
Britannia     Full-text available via subscription   (Followers: 11, SJR: 0.111, CiteScore: 0)
British Actuarial J.     Full-text available via subscription  
British Catholic History     Hybrid Journal   (Followers: 2, SJR: 0.133, CiteScore: 1)
British J. for the History of Science     Hybrid Journal   (Followers: 25, SJR: 0.235, CiteScore: 0)
British J. of Anaesthetic and Recovery Nursing     Full-text available via subscription   (Followers: 8)
British J. of Music Education     Hybrid Journal   (Followers: 24, SJR: 0.564, CiteScore: 1)
British J. Of Nutrition     Hybrid Journal   (Followers: 92, SJR: 1.612, CiteScore: 4)
British J. of Political Science     Hybrid Journal   (Followers: 220, SJR: 4.661, CiteScore: 4)
British J. of Psychiatry     Hybrid Journal   (Followers: 232, SJR: 2.844, CiteScore: 3)
Bulletin of Entomological Research     Hybrid Journal   (Followers: 13, SJR: 0.805, CiteScore: 2)
Bulletin of Symbolic Logic     Full-text available via subscription   (Followers: 2, SJR: 0.555, CiteScore: 1)
Bulletin of the Australian Mathematical Society     Full-text available via subscription   (Followers: 2, SJR: 0.44, CiteScore: 0)
Bulletin of the School of Oriental and African Studies     Hybrid Journal   (Followers: 21, SJR: 0.146, CiteScore: 0)
Business and Human Rights J.     Full-text available via subscription   (Followers: 4, SJR: 0.536, CiteScore: 1)
Business Ethics Quarterly     Full-text available via subscription   (Followers: 18, SJR: 1.098, CiteScore: 2)
Business History Review     Hybrid Journal   (Followers: 16, SJR: 0.347, CiteScore: 1)
Cambridge Archaeological J.     Hybrid Journal   (Followers: 155, SJR: 1.121, CiteScore: 1)
Cambridge Classical J.     Full-text available via subscription   (Followers: 24, SJR: 0.101, CiteScore: 0)
Cambridge J. of Postcolonial Literary Inquiry     Hybrid Journal   (Followers: 7)
Cambridge Law J.     Hybrid Journal   (Followers: 204, SJR: 0.213, CiteScore: 0)
Cambridge Opera J.     Hybrid Journal   (Followers: 4, SJR: 0.14, CiteScore: 0)
Cambridge Quarterly of Healthcare Ethics     Hybrid Journal   (Followers: 11, SJR: 0.299, CiteScore: 1)
Cambridge Yearbook of European Legal Studies     Full-text available via subscription  
Camden Fifth Series     Full-text available via subscription   (Followers: 3)
Canadian Entomologist     Hybrid Journal   (Followers: 5, SJR: 0.482, CiteScore: 1)
Canadian J. of Emergency Medicine     Hybrid Journal   (Followers: 13, SJR: 0.624, CiteScore: 1)
Canadian J. of Law & Jurisprudence     Full-text available via subscription   (Followers: 11, SJR: 0.237, CiteScore: 0)
Canadian J. of Law and Society     Hybrid Journal   (Followers: 20, SJR: 0.259, CiteScore: 1)
Canadian J. of Mathematics / J. canadien de mathématiques     Hybrid Journal  
Canadian J. of Neurological Sciences     Full-text available via subscription   (SJR: 0.549, CiteScore: 1)
Canadian J. of Political Science/Revue canadienne de science politique     Full-text available via subscription   (Followers: 25, SJR: 0.385, CiteScore: 1)
Canadian J. on Aging     Hybrid Journal   (Followers: 13, SJR: 0.426, CiteScore: 1)
Canadian Mathematical Bulletin     Hybrid Journal  
Canadian Yearbook of Intl. Law / Annuaire canadien de droit international     Full-text available via subscription   (Followers: 3)
Cardiology in the Young     Hybrid Journal   (Followers: 34, SJR: 0.372, CiteScore: 1)
Central European History     Full-text available via subscription   (Followers: 33, SJR: 0.159, CiteScore: 0)
Children Australia     Partially Free   (Followers: 2, SJR: 0.255, CiteScore: 0)
China Quarterly     Hybrid Journal   (Followers: 54, SJR: 2.289, CiteScore: 3)
Chinese J. of Agricultural Biotechnology     Full-text available via subscription   (Followers: 4)
Church History : Studies in Christianity and Culture     Full-text available via subscription   (Followers: 77, SJR: 0.106, CiteScore: 0)
Classical Quarterly     Full-text available via subscription   (Followers: 36, SJR: 0.204, CiteScore: 0)
Classical Review     Full-text available via subscription   (Followers: 29)
CNS Spectrums     Hybrid Journal   (Followers: 3, SJR: 1.391, CiteScore: 3)
Cognitive Behaviour Therapist     Hybrid Journal   (Followers: 14)
Combinatorics, Probability and Computing     Hybrid Journal   (Followers: 4, SJR: 0.839, CiteScore: 1)
Communications in Computational Physics     Full-text available via subscription   (Followers: 3, SJR: 1.048, CiteScore: 2)
Comparative Studies in Society and History     Full-text available via subscription   (Followers: 49, SJR: 0.585, CiteScore: 1)
Compositio Mathematica     Full-text available via subscription   (SJR: 3.139, CiteScore: 1)
Contemporary European History     Hybrid Journal   (Followers: 35, SJR: 0.263, CiteScore: 1)
Continuity and Change     Hybrid Journal   (Followers: 12, SJR: 0.107, CiteScore: 0)
Dance Research J.     Full-text available via subscription   (Followers: 17, SJR: 0.211, CiteScore: 0)
Development and Psychopathology     Hybrid Journal   (Followers: 9, SJR: 2.068, CiteScore: 4)
Dialogue Canadian Philosophical Review/Revue canadienne de philosophie     Full-text available via subscription   (Followers: 5, SJR: 0.156, CiteScore: 0)
Diamond Light Source Proceedings     Full-text available via subscription   (Followers: 1)
Disaster Medicine and Public Health Preparedness     Hybrid Journal   (Followers: 15, SJR: 0.471, CiteScore: 1)
Du Bois Review: Social Science Research on Race     Full-text available via subscription   (Followers: 10, SJR: 0.561, CiteScore: 1)
Early China     Hybrid Journal   (Followers: 3)
Early Music History     Hybrid Journal   (Followers: 9, SJR: 0.101, CiteScore: 0)
Earth and Environmental Science Transactions of the Royal Society of Edinburgh     Hybrid Journal   (Followers: 5)
East Asian J. on Applied Mathematics     Full-text available via subscription   (SJR: 0.418, CiteScore: 1)
Ecclesiastical Law J.     Full-text available via subscription   (Followers: 6, SJR: 0.114, CiteScore: 0)
Econometric Theory     Hybrid Journal   (Followers: 18, SJR: 2.915, CiteScore: 1)
Economics and Philosophy     Hybrid Journal   (Followers: 18, SJR: 0.622, CiteScore: 1)
Edinburgh J. of Botany     Hybrid Journal   (SJR: 0.283, CiteScore: 1)
Educational and Developmental Psychologist     Full-text available via subscription   (Followers: 10, SJR: 0.146, CiteScore: 0)
Eighteenth-Century Music     Hybrid Journal   (Followers: 15, SJR: 0.113, CiteScore: 0)
English Language and Linguistics     Hybrid Journal   (Followers: 24, SJR: 0.52, CiteScore: 1)
English Profile J.     Hybrid Journal   (Followers: 2)
English Today     Hybrid Journal   (Followers: 13, SJR: 0.279, CiteScore: 0)
Enterprise & Society : The Intl. J. of Business History     Hybrid Journal   (Followers: 18, SJR: 0.245, CiteScore: 1)
Environment and Development Economics     Hybrid Journal   (Followers: 39, SJR: 0.617, CiteScore: 1)
Environmental Conservation     Hybrid Journal   (Followers: 60, SJR: 1.028, CiteScore: 2)
Environmental Practice     Full-text available via subscription   (Followers: 3, SJR: 0.145, CiteScore: 0)
Epidemiology & Infection     Hybrid Journal   (Followers: 22, SJR: 1.128, CiteScore: 2)
Epidemiology and Psychiatric Sciences     Hybrid Journal   (Followers: 3, SJR: 1.494, CiteScore: 2)
Episteme     Hybrid Journal   (Followers: 12, SJR: 0.756, CiteScore: 1)
Ergodic Theory and Dynamical Systems     Hybrid Journal   (Followers: 2, SJR: 1.193, CiteScore: 1)
Ethics & Intl. Affairs     Full-text available via subscription   (Followers: 17, SJR: 0.557, CiteScore: 1)
European Constitutional Law Review (EuConst)     Full-text available via subscription   (Followers: 35, SJR: 1.009, CiteScore: 1)
European J. of Applied Mathematics     Hybrid Journal   (SJR: 0.52, CiteScore: 1)
European J. of Intl. Security     Hybrid Journal   (Followers: 1)
European J. of Sociology     Hybrid Journal   (Followers: 36, SJR: 0.643, CiteScore: 1)
European Political Science Review     Hybrid Journal   (Followers: 27, SJR: 1.816, CiteScore: 2)
European Review     Hybrid Journal   (Followers: 19, SJR: 0.131, CiteScore: 0)
Evolutionary Human Sciences     Open Access   (Followers: 1)
Experimental Agriculture     Hybrid Journal   (Followers: 14, SJR: 0.542, CiteScore: 1)
Expert Reviews in Molecular Medicine     Hybrid Journal   (Followers: 1, SJR: 1.647, CiteScore: 4)
Fetal and Maternal Medicine Review     Hybrid Journal   (Followers: 6)
Financial History Review     Full-text available via subscription   (Followers: 15, SJR: 0.238, CiteScore: 1)
Foreign Policy Bulletin     Hybrid Journal   (Followers: 6)
Forum of Mathematics, Pi     Open Access   (Followers: 1)
Forum of Mathematics, Sigma     Open Access   (Followers: 1)
Genetics Research     Hybrid Journal   (Followers: 4, SJR: 0.483, CiteScore: 1)
Geological Magazine     Hybrid Journal   (Followers: 16, SJR: 0.966, CiteScore: 2)
Glasgow Mathematical J.     Full-text available via subscription   (SJR: 0.604, CiteScore: 0)
Global Constitutionalism     Hybrid Journal   (Followers: 17)
Global Mental Health     Open Access   (Followers: 9)
Global Sustainability     Open Access   (Followers: 1)
Government and Opposition     Full-text available via subscription   (Followers: 24, SJR: 0.965, CiteScore: 2)
Greece & Rome     Partially Free   (Followers: 30, SJR: 0.113, CiteScore: 0)
Hague J. on the Rule of Law     Full-text available via subscription   (Followers: 14, SJR: 0.271, CiteScore: 1)
Harvard Theological Review     Full-text available via subscription   (Followers: 78, SJR: 0.165, CiteScore: 0)
Health Economics, Policy and Law     Hybrid Journal   (Followers: 29, SJR: 0.745, CiteScore: 1)
Hegel Bulletin     Full-text available via subscription   (Followers: 2)
High Power Laser Science and Engineering     Open Access   (Followers: 3, SJR: 0.901, CiteScore: 3)
Historical J.     Hybrid Journal   (Followers: 39, SJR: 0.247, CiteScore: 1)
History in Africa     Full-text available via subscription   (Followers: 9)
Horizons     Partially Free   (Followers: 1, SJR: 0.129, CiteScore: 0)
Industrial and Organizational Psychology     Hybrid Journal   (Followers: 27, SJR: 0.916, CiteScore: 1)
Infection Control and Hospital Epidemiology     Full-text available via subscription   (Followers: 43, SJR: 1.97, CiteScore: 3)
Intl. & Comparative Law Quarterly     Full-text available via subscription   (Followers: 260, SJR: 0.369, CiteScore: 1)
Intl. Annals of Criminology     Full-text available via subscription  
Intl. J. of Asian Studies     Hybrid Journal   (Followers: 13, SJR: 0.143, CiteScore: 0)
Intl. J. of Astrobiology     Hybrid Journal   (Followers: 4, SJR: 0.548, CiteScore: 1)
Intl. J. of Cultural Property     Full-text available via subscription   (Followers: 13, SJR: 0.253, CiteScore: 1)
Intl. J. of Disability Management Research     Full-text available via subscription   (Followers: 9, SJR: 0.105, CiteScore: 0)
Intl. J. of Law in Context     Hybrid Journal   (Followers: 17, SJR: 0.275, CiteScore: 1)
Intl. J. of Legal Information     Full-text available via subscription   (Followers: 341)
Intl. J. of Microwave and Wireless Technologies     Hybrid Journal   (Followers: 10, SJR: 0.184, CiteScore: 1)
Intl. J. of Middle East Studies     Hybrid Journal   (Followers: 72, SJR: 0.434, CiteScore: 0)
Intl. J. of Technology Assessment in Health Care     Hybrid Journal   (Followers: 14, SJR: 0.714, CiteScore: 1)
Intl. Labor and Working-Class History     Full-text available via subscription   (Followers: 14, SJR: 0.182, CiteScore: 0)
Intl. Organization     Full-text available via subscription   (Followers: 109, SJR: 8.527, CiteScore: 5)
Intl. Psychogeriatrics     Hybrid Journal   (Followers: 12, SJR: 1.048, CiteScore: 2)
Intl. Review of Social History     Full-text available via subscription   (Followers: 27, SJR: 0.315, CiteScore: 1)
Intl. Review of the Red Cross     Full-text available via subscription   (Followers: 13, SJR: 0.214, CiteScore: 0)
Intl. Theory: A J. of Intl. Politics, Law and Philosophy     Hybrid Journal   (Followers: 19, SJR: 2.293, CiteScore: 2)
Iraq     Full-text available via subscription   (Followers: 4)
Irish Historical Studies     Hybrid Journal   (Followers: 7, SJR: 0.103, CiteScore: 0)
Irish J. of Psychological Medicine     Hybrid Journal   (Followers: 2, SJR: 0.221, CiteScore: 0)
Israel Law Review     Hybrid Journal   (Followers: 2, SJR: 0.165, CiteScore: 0)
Italian Political Science Review / Rivista Italiana di Scienza Politica     Hybrid Journal  
Itinerario     Full-text available via subscription   (Followers: 11, SJR: 0.158, CiteScore: 0)
J. of African History     Hybrid Journal   (Followers: 22, SJR: 0.348, CiteScore: 1)
J. of African Law     Full-text available via subscription   (Followers: 3, SJR: 0.113, CiteScore: 0)
J. of Agricultural and Applied Economics     Open Access   (Followers: 3, SJR: 0.263, CiteScore: 1)
J. of Agricultural Science     Full-text available via subscription   (Followers: 8, SJR: 0.563, CiteScore: 1)
J. of American Studies     Hybrid Journal   (Followers: 20, SJR: 0.164, CiteScore: 0)
J. of Anglican Studies     Hybrid Journal   (Followers: 7, SJR: 0.101, CiteScore: 0)
J. of Applied Animal Nutrition     Hybrid Journal   (Followers: 4)
J. of Asian Studies     Full-text available via subscription   (Followers: 42, SJR: 0.591, CiteScore: 1)
J. of Benefit-Cost Analysis     Hybrid Journal   (Followers: 4)
J. of Biosocial Science     Hybrid Journal   (Followers: 5, SJR: 0.48, CiteScore: 1)
J. of British Studies     Full-text available via subscription   (Followers: 32, SJR: 0.246, CiteScore: 0)

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Similar Journals
Journal Cover
Cardiology in the Young
Journal Prestige (SJR): 0.372
Citation Impact (citeScore): 1
Number of Followers: 34  
 
  Hybrid Journal Hybrid journal (It can contain Open Access articles)
ISSN (Print) 1047-9511 - ISSN (Online) 1467-1107
Published by Cambridge University Press Homepage  [387 journals]
  • Echocardiographic examination of mitral valve abnormalities in the
           paediatric population: current practices
    • Authors: Massimiliano Cantinotti; Raffaele Giordano, Martin Koestenberger, Inga Voges, Giuseppe Santoro, Eliana Franchi, Nadia Assanta, Israel Valverde, John Simpson, Shelby Kutty
      Pages: 1 - 11
      Abstract: We reviewed the recent literature for echocardiographic assessment of mitral valve abnormalities in children. A literature search was performed within the National Library of Medicine using the keywords “mitral regurgitation and/or stenosis, children.” The search was refined by adding the keywords “echocardiographic definition, classification, and evaluation.” Thirty-one studies were finally included. Significant advances in echocardiographic imaging of mitral valve defects, mainly due to the implementation of three-dimensional technology, contribute to a better understanding of the underlying anatomy. However, heterogeneity between classification systems of mitral valve disease severity is a serious problem. For regurgitant lesions, there is only very limited evidence from small studies that support the adoption of quantitative/semi-quantitative indexes commonly employed in adults. Despite the lack of evidence base, qualitative evaluation of regurgitation severity is often employed. For stenotic lesions, no clear categorisation based on trans-valvular echocardiography-derived “gradients” has been consistently applied to define mild, moderate, or severe obstruction across different paediatric age ranges. Quantitative parameters such as valve area have also been poorly validated in children. Adult recommendations are frequently applied without validation for the paediatric age. In conclusion, significant advances in the anatomical evaluation of mitral valve diseases have been made, thanks to three-dimensional echocardiography; however, limitations remain in the quantitative/semi-quantitative estimation of disease severity, both with respect to valvular regurgitation and stenosis. Because adult echocardiographic recommendations should not be simply translated to the paediatric age, more specific paediatric guidelines and standards for the assessment of mitral valve diseases are needed.
      PubDate: 2020-01-01T00:00:00.000Z
      DOI: 10.1017/S1047951119003196
      Issue No: Vol. 30, No. 1 (2020)
       
  • Left ventricular non-compaction in patients with single ventricle heart
           disease
    • Authors: Preeti Choudhary; Wendy Strugnell, Rajesh Puranik, Christian Hamilton-Craig, Shelby Kutty, David S Celermajer
      Pages: 12 - 18
      Abstract: Objective:Left ventricular non-compaction is an architectural abnormality of the myocardium, associated with heart failure, systemic thromboembolism, and arrhythmia. We sought to assess the prevalence of left ventricular non-compaction in patients with single ventricle heart disease and its effects on ventricular function.Methods:Cardiac MRI of 93 patients with single ventricle heart disease (mean age 24 ± 8 years; 55% male) from three tertiary congenital centres was retrospectively reviewed; 65 of these had left ventricular morphology and are the subject of this report. The presence of left ventricular non-compaction was defined as having a non-compacted:compacted (NC:C) myocardial thickness ratio >2.3:1. The distribution of left ventricular non-compaction, ventricular volumes, and function was correlated with clinical data.Results:The prevalence of left ventricular non-compaction was 37% (24 of 65 patients) with a mean of 4 ± 2 affected segments. The distribution was apical in 100%, mid-ventricular in 29%, and basal in 17% of patients. Patients with left ventricular non-compaction had significantly higher end-diastolic (128 ± 44 versus 104 ± 46 mL/m2, p = 0.047) and end-systolic left ventricular volumes (74 ± 35 versus 56 ± 35 mL/m2, p = 0.039) with lower left ventricular ejection fraction (44 ± 11 versus 50 ± 9%, p = 0.039) compared to those with normal compaction. The number of segments involved did not correlate with ventricular function (p = 0.71).Conclusions:Left ventricular non-compaction is frequently observed in patients with left ventricle-type univentricular hearts, with predominantly apical and mid-ventricular involvement. The presence of non-compaction is associated with increased indexed end-diastolic volumes and impaired systolic function.
      PubDate: 2020-01-01T00:00:00.000Z
      DOI: 10.1017/S1047951119001872
      Issue No: Vol. 30, No. 1 (2020)
       
  • Incidence and outcomes of prosthetic valve endocarditis in adults with
           tetralogy of Fallot
    • Authors: Alexander C. Egbe; Srikanth Kothapalli, William R. Miranda, Raja Jadav, Keerthana Banala, Rahul Vojjini, Faizan Faizee, Fouad Khalil, Maria Najam, Mounika Angirekula, Daniel C. Desimone, Heidi M. Connolly
      Pages: 19 - 23
      Abstract: Background:The risk of endocarditis varies with CHD complexity and the presence of prosthetic valves. The purpose of the study was therefore to describe incidence and outcomes of prosthetic valve endocarditis in adults with repair tetralogy of Fallot.Methods:Retrospective review of adult tetralogy of Fallot patients who underwent prosthetic valve implantation, 1990–2017. We defined prosthetic valve endocarditis-related complications as prosthetic valve dysfunction, perivalvular extension of infection such abscess/aneurysm/fistula, heart block, pulmonary/systemic embolic events, recurrent endocarditis, and death due to sepsis.Results:A total of 338 patients (age: 37 ± 15 years) received 352 prosthetic valves (pulmonary [n = 308, 88%], tricuspid [n = 13, 4%], mitral [n = 9, 3%], and aortic position [n = 22, 6%]). The annual incidence of prosthetic valve endocarditis was 0.4%. There were 12 prosthetic valve endocarditis-related complications in six patients, and these complications were prosthetic valve dysfunction (n = 4), systemic/pulmonary embolic events (n = 2), heart block (n = 1), aortic root abscess (n = 1), recurrent endocarditis (n = 2), and death due to sepsis (n = 1). Three (50%) patients required surgery at 2 days, 6 weeks, and 23 weeks from the time of prosthetic valve endocarditis diagnosis. Altogether three of the six (50%) patients died, and one of these deaths was due to sepsis.Conclusions:The incidence, complication rate, and outcomes of prosthetic valve endocarditis in tetralogy of Fallot patients underscore some of the risks of having a prosthetic valve. It is important to educate the patients on the need for early presentation if they develop systemic symptoms, have a high index of suspicion for prosthetic valve endocarditis, and adopt a multi-disciplinary care approach in this high-risk population.
      PubDate: 2020-01-01T00:00:00.000Z
      DOI: 10.1017/S1047951119001975
      Issue No: Vol. 30, No. 1 (2020)
       
  • A surgical technique for ascending aorta, aortic arch and descending aorta
           replacement without cross-clamp, circulatory arrest or hypothermia
    • Authors: Murat Ugurlucan; Yahya Yildiz, Mustafa O. Ulukan, Didem M. Oztas, Metin O. Beyaz, Emin Canata, Senay Coban, Orcun Unal, Korhan Erkanli, Halil Turkoglu
      Pages: 24 - 27
      Abstract: Treatment of the aneurysms comprising the aortic arch is challenging. Surgical reconstruction usually requires aortic cross-clamping, cardiac arrest, and even deep hypothermia for a bloodless field. In this report, we present our surgical technique providing normothermic ascending aorta, aortic arch, and proximal descending aorta replacement with selective cannulation and perfusion of the whole body.
      PubDate: 2020-01-01T00:00:00.000Z
      DOI: 10.1017/S1047951119003159
      Issue No: Vol. 30, No. 1 (2020)
       
  • A tool for routine monitoring and feedback of morbidities following
           paediatric cardiac surgery
    • Authors: Luca Grieco; Christina Pagel, Martin Utley, David J. Barron, Serban Stoica, Shane Tibby, Warren Rodrigues, Victor Tsang, Katherine L. Brown
      Pages: 28 - 33
      Abstract: Short-term survival after paediatric cardiac surgery has improved significantly over the past 20 years and increasing attention is being given to measuring and reducing incidence of morbidities following surgery. How to best use routinely collected data to share morbidity information constitutes a challenge for clinical teams interested in analysing their outcomes for quality improvement. We aimed to develop a tool facilitating this process in the context of monitoring morbidities following paediatric cardiac surgery, as part of a prospective multi-centre research study in the United Kingdom.We developed a prototype software tool to analyse and present data about morbidities associated with cardiac surgery in children. We used an iterative process, involving engagement with potential users, tool design and implementation, and feedback collection. Graphical data displays were based on the use of icons and graphs designed in collaboration with clinicians.Our tool enables automatic creation of graphical summaries, displayed as a Microsoft PowerPoint presentation, from a spreadsheet containing patient-level data about specified cardiac surgery morbidities. Data summaries include numbers/percentages of cases with morbidities reported, co-occurrences of different morbidities, and time series of each complication over a time window.Our work was characterised by a very high level of interaction with potential users of the tool, enabling us to promptly account for feedback and suggestions from clinicians and data managers. The United Kingdom centres involved in the project received the tool positively, and several expressed their interest in using it as part of their routine practice.
      PubDate: 2020-01-01T00:00:00.000Z
      DOI: 10.1017/S1047951119002956
      Issue No: Vol. 30, No. 1 (2020)
       
  • Outcome of congenital tracheal stenosis in children over two decades in a
           national cardiothoracic surgical unit
    • Authors: Colin J. McMahon; Karim Ayoubi, Rania Mehanna, Eithne Phelan, Eoin O’Cearbhaill, John Russell, Lars Nölke
      Pages: 34 - 38
      Abstract: Objective:To assess the outcomes of congenital tracheal stenosis among children.Materials and methods:A retrospective review of all children who underwent surgical repair of congenital tracheal stenosis reviewing charts, operative notes, echocardiograms, CT and MRI data from January 2002 to February 2019.Results:Twenty-six children underwent surgical treatment for tracheal stenosis. The median age was 3 months (range 0.3–35 months) and the median weight was 4.7 kg (range 2.5–13 kg) at the time of surgical intervention. Stridor was the most common presenting symptom in 17 patients (65% of patients). Twenty-one patients (81%) had concurrent cardiac anomalies, with pulmonary arterial sling being the most common, present in nine patients (34%). Extracorporeal life support was utilised in seven patients (27%) pre-operatively. Laryngeal release was required in 16 patients. In 7 patients an end-to-end anastomosis was performed, in 18 patients slide tracheoplasty, and 1 patient had a double slide tracheoplasty. The median cardiopulmonary bypass time was 106 minutes (range 25–255 minutes). The median cross-clamp time was 30 minutes (range 5–67 minutes). The median post-operative duration of ventilation was 5 days (range 0.5–16 days). The median ICU length of stay was 12.5 days (range 2–60 days). There were three hospital mortalities with 88% survival. One patient only required reintervention with balloon dilation. Twenty-two patients (85%) remained symptom-free on median follow-up at 7.6 years (range 0.2–17 years). Two patients since 2017 had 3D printed tracheas produced from CT imaging to assist surgical planning.Conclusion:Congenital tracheal stenosis can be managed effectively with excellent outcomes and 3D printed models assist in planning the optimal surgical intervention.
      PubDate: 2020-01-01T00:00:00.000Z
      DOI: 10.1017/S1047951119002725
      Issue No: Vol. 30, No. 1 (2020)
       
  • Prenatal diagnosis of anomalous origin of one pulmonary artery branch by
           two-dimensional echocardiography: summary of 12 cases
    • Authors: Li Wenxiu; Zhang Yuan, Huang Chaoning, Geng Bin, Wu Jiang, Yang Shuang
      Pages: 39 - 46
      Abstract: Objectives:To improve the prenatal diagnosis for anomalous origin of pulmonary artery branches by comparing and analyzing different types of fetal echocardiography features.Methods:Between June 2012 and December 2018, fetal echocardiographic features were analyzed retrospectively from fetuses with a prenatal diagnosis of anomalous origin of pulmonary artery branch. The main points of identification were summarized.Results:A total of 12 fetuses were diagnosed, including anomalous origin of a pulmonary artery branch from the innominate artery and six cases with unilateral absence of pulmonary artery. The shared characteristic sonographic finding was the lack of confluence at the bifurcation of the main pulmonary artery. The differences between the two conditions are highlighted by the origin of the anomalous vessel. In fetuses with anomalous origin of one pulmonary artery branch, the affected pulmonary artery arose from the posterior wall of the ascending aorta as noted on three vessels and trachea view as well as the long axis of the left ventricular outflow tract. This is in contrast to fetuses with unilateral absence of pulmonary artery, where the origin of affected pulmonary artery arises from the base of the innominate artery via the ipsilateral patent arterial duct as evident on the three vessels and trachea view and the coronal view of innominate (brachiocephalic) artery.Conclusion:(1) The main similarity is an absence of a confluence at the bifurcation of the main pulmonary artery. (2) The main distinguishing feature is the origin of the anomalous vessel from either the subclavian or directly from the aorta.
      PubDate: 2020-01-01T00:00:00.000Z
      DOI: 10.1017/S1047951119002890
      Issue No: Vol. 30, No. 1 (2020)
       
  • Aortopulmonary window with anomalous right coronary artery from the
           pulmonary artery. Case report and literature review
    • Authors: Ali A. Alakhfash; Mohamad Tagelden, Abdulrahman Almesned, Abdullah Alqwaiee
      Pages: 47 - 49
      Abstract: Aortopulmonary window is a rare congenital heart lesion. It might be associated with other CHDs, as well as with anomalous origin of the coronary arteries. Anomalous origin of the right coronary artery from the pulmonary artery (ARCAPA) is the most commonly described coronary artery anomaly in association with aortopulmonary window. We are describing a premature neonate who was diagnosed to have aortopulmonary window and ARCAPA immediately after birth, and had a successful operation at the age of 4 months. This report highlights the importance of very careful assessment of the coronary arteries in patients with aortopulmonary window.
      PubDate: 2020-01-01T00:00:00.000Z
      DOI: 10.1017/S1047951119002543
      Issue No: Vol. 30, No. 1 (2020)
       
  • Heart failure biomarker levels correlate with invasive haemodynamics in
           pulmonary valve replacement
    • Authors: Phillip M. Zegelbone; Richard E. Ringel, John D. Coulson, Melanie K. Nies, Meagan E. Stabler, Jeremiah R. Brown, Allen D. Everett
      Pages: 50 - 54
      Abstract: Background:Although widely used in cardiology, relation of heart failure biomarkers to cardiac haemodynamics in patients with CHD (and in particular with pulmonary insufficiency undergoing pulmonary valve replacement) remains unclear. We hypothesised that the cardiac function biomarkers N-terminal pro-brain natriuretic peptide (NT-proBNP), soluble suppressor of tumorigenicity 2, and galectin-3 would have significant associations to right ventricular haemodynamic derangements.Methods:Consecutive patients ( n = 16) undergoing cardiac catheterisation for transcatheter pulmonary valve replacement were studied. NT-proBNP, soluble suppressor of tumorigenicity 2, and galectin-3 levels were measured using a multiplex enzyme-linked immunosorbent assay from a pre-intervention blood sample obtained after sheath placement. Spearman correlation was used to identify significant correlations (p ≤ 0.05) of biomarkers with baseline cardiac haemodynamics. Cardiac MRI data (indexed right ventricular and left ventricular end-diastolic volumes and ejection fraction) prior to device placement were also compared to biomarker levels.Results:NT-proBNP and soluble suppressor of tumorigenicity 2 were significantly correlated (p < 0.01) with baseline mean right atrial pressure and right ventricular end-diastolic pressure. Only NT-proBNP was significantly correlated with age. Galectin-3 did not have significant associations in this cohort. Cardiac MRI measures of right ventricular function and volume were not correlated to biomarker levels or right heart haemodynamics.Conclusions:NT-proBNP and soluble suppressor of tumorigenicity 2, biomarkers of myocardial strain, significantly correlated to invasive pressure haemodynamics in transcatheter pulmonary valve replacement patients. Serial determination of soluble suppressor of tumorigenicity 2, as it was not associated with age, may be superior to serial measurement of NT-proBNP as an indicator for timing of pulmonary valve replacement.
      PubDate: 2020-01-01T00:00:00.000Z
      DOI: 10.1017/S1047951119002737
      Issue No: Vol. 30, No. 1 (2020)
       
  • The effect of milrinone on hemodynamic and gas exchange parameters in
           children
    • Authors: Rohit S. Loomba; Vincent Dorsey, Enrique G. Villarreal, Saul Flores
      Pages: 55 - 61
      Abstract: Milrinone is a drug frequently used for hemodynamic support in children during critical illness. Although the hemodynamic changes induced by milrinone in children may appear similar to those of adults, the physiologic contributors of these changes remain vastly unknown. A systematic review was conducted to identify studies characterising the hemodynamic effects of milrinone in children during critical illness for hemodynamic support for various medical conditions. Studies were assessed for quality and those of satisfactory quality with pre- and post-operative hemodynamics for each patient were included in the final analyses. Those not limited to children and those not limited to patients with critical illness were excluded from the final analyses. A total of six studies with 791 patients were included in the final analyses. Milrinone infusion doses ranged from 0.3 to 0.75 mcg/kg/minute with the mean infusion dose being 0.5 mcg/kg/minute. Patients whom received milrinone infusion had greater cardiac output, greater left ventricle shortening fraction, lower right ventricular systolic pressure, and lower serum lactate levels. Systolic blood pressure mean arterial blood pressure and arterial oxygen concentration did not significantly change with administration of milrinone. These results were irrespective of milrinone infusion dose, infusion duration, and study size. Milrinone was found to have several beneficial hemodynamic effects in children during critical illness when used at usual clinical doses.
      PubDate: 2020-01-01T00:00:00.000Z
      DOI: 10.1017/S1047951119002865
      Issue No: Vol. 30, No. 1 (2020)
       
  • Characteristics and outcomes of children with congenital heart disease
           needing diaphragm plication
    • Authors: Cortney B. Foster; Antonio G. Cabrera, Dayanand Bagdure, William Blackwelder, Brady S Moffett, Adrian Holloway, Vladimir Mishcherkin, Adnan Bhutta
      Pages: 62 - 65
      Abstract: Background:Diaphragm dysfunction following surgery for congenital heart disease is a known complication leading to delays in recovery and increased post-operative morbidity and mortality. We aimed to determine the incidence of and risk factors associated with diaphragm plication in children undergoing cardiac surgery and evaluate timing to repair and effects on hospital cost and length of stay.Methods:We conducted a multi-institutional retrospective observational cohort study. Forty-three hospitals from the Pediatric Health Information System database were included, and a total of 112,110 patients admitted between January 2004 and December 2014 were analysed.Results:Patients less than 18 years of age who underwent cardiac surgery were included. Risk Adjustment for Congenital Heart Surgery was utilized to determine procedure complexity. The overall incidence of diaphragm dysfunction was 2.2% (n = 2513 out of 112,110). Of these, 24.0% (603 patients) underwent diaphragm plication. Higher complexity cardiac surgery (Risk Adjustment for Congenital Heart Surgery 5–6) and age less than 4 weeks were associated with a higher likelihood of diaphragm plication (p-value < 0.01). Diaphragmatic plication was associated with increased hospital length of stay (p-value < 0.01) and increased medical cost.Conclusions:Diaphragm plication after surgery for congenital heart disease is associated with longer hospital length of stay and increased cost. There is a strong correlation of prolonged time to plication with increased length of stay and medical cost. The likelihood of plication increases with younger age and higher procedure complexity. Methods to improve early recognition and treatment of diaphragm dysfunction should be developed.
      PubDate: 2020-01-01T00:00:00.000Z
      DOI: 10.1017/S1047951119002671
      Issue No: Vol. 30, No. 1 (2020)
       
  • Association between preoperative respiratory support and outcomes in
           paediatric cardiac surgery
    • Authors: Elizabeth C. Ciociola; Karan R. Kumar, Kanecia O. Zimmerman, Elizabeth J. Thompson, Melissa Harward, Laura N. Sullivan, Joseph W. Turek, Christoph P. Hornik
      Pages: 66 - 73
      Abstract: Background:Preoperative mechanical ventilation is associated with morbidity and mortality following CHD surgery, but prior studies lack a comprehensive analysis of how preoperative respiratory support mode and timing affects outcomes.Methods:We retrospectively collected data on children
      PubDate: 2020-01-01T00:00:00.000Z
      DOI: 10.1017/S1047951119002786
      Issue No: Vol. 30, No. 1 (2020)
       
  • Post-operative blood loss is higher among African American neonates
           undergoing open-heart surgery with cardiopulmonary bypass for CHD
    • Authors: Vyas M. Kartha; Mohamed Rehman, Anh Thy H. Nguyen, Ernest Amankwah, Erica M.S. Sibinga, Neil A. Goldenberg, Jeffrey P. Jacobs
      Pages: 74 - 81
      Abstract: Background:Neonates are at high risk of bleeding after open-heart surgery. We sought to determine pre-operative and intra-operative risk factors for increased bleeding after neonatal open-heart surgery with cardiopulmonary bypass.Methods:We conducted a retrospective cohort study of neonates (0–30 days old) who underwent open-heart surgery with cardiopulmonary bypass from January, 2009, to March, 2013. Cardiac diagnosis; demographic and surgical data; and blood products, haemostatic agents, and anti-thrombotic agents administered before, during, and within 24 hours after surgery were abstracted from the electronic health record and anaesthesia records. The outcome of interest was chest tube output (in ml/kg body weight) within 24 hours. Relationships between chest tube output and putative associated factors were evaluated by unadjusted and adjusted linear regression.Results:The cohort consisted of 107 neonates, of whom 79% had a Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery (STAT) Mortality Category of 4 or 5. Median chest tube output was 37 ml/kg (range 9–655 ml/kg). Age, African-American race, and longer durations of surgery and cardiopulmonary bypass each had statistically significant associations with increased chest tube output in unadjusted analyses. In multivariable analysis, African-American race retained an independent, statistically significant association with increased chest tube output; the geometric mean of chest tube output among African-American neonates was 71% higher than that of Caucasians (95% confidence interval, 29–125%; p = 0.001).Conclusion:Among neonates with CHD undergoing open-heart surgery with cardiopulmonary bypass, African-American race is independently associated with greater chest tube output over the first 24 hours post-operatively.
      PubDate: 2020-01-01T00:00:00.000Z
      DOI: 10.1017/S1047951119002683
      Issue No: Vol. 30, No. 1 (2020)
       
  • Association between physical activity level and blood pressure: varied and
           graded mediating effects of obesity indices in schoolchildren
    • Authors: Fatai A. Maruf; Marufat O. Odetunde, Prosper U. Okonkwo
      Pages: 82 - 88
      Abstract: Objective:To explore the mediating effects of adiposity indices in the association between physical activity level and blood pressure in a Nigerian schoolchildren population.Materials and Methods:One thousand five hundred and seventeen schoolchildren (714 males and 803 females) from randomly selected primary schools participated. Physical activity level, sum of skinfold thickness at three sites, waist circumference, body mass index, and blood pressure were measured using standardised procedures. The statistical significance of the mediating effects of adiposity indices was determined using Sobel Test.Results:Some obesity indices mediated the association between physical activity level and systolic blood pressure [males: waist circumference (t = 5.31; p < 0.001), skinfold thickness (t = 3.80; p < 0.001), and waist circumference/height (t = 2.21; p < 0.001); females: body mass index (t = 8.03; p < 0.001), waist circumference (t = 7.80; p < 0.001), and skinfold thickness (t = 5.94; p < 0.001)]. Similarly, some obesity indices mediated the prediction of diastolic blood pressure in females [males: body mass index (t = 1.95; p = 0.05), waist circumference (t = 2.65; p = 0.01), and skinfold thickness (t = 1.97; p = 0.05); females blood pressure: body mass index (t = 6.49; p < 0.001), waist circumference (t = 6.29; p < 0.001), skinfold thickness (t = 2.31; p = 0.02), and waist circumference/height (t = 2.59; p = 0.01)].Conclusion:The obesity indices that mediate the association between physical activity level and blood pressure vary, and their mediating effects are graded. While waist circumference and skinfold thickness exert the greatest mediating effects on the association in males, body mass index and waist circumference do in females.
      PubDate: 2020-01-01T00:00:00.000Z
      DOI: 10.1017/S1047951119003172
      Issue No: Vol. 30, No. 1 (2020)
       
  • Early weight trends after congenital heart surgery and their determinants
    • Authors: Nayan Banerji; Abish Sudhakar, Rakhi Balachandran, Gopalraj Sumangala Sunil, Brijesh P. Kotayil, Raman Krishna Kumar
      Pages: 89 - 94
      Abstract: Background:Early weight trends after cardiac surgery in infants from low- and middle-income countries where the majority are undernourished have not been defined. We studied the early post-operative weight trends to identify specific factors associated with early weight loss and poor weight gain after discharge following congenital heart surgery in consecutive infants undergoing cardiac surgery at a referral hospital in Southern India.Methods:This was a prospective observational study. Weights of the babies were recorded at different time points during the hospital stay and at 1-month post-discharge. A comprehensive database of pre-operative, operative, and post-operative variables was created and entered into a multivariate logistic regression analysis model to identify factors associated with excessive early weight loss after cardiac surgery, and poor weight gain following hospital discharge.Results:The study enrolled 192 infants (mean age 110.7 ± 99.9 days; weight z scores − 2.5 ± 1.5). There was a small but significant (p < 0.001) decline in weight in the hospital following surgery (1.6% decline (interquartile range −5.3 to +1.7)); however, there was substantial growth following discharge (26.7% increase (interquartile range 15.3–41.8)). The variables associated with post-operative weight loss were cumulative nil-per-oral duration and cardiopulmonary bypass time, while weight gain following discharge was only associated with age.Conclusion:Weight loss is almost universal early after congenital heart surgery and is associated with complex surgery and cumulative nil-per-oral duration. After discharge, weight gain is almost universal and not associated with any of the perioperative variables.
      PubDate: 2020-01-01T00:00:00.000Z
      DOI: 10.1017/S1047951119002944
      Issue No: Vol. 30, No. 1 (2020)
       
  • Spontaneous aortic thrombosis in neonates: a case report and review of
           literature
    • Authors: John P. Mulcaire-Jones; David K. Bailly, Deborah U. Frank, Anupam R. Verma, Bradley J. Barney, Heather M. Siefkes
      Pages: 95 - 99
      Abstract: Neonatal aortic thrombosis is a rare occurrence but can be life-threatening. Most aortic thrombosis in neonates is related to umbilical artery catheters. A case of a neonate with a spontaneous aortic thrombosis is described here along with a comprehensive review of the literature for cases of neonatal aortic thrombosis not related to any intravascular device or procedure. The aetiologies of these spontaneous thromboses and the relevance of hypercoagulable disorders are discussed. The cases were analysed for odds of death by treatment method adjusted for era. The reference treatment method was thrombolysis and anticoagulation. No other treatment modality had significantly lower odds than the reference. Surgery alone had higher odds for death than the reference, but this may be confounded by severity of case. The management recommendations for clinicians encountering neonates with spontaneous neonatal aortic thrombosis are discussed.
      PubDate: 2020-01-01T00:00:00.000Z
      DOI: 10.1017/S1047951119003093
      Issue No: Vol. 30, No. 1 (2020)
       
  • MYBPC3+c.2737+1+(IVS26)+G>T+mutation+responsible+for+high-risk+hypertrophic+cardiomyopathy&rft.title=Cardiology+in+the+Young&rft.issn=1047-9511&rft.date=2020&rft.volume=30&rft.spage=100&rft.epage=106&rft.aulast=Tong&rft.aufirst=Wuyang&rft.au=Wuyang+Tong&rft.au=Wei+Liu,+Hong+Guo,+Jiang+Wang,+Shiyong+Yu,+Jihang+Zhang,+Chuan+Liu,+Jia+Chen,+Xiaohui+Zhao&rft_id=info:doi/10.1017/S1047951119002701">A novel MYBPC3 c.2737+1 (IVS26) G>T mutation responsible for high-risk
           hypertrophic cardiomyopathy
    • Authors: Wuyang Tong; Wei Liu, Hong Guo, Jiang Wang, Shiyong Yu, Jihang Zhang, Chuan Liu, Jia Chen, Xiaohui Zhao
      Pages: 100 - 106
      Abstract: Background:Hypertrophic cardiomyopathy is an autosomal dominant hereditary disease characterised by left ventricular asymmetry hypertrophy. However, our knowledge of the genetic background in hypertrophic cardiomyopathy cases is limited. Here, we aimed to evaluate pathogenic gene mutations in a family with high-risk hypertrophic cardiomyopathy and analyse the genotype/phenotype relationships in this family.Methods:The proband, her parents, and her niece underwent whole-exome sequencing, and the genotypes of family members were identified using Sanger sequencing. mRNA expression was detected using reverse transcription sequencing. Structural impairments were predicted by homologous modelling. A family survey was conducted for patients with positive results to obtain information on general clinical symptoms, electrocardiography, ambulatory electrocardiography, echocardiography, and 3.0T cardiac magnetic resonance findings. Regular follow-up was performed for up to 6 months.Results:Five family members, including the proband, carried a cleavage site mutation in the MYBPC3 gene (c.2737+1 (IVS26) G>T), causing exon 26 of the MYBPC3 gene transcript to be skipped and leading to truncation of cardiac myosin-binding protein C. Family survey showed that the earliest onset age was 13 years old, and three people had died suddenly at less than 40 years old. Three pathogenic gene carriers were diagnosed with hypertrophic cardiomyopathy, and all showed severe ventricular septal hypertrophy.Conclusion:The c.2737+1 (IVS26) G>T mutation in the MYBPC3 gene led to exon 26 skipping, thereby affecting the structure and function of cardiac myosin-binding protein C and leading to severe ventricular hypertrophy and sudden death.
      PubDate: 2020-01-01T00:00:00.000Z
      DOI: 10.1017/S1047951119002701
      Issue No: Vol. 30, No. 1 (2020)
       
  • Use of the terminal complement inhibitor eculizumab in paediatric heart
           transplant recipients
    • Authors: Yuk M. Law; Deipanjan Nandi, Kimberly Molina, Katheryn Gambetta, Kevin P. Daly, Bibhuti Das
      Pages: 107 - 113
      Abstract: Antibody-mediated rejection is a major clinical challenge that limits graft survival. Various modalities of treatment have been reported in small studies in paediatric heart recipients. A novel approach is to use complement-inhibiting agents, such as eculizumab, which inhibits cleavage of C5 to C5a thereby limiting the formation of membrane attack complex and terminal complement-mediated injury of tissue-bound antibodies. This medical modality of treatment has theoretical advantages but the collective experience in its use in the solid organ transplant community remains small. We add to this experience by combining 14 cases from 6 paediatric heart centres in this descriptive study.
      PubDate: 2020-01-01T00:00:00.000Z
      DOI: 10.1017/S1047951119003056
      Issue No: Vol. 30, No. 1 (2020)
       
  • The patient encounter index: a novel method of measuring clinical workload
           in a paediatric cardiology service
    • Authors: Michael J. Harrison; Oliver M. Barry, Rachel A. Hounsell, Rik De Decker
      Pages: 114 - 118
      Abstract: Technological advances have led to better patient outcomes and the expansion of clinical services in paediatric cardiology. This expansion creates an ever-growing workload for clinicians, which has led to workflow and staffing issues that need to be addressed. The objective of this study was the development of a novel tool to measure the clinical workload of a paediatric cardiology service in Cape Town, South Africa: The patient encounter index is a tool designed to quantify clinical workload. It is defined as a ratio of the measured duration of clinical work to the total time available for such work. This index was implemented as part of a prospective cross-sectional study design. Clinical workload data were collected over a 10-day period using time-and-motion sampling. Clinicians were contractually expected to spend 50% of their daily workload on patient care. The median patient encounter index for the Western Cape Paediatric Cardiac Service was 0.81 (range 0.19–1.09), reflecting that 81% of total contractual working time was spent on clinical activities. This study describes the development and implementation of a novel tool for clinical workload quantification and describes its application to a busy paediatric cardiology service in Cape Town, South Africa. This tool prospectively quantifies clinical workload which may directly influence patient outcomes. Implementation of this novel tool in the described setting clearly demonstrated the excessive workload of the clinical service and facilitated effective motivation for improved allocation of resources.
      PubDate: 2020-01-01T00:00:00.000Z
      DOI: 10.1017/S1047951119003068
      Issue No: Vol. 30, No. 1 (2020)
       
  • Congenital coronary collateral
    • Authors: Rita I. Moreira; Rui C. Ferreira
      Pages: 119 - 120
      Abstract: Intercoronary communications are a very rare congenital coronary artery anomalies. We report a case of a man who underwent elective coronary angiography that showed a bidirectional direct intercoronary communication between right coronary and left circumflex arteries.
      PubDate: 2020-01-01T00:00:00.000Z
      DOI: 10.1017/S1047951119002750
      Issue No: Vol. 30, No. 1 (2020)
       
  • Unilateral pulmonary aplasia and congenital diaphragmatic hernia
           associated with tetralogy of Fallot: a rare trifecta
    • Authors: Niraj N. Pandey; Mumun Sinha, Sanjeev Kumar
      Pages: 121 - 122
      Abstract: We present a case of a 7-month-old boy with tetralogy of Fallot associated with unilateral pulmonary aplasia and herniation of the liver and small bowel loops in the right hemithorax.
      PubDate: 2020-01-01T00:00:00.000Z
      DOI: 10.1017/S1047951119003251
      Issue No: Vol. 30, No. 1 (2020)
       
  • MYH11+mutation:+a+case+report&rft.title=Cardiology+in+the+Young&rft.issn=1047-9511&rft.date=2020&rft.volume=30&rft.spage=123&rft.epage=125&rft.aulast=Ardhanari&rft.aufirst=Mohanageetha&rft.au=Mohanageetha+Ardhanari&rft.au=Sethuraman+Swaminathan&rft_id=info:doi/10.1017/S1047951119003287">Congenital ductus arteriosus aneurysm in association with MYH11 mutation:
           a case report
    • Authors: Mohanageetha Ardhanari; Sethuraman Swaminathan
      Pages: 123 - 125
      Abstract: Congenital ductus arteriosus aneurysms develop in the third trimester of fetal life, possibly due to abnormal intimal cushion formation or elastin expression in the ductal wall. It is often diagnosed in infants before 2 months of age. Most have a benign course and resolve spontaneously. However, life-threatening complications have been reported. We report a case of large ductal aneurysm diagnosed incidentally in a neonate, in whom there was a novel mutation in the smooth muscle myosin protein gene—MYH11.
      PubDate: 2020-01-01T00:00:00.000Z
      DOI: 10.1017/S1047951119003287
      Issue No: Vol. 30, No. 1 (2020)
       
  • Tricuspid atresia with absent pulmonary valve and intact ventricular
           septum: successful bidirectional cavopulmonary anastomosis with complete
           exclusion of the right ventricle
    • Authors: Cheul Lee; Kyung Min Kim, Jae Young Lee, Jihong Yoon
      Pages: 126 - 128
      Abstract: Tricuspid atresia with absent pulmonary valve and intact ventricular septum is an extremely rare cardiac malformation, historically associated with a poor prognosis. Only a few cases with successful surgical palliation have been reported in the literature. We present the case of an 8-month-old infant with this malformation who underwent successful bidirectional cavopulmonary anastomosis with complete exclusion of the right ventricle.
      PubDate: 2020-01-01T00:00:00.000Z
      DOI: 10.1017/S1047951119002749
      Issue No: Vol. 30, No. 1 (2020)
       
  • A large ventricular fibroma requiring surgical resection in a symptomatic
           3-month-old infant
    • Authors: Anderson S. Marshall; Robert J. Dabal, Mark A. Law
      Pages: 129 - 130
      Abstract: Cardiac Fibromas are primary cardiac tumours more common in children than in adults. Surgical intervention is often not required except in the case of limited cardiac output or significant arrhythmia burden. We present a symptomatic 3-month-old infant who had successful surgical intervention for a giant right ventricle fibroma found on prenatal imaging.
      PubDate: 2020-01-01T00:00:00.000Z
      DOI: 10.1017/S1047951119002877
      Issue No: Vol. 30, No. 1 (2020)
       
  • Weight loss supplement causing acute heart block in a child
    • Authors: Daniel R. O’Brien; Vivian Szymczuk, Cecilia A. Albaro
      Pages: 131 - 133
      Abstract: A 16-year-old male was admitted to the paediatric ICU with acute onset of vomiting, somnolence, and chest pain, and electrocardiogram showing 2nd degree heart block after ingesting an Aleurites moluccana (Candlenut) seed as a herbal weight loss supplement. Electrocardiogram showed progressively worsening heart block with down-sloping of the ST segments, resembling digoxin toxicity. After 2 days of ICU observation, his symptoms began to improve and eventually resolved. The side effects of herbal supplements are often unknown but by analysing cases such as these, physicians can develop a better understanding of these substances to help guide management.
      PubDate: 2020-01-01T00:00:00.000Z
      DOI: 10.1017/S104795111900283X
      Issue No: Vol. 30, No. 1 (2020)
       
  • Contained rupture of right ventricular outflow tract after Rastelli-type
           operation
    • Authors: Takuji Watanabe; Kyoichi Nishigaki, Yoichi Kawahira
      Pages: 134 - 135
      Abstract: This report describes a 3-year-old infant with post-operative mediastinitis complicated by a contained rupture of the right ventricle. A contained rupture is recognised as the huge pulsating prominence of the anterior chest wall. CT confirmed blood communication between the right ventricular outflow tract and the cavity surrounded by the pectoral major musculocutaneous flap. This is a significant case in which severe adhesion between the right ventricle and the musculocutaneous flap could maintain her stable haemodynamics with a pulsating prominence.
      PubDate: 2020-01-01T00:00:00.000Z
      DOI: 10.1017/S1047951119003123
      Issue No: Vol. 30, No. 1 (2020)
       
  • Pseudoaneurysm of the aortic arch early after coarctation repair in the
           neonatal period
    • Authors: Ana R. Sousa; Ana M. Teixeira, José P. Neves
      Pages: 136 - 138
      Abstract: Aortic arch aneurysm or pseudoaneurysm is a rare complication early after coarctation repair in the neonatal period. We report the case of a newborn with a ventricular septal defect and aortic coarctation with a hypoplastic aortic arch that developed a large aortic arch pseudoaneurysm following a radically extended end-to-end coarctation repair. Successful surgical correction of the pseudoaneurysm was performed.
      PubDate: 2020-01-01T00:00:00.000Z
      DOI: 10.1017/S1047951119002841
      Issue No: Vol. 30, No. 1 (2020)
       
  • Percutaneous rescue therapy in a child with blocked cavo-pulmonary shunt
    • Authors: Aritra Mukherji; Sanjiban Ghosh, Amitabha Chattopadhyay
      Pages: 139 - 141
      Abstract: A 7-month-old infant presented with bilateral blocked cavo-pulmonary anastomosis within 2 months of surgery. Due to extreme haemodynamic instability, surgical options were abandoned and rescue intervention from left jugular line was planned. Acute thrombosis of the left-sided Glenn was noted with significant anastomotic narrowing. Successful rescue thrombolysis was done using recombinant tissue plasminogen activator (Alteplase) along with balloon dilatation of the attenuated segments.
      PubDate: 2020-01-01T00:00:00.000Z
      DOI: 10.1017/S1047951119002798
      Issue No: Vol. 30, No. 1 (2020)
       
  • Internal iliac artery thrombosis in a 2-month-old infant with incomplete
           Kawasaki disease
    • Authors: Husna Musa; Putri Yubbu, Ghee Tiong Koh
      Pages: 142 - 144
      Abstract: We report a case of a 2-month-old infant with incomplete Kawasaki disease with multiple coronary and systemic arteries aneurysms complicated with internal iliac arteries thrombosis. The atypical clinical presentations and severity of systemic vascular involvements discuss the importance of high index of suspicions in younger infants and treatment options in such cases.
      PubDate: 2020-01-01T00:00:00.000Z
      DOI: 10.1017/S1047951119002609
      Issue No: Vol. 30, No. 1 (2020)
       
  • Anomalous origin of left pulmonary artery from the ascending aorta:
           echocardiography assessment
    • Authors: Samir Atmani; Imane Bendris
      Pages: 145 - 147
      Abstract: Anomalous origin of one pulmonary artery from the ascending aorta is a rare cardiac anomaly in which the pulmonary artery abnormally arises from the ascending aorta. Physiologically, most patients develop signs of cardiac failure due to high flow to both lungs. The purpose of this study is to demonstrate, with this rare anomaly, the accurate place of the echocardiography to establish diagnosis especially in the systemic or supra-systemic pulmonary hypertension.
      PubDate: 2020-01-01T00:00:00.000Z
      DOI: 10.1017/S1047951119002804
      Issue No: Vol. 30, No. 1 (2020)
       
  • Cardiac surgery in West Africa: the tipping point
    • Authors: Dominique Vervoort; Jacques Kpodonu
      Pages: 148 - 148
      PubDate: 2020-01-01T00:00:00.000Z
      DOI: 10.1017/S104795111900310X
      Issue No: Vol. 30, No. 1 (2020)
       
  • Paths to improved rheumatic heart disease: screening and prophylaxis
    • Authors: Jonathan M. Fairley; Mahmood Ahmad
      Pages: 149 - 150
      PubDate: 2020-01-01T00:00:00.000Z
      DOI: 10.1017/S1047951119002774
      Issue No: Vol. 30, No. 1 (2020)
       
  • Echocardiographic screening for rheumatic heart disease in Turkish school
           children
    • Authors: Semra Atalay; Ercan Tutar, Tayfun Uçar, Seda Topçu, Serdal K. Köse, Melih T. Doğan
      Pages: 151 - 151
      PubDate: 2020-01-01T00:00:00.000Z
      DOI: 10.1017/S1047951119002981
      Issue No: Vol. 30, No. 1 (2020)
       
 
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