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Research Topic : Congenital Heart Disease
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    Central Aortic Blood Pressure In Children: Establishing A Gold Standard Non-invasive Assessment Of Cardiovascular Risk

    Funder
    National Health and Medical Research Council
    Funding Amount
    $694,342.00
    Summary
    The best way of assessing early risk of cardiovascular disease involves measuring blood pressure near the heart (central pressure), but existing devices used in adults for this purpose are inaccurate in children. We will develop a children-specific method and apply it to study early cardiovascular risk in a comprehensive health study of 2000 children Australia-wide. We will also investigate why children with congenital heart disease frequently develop ‘older-adult’ heart disease at a young age.
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    Funded Activity

    Improving Functional Outcomes After Fontan Surgery By A Cross-sectional Study Of The Outcomes Following Variation In Practice In Australia And New Zealand: Focus On Anticoagulation And Cardiac Shunting By The Fenestration

    Funder
    National Health and Medical Research Council
    Funding Amount
    $768,643.00
    Summary
    The Fontan procedure is the last of a series of life-saving operations offered to children born with only one pumping chamber in their heart. We intend to perform the largest and most detailed investigation to date of the patients enrolled in the Australia and New Zealand Fontan Registry, today the world's largest database of this kind. This cross-sectional study will enable us to identify the drugs and interventions that will best maximize their exercise capacity and quality of life
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    Funded Activity

    The ZIC3 Heterotaxy-associated Transcription Factor: A New Player In Nuclear Control Of Canonical Wnt Signalling

    Funder
    National Health and Medical Research Council
    Funding Amount
    $992,822.00
    Summary
    Humans have many internal asymmetries that need to occur in a consistent manner across all individuals. Examples of asymmetry include our unpaired organs (like the heart or liver) or a paired organ with asymmetry (like the lungs). In this project we will use cutting edge molecular embryology and cell biology techniques to explore the mechanisms behind the remarkable feat of establishing asymmetry so we are better able to help those individuals with laterality disorders.
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    Funded Activity

    Critical Illness In Children: Can We Afford To Neglect The Psychosocial Risks? The Impact, Acceptability, And Cost-effectiveness Of Routine Psychosocial Assessment And Stepped Care For Families Of Infants With Heart Disease

    Funder
    National Health and Medical Research Council
    Funding Amount
    $975,116.00
    Summary
    There is overwhelming evidence that children with heart disease (CHD) and their families suffer high levels of psychological stress, with consequent heightened suffering, impaired cooperation with treatment, and high financial costs. This research will produce a system-wide increase in the proportion of families of babies with CHD who have access to psychosocial care, and will reduce the proportion of parents who experience depression and anxiety. Economic impacts of CHD will also be determined.
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    Funded Activity

    The Role Of Micro-RNAs In Human Cardiomyocyte Specification

    Funder
    National Health and Medical Research Council
    Funding Amount
    $345,534.00
    Summary
    The heart is the first organ to form and is vital for the survival of the developing embryo. We are seeking to improve our knowledge of the process of heart formation. Understanding how primitive cells become cardiac cells may pave the way for production of “tailor made” cardiac cells for treatment of a weakened heart. It may also give insights to the causes of congenital heart defects (such as “hole in the heart” babies), which are the most common type of birth defects.
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    Funded Activity

    Detection Of Liver And Renal Function Abnormalities In The Australian & New Zealand Population Of Fontan Patients

    Funder
    National Health and Medical Research Council
    Funding Amount
    $345,080.00
    Summary
    Children born with complex heart defects and only one pumping chamber can now live into adulthood with an operation called the Fontan procedure. As this operation has only existed for 40 years, the long-term expectations for these children and young adults are still unclear, and their population is growing every year. There is now evidence that they may suffer from liver and kidney failure. This project will identify the severity of liver and kidney damage in our population of Fontan patients.
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    Funded Activity

    Novel Roles For Neural Crest Cells In Cardiac Morphogenesis

    Funder
    National Health and Medical Research Council
    Funding Amount
    $553,848.00
    Summary
    Abnormal formation of the cardiac outflow tract leads to common malformations affecting over 1% of all births. Taking advantage of novel mouse models this grant aims to identify the molecular mechanisms by which neural crest cells control formation of the cardiac outflow tract. New information generated from this study stands to identify new targets which may be used for predictive testing and regenerative therapies.
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    Funded Activity

    Approaches To Therapy For The Skeletal Muscle Actin Diseases

    Funder
    National Health and Medical Research Council
    Funding Amount
    $912,078.00
    Summary
    We have shown that errors in a crucial muscle protein called actin cause muscle diseases that affect newborn children. These diseases are mainly very severe, causing death within the first year of life. Currently there is no cure. This project will investigate possible therapies for these diseases, such as viral delivery of a normal version of actin and finding a drug to overcome the weakness. Successful outcomes will crucially bring treatment closer for the patients.
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    Funded Activity

    NDI1 Therapy For NADH-Ubiquinone Oxidoreductase Deficiency

    Funder
    National Health and Medical Research Council
    Funding Amount
    $575,762.00
    Summary
    This study will test a new protein therapy that can act as a surrogate for a deficient or defective enzyme called Mitochondrial Complex 1. The deficiency occurs in newborns with defective genes for the proteins that form the enzyme. The defect causes metabolic malfunction in most organs, with patients needing specialist hospital and parental care, but there is no cure yet. We have successfully tested this in the lab but will now test this in our new animal model of the disease.
    More information
    Funded Activity

    Regulation Of Heart Development And Regeneration By DNA Methylation.

    Funder
    National Health and Medical Research Council
    Funding Amount
    $552,709.00
    Summary
    The adult mammalian heart has an extremely limited capacity for regeneration following a heart attack, which is in stark contrast to the robust regenerative capacity of the newborn heart. How and why mammals lose their ability to regenerate heart tissue after birth is not well understood. We propose a new approach to unravel the complex mechanisms that control gene expression during heart development in rodents and humans, which could provide new therapeutic avenues for heart regeneration.
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