mortality/aging
• reduced viability at 10 months of age
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homeostasis/metabolism
• left atrial thrombi
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cardiovascular system
N |
• mitral valve flow analysis did not detect defects in valve function
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• at 10 months of age, ultrastructural disorganization is progressed in the ventricles with increased myofibril loss, mitochondrial damage, and accumulation of lipid droplets
• however, myofibrillar loss is not detected
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• damaged at 10 months of age
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• alterations in cardiac venous pole remodeling
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• swollen mitochondria with abnormal cristae are seen in neonatal heart ventricles
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• at E10.5 cardiomyocytes are disorganized; at E14.5, cardiomyocytes are mostly distorted and show sarcomeric disalignment
• swollen mitochondria with abnormal cristae and rare lipid droplets in the cytoplasm are present
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• congenital sinus venosus defect
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• at E10.5 expression analysis indicates that the hearts are in a more immature topological arrangement that strongly affects venous pole remodelling
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• malpositioning of venous inlets,which, however drain in the correct atrial chamber
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• microcanalizations create a fine network of ectopic connections between the pulmonary vein and systemic veins that partially drain into the vestibular region through small fenestrations
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• at E10.5 the atrial chambers appear more compressed along the cranio-caudal and dorso-ventral axes, are laterally displaced, and present a reduced rotational angle
• at E9.5 the sino-atria region is more caudally oriented
• at mid-fetal stages the sino-atrial region is misshapen with malpositioning of venous inlets
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• severely dilated at 10 months of age
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• misshapen fossa ovalis at E13.5
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• at 10 months of age
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• at E10.5 the ventricular chambers appear more compressed along the cranio-caudal and dorso-ventral axes, are laterally displaced, and present a reduced rotational angle
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• moderate ventricular but not atrial fibrosis
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• by 10 months of age, diastolic dysfunction progresses to diastolic heart failure
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• transmitralic flow analysis analysis of the heart found increased E/A ratio and reduced DT values at 10 months of age
• pulmonary vein flow analysis showed a small late diastolic wave indicating additional blood inflow into the left atrium at 2 but not 10 months of age
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• neonatal myofibrils display a stiffer, more adult phenotype
• increased maximally calcium activated isometric force value in neonatal myofibrils
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• impaired force relaxation in adult myofibrils
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• increase myocardial performance index at 10 months of age indicating progression of diastolic dysfunction
• at 2 months the a wave is increased indicating a more effective atrial contraction and the s and d wave values are higher indicating ventricular systolic compensation
• at 2 months of age the s/d ratio is less than 1
• at 10 months of age a value,s value, s/d, and a/d ratios are all altered indicating less effective atrial contraction and altered hemodynamics
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cellular
• damaged at 10 months of age
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• swollen mitochondria with abnormal cristae are seen in neonatal heart ventricles
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muscle
• at 10 months of age, ultrastructural disorganization is progressed in the ventricles with increased myofibril loss, mitochondrial damage, and accumulation of lipid droplets
• however, myofibrillar loss is not detected
|
• damaged at 10 months of age
|
• swollen mitochondria with abnormal cristae are seen in neonatal heart ventricles
|
• neonatal myofibrils display a stiffer, more adult phenotype
• increased maximally calcium activated isometric force value in neonatal myofibrils
|
• impaired force relaxation in adult myofibrils
|