Abstract
BackgroundIn recent years, several new technologies for small-animal imaging have been developed. In particular, the use of ultrasound in animal imaging has focused on the investigation of accessible biological structures such as the heart, of which it provides a morphological and functional assessment. The purpose of this study was to investigate the role of micro-ultrasonography (μ-US) in a longitudinal study on BIO14.6 cardiomyopathic hamsters treated with gene therapy.MethodsThirty hamsters were divided into three groups (n = 10): Group I, untreated BIO 14.6 hamsters; Group II, BIO 14.6 hamsters treated with gene therapy; Group III, untreated wild type (WT) hamsters. All hamsters underwent serial μ-US sessions and were sacrificed at predetermined time points.Resultsμ-US revealed: in Group I, progressive dilation of the left ventricle with a change in heart morphology from an elliptical to a more spherical shape, altered configuration of the mitral valve and subvalvular apparatus, and severe reduction in ejection fraction; in Group II, mild decrease in contractile function and ejection fraction; in Group III, normal cardiac chamber morphology and function. There was a negative correlation between the percentage of fibrosis observed at histology and the ejection fraction obtained on μ-echocardiography (Spearman r: -0.839; p < 0.001).ConclusionsAlthough histological examination remains indispensable for a conclusive diagnosis, high-frequency μ-echocardiography, thanks to the high spatial and contrast resolution, can be considered sufficient for monitoring therapeutic efficacy and/or the progression of dilated cardiomyopathy, providing an alternative tool for repeatable and noninvasive evaluation.
Highlights
In recent years, several new technologies for small-animal imaging have been developed including microradiography (μ-XR), micro-computed tomography, micro-magnetic resonance imaging (μ-MRI), micro-positron emission tomography (μ-PET) and micro-ultrasonography (μ-US)
This study aimed to define the role of μ-US in the longitudinal study of BIO 14.6 hamsters with dilated cardiomyopathy treated with gene therapy
- Group I: untreated BIO 14.6 hamsters; - Group II: BIO 14.6 hamsters which received an intraperitoneal injection of AAV2/8-CMV-hSCGD (Recombinant associated viruses (AAV) vector containing human delta-sarcoglycan cDNA, driven by the cytomegalovirus promoter packaged into AAV2 serotype transcapsidated with AAV8 serotype) after 2 weeks of age and an intrajugular injection of AAV2/1(AAV2 serotype transcapsidated with AAV1 serotype)-CMV-hSCGD after 5 months of age
Summary
Several new technologies for small-animal imaging have been developed including microradiography (μ-XR), micro-computed tomography (μCT), micro-magnetic resonance imaging (μ-MRI), micro-positron emission tomography (μ-PET) and micro-ultrasonography (μ-US). Muscular dystrophies constitute a heterogeneous group of degenerative diseases characterized by a progressive wasting and weakening of skeletal muscle, of varying severity and distribution [4] The ethiopathogenesis of these disorders is ascribed to mutations in genes coding for the proteins forming the dystrophin-associated protein complex (DAPC) mutations, which cause loss of integrity of the sarcolemma rendering the muscle fibers more prone to injury [5]. The BIO 14.6 hamster faithfully reproduces the LGMD2F phenotype seen in humans In this animal model, the absence of delta-SG from the membrane of skeletal and cardiac muscle results in a secondary deficiency of alpha-, beta-, and gamma-SG. The purpose of this study was to investigate the role of micro-ultrasonography (μ-US) in a longitudinal study on BIO14.6 cardiomyopathic hamsters treated with gene therapy
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