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Natural History of Cardiomyopathy in Adult Dogs With Golden Retriever Muscular Dystrophy

dc.contributor.authorGuo, Lee-Jae
dc.contributor.authorSoslow, Jonathan H.
dc.contributor.authorBettis, Amanda K.
dc.contributor.authorNghiem, Peter P.
dc.contributor.authorCummings, Kevin J.
dc.contributor.authorLenox, Mark W.
dc.contributor.authorMiller, Matthew W.
dc.contributor.authorKornegay, Joe N.
dc.contributor.authorSpurney, Christopher F.
dc.identifier.citationGuo, L. J., Soslow, J. H., Bettis, A. K., Nghiem, P. P., Cummings, K. J., Lenox, M. W., Miller, M. W., Kornegay, J. N., & Spurney, C. F. (2019). Natural History of Cardiomyopathy in Adult Dogs With Golden Retriever Muscular Dystrophy. Journal of the American Heart Association, 8(16), e012443.
dc.description.abstractBackground-Duchenne muscular dystrophy (DMD) is an X-linked disease that causes progressive muscle weakness. Affected boys typically die from respiratory or cardiac failure. Golden retriever muscular dystrophy (GRMD) is genetically homologous with DMD and causes analogous skeletal and cardiac muscle disease. Previous studies have detailed features of GRMD cardiomyopathy in mostly young dogs. Cardiac disease is not well characterized in adult GRMD dogs, and cardiac magnetic resonance (CMR) imaging studies have not been completed. Methods and Results-We evaluated echocardiography and CMR in 24 adult GRMD dogs at different ages. Left ventricular systolic and diastolic functions, wall thickness, and myocardial strain were assessed with echocardiography. Features evaluated with CMR included left ventricular function, chamber size, myocardial mass, and late gadolinium enhancement. Our results largely paralleled those of DMD cardiomyopathy. Ejection fraction and fractional shortening correlated well with age, with systolic dysfunction occurring at approximate to 30 to 45 months. Circumferential strain was more sensitive than ejection fraction in early disease detection. Evidence of left ventricular chamber dilatation provided proof of dilated cardiomyopathy. Late gadolinium enhancement imaging showed DMD-like left ventricular lateral wall lesions and earlier involvement of the anterior septum. Multiple functional indexes were graded objectively and added, with and without late gadolinium enhancement, to give cardiac and cardiomyopathy scores of disease severity. Consistent with DMD, there was parallel skeletal muscle involvement, as tibiotarsal joint flexion torque declined in tandem with cardiac function. Conclusions-This study established parallels of progressive cardiomyopathy between dystrophic dogs and boys, further validating GRMD as a model of DMD cardiac disease.en_US
dc.publisherJournal of the American Heart Associationen_US
dc.rightsCopyright © 2019 The Authors. Published on behalf of the American Heart Association, Inc., by Wiley. This is an open access article under the terms of the License, which permits use and distribution in any medium, provided the original work is properly cited, the use is non‐commercial and no modifications or adaptations are made.
dc.subjectcardiac imagingen_US
dc.subjectDuchenne muscular dystrophyen_US
dc.subjectgolden retriever muscular dystrophyen_US
dc.subjectnatural historyen_US
dc.titleNatural History of Cardiomyopathy in Adult Dogs With Golden Retriever Muscular Dystrophyen_US

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