Cited by 89Cardiomyopathy and Myosin StudiesMuscle Physiology and DisordersCardiac electrophysiology and arrhythmias

Duchenne's cardiomyopathy in a canine model: electrocardiographic and echocardiographic studies

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Moise, N. Sydney, Valentine, Beth A., Brown, Cynthia A., Erb, Hollis N., Beck, Kathy A., Cooper, Barry J., Gilmour, Robert F.·1991·Journal of the American College of Cardiology

Abstract

Thirteen dogs affected with X-linked Duchenne's muscular dystrophy and 11 female carrier dogs were studied by electrocardiography (ECG) and echocardiography. Twelve of the affected dogs were studied as immature animals and followed at 1 to 6 month intervals until they were 7 to 46 months of age. Compared with control dogs, affected dogs had significantly increased (p less than 0.02) Q/R ratios in ECG leads II, III, aVF, CV6LL (V2) and CV6LU (V4). Carrier dogs had significantly increased (p less than 0.02) Q/R ratios in leads V2 and V4. The Q/R ratio increased in three of six dogs followed up from age 6 months to greater than 2 years. The PR intervals were significantly shorter (p less than 0.02) in affected dogs. Ventricular arrhythmias were identified in four of six mature affected dogs. Two-dimensional echocardiography revealed distinctive hyperechoic lesions in 12 of the 13 affected dogs and in 6 of the 11 carrier dogs. Hyperechoic lesions corresponded to calcified myocardium and surrounding dense connective tissue. This study establishes the dog affected with Duchenne's muscular dystrophy as an animal model of Duchenne's cardiomyopathy and demonstrates that the heart in carrier dogs is affected by the dystrophic process.

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  • The Golden Retriever Muscular Dystrophy line develops cardiomyopathy closely resembling affected humans and is considered to better represent cardiac involvement in human DMD than mouse mdx counterparts.

    “One of these lines is the Golden Retriever Muscular Dystrophy (Cooper, et al., 1988) which develops cardiomyopathy with features that closely resemble those of affected humans (Moise, et al., 1991; Valentine, Cooper, de Lahunta, O'Quinn, & Blue, 1988; Valentine, Cummings, & Cooper, 1989) and is considered to better represent cardiac involvement in human DMD than their mouse mdx counterparts (Nakam”

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Research works printed but not linked: 5

  1. DUCHENNE'S CARDIOMYOPATHY 33. Emery AEH. Abnormalities of the electrocardiogram in heredity myop- athies. J Med Genet 1972;9:8-12.
  2. Tanaka H, Nishi S, Katanasko H. Natural course of cardiomyopathy in Duchenne muscular dystrophy. Jpn Circ J 1979;43:974-84.
  3. Vincent GM, Davis D, Ziter F. Echocardiography in Duchenne dystrophy (abstr). Circulation 1975(suppli);52:11-253.
  4. Morrison SA, Moise NS, Scarlett J, Mohammed H, Yeager AE. Echo- cardiographic evaluation of clinically-normal dogs from 4 breeds of different sizes and somatotypes. J Vet Intern Med, in press.
  5. Mann 0, DeLeon AC, Perloff JK, Simanis J, Harrigan FD. Duchenne muscular dystrophy: the electrocardiogram in female relatives. Am J Med Sci 1968;255:376-81.

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Authors
Moise, N. S., Valentine, B. A., Brown, C. A., Erb, H. N., Beck, K. A., Cooper, B. J., & Gilmour, R. F.
Venue
Journal of the American College of Cardiology
Publisher
Elsevier BV
Published
1991-03
Volume
17
Issue
3
Pages
812-820
ISSN
0735-1097 (ISSN-L)
OpenAlex
W2039230574
Cited by
89
References
64
Citations by year
2026: 1 · 2025: 1 · 2024: 2 · 2023: 1 · 2022: 2 · 2021: 3 · 2020: 3 · 2019: 3 · 2018: 4 · 2017: 4 · 2016: 3 · 2015: 9 · 2014: 3 · 2013: 1 · 2012: 3
FWCI
3.15
Type
journal article
Language
en
Open access
Locations
  • Journal of the American College of Cardiology (publishedVersion)
  • PubMed (publishedVersion)
Topics
  • Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Cardiomyopathy and Myosin Studies
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Muscle Physiology and Disorders
  • Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Cardiac electrophysiology and arrhythmias
Keywords
Duchenne muscular dystrophy, Duchenne cardiomyopathy, canine model, X-linked muscular dystrophy, carrier dogs, electrocardiographic changes, PR interval, ventricular arrhythmias, myocardial calcification, hyperechoic lesions, echocardiographic findings, animal models
MeSH terms
Animals, Dogs, Echocardiography, Electrocardiography, Female, Heart Diseases, Cardiomegaly, Male, Muscular Dystrophy, Animal, Radiography

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