title: Age-matched comparison reveals early electrocardiography and echocardiography changes in dystrophin-deficient dogs.
abstract: The absence of dystrophin in the heart leads to Duchenne cardiomyopathy. Dystrophin-deficient dogs represent a critical model to translate novel therapies developed in mice to humans. Unfortunately, little is known about cardiophysiology changes in these dogs. We performed prospective electrocardiographic and echocardiographic examinations at 3, 6 and 12 months of age in four normal and three affected dogs obtained from the same litter. Affected dogs showed growth retardation and serum creatine kinase elevation. Necropsy confirmed cardiac dystrophin deficiency and histopathology. Q/R ratio elevation and diastolic left ventricular (LV) internal diameter reduction were the most consistent findings in affected dogs at all ages. At 6 and 12 months, dystrophic dogs also showed significant reduction of PR intervals, LV end diastolic/systolic volumes and systolic LV internal diameters. Epicardial and endocardial slope times were significantly reduced in affected dogs at 12 months. These results establish the baseline for evaluating experimental therapies in the future.
authors.affiliation: Department of Veterinary Medicine and Surgery, College of Veterinary Medicine, The University of Missouri, Columbia, MO, USA.
authors.forename: Deborah M
authors.initials: DM
authors.lastname: Fine
authors.forename: Jin-Hong
authors.initials: JH
authors.lastname: Shin
authors.forename: Yongping
authors.initials: Y
authors.lastname: Yue
authors.forename: Dietrich
authors.initials: D
authors.lastname: Volkmann
authors.forename: Stacey B
authors.initials: SB
authors.lastname: Leach
authors.forename: Bruce F
authors.initials: BF
authors.lastname: Smith
authors.forename: Mark
authors.initials: M
authors.lastname: McIntosh
authors.forename: Dongsheng
authors.initials: D
authors.lastname: Duan
mesh_terms: D000818:Animals; D009202:Cardiomyopathies / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000473:pathology; D003402:Creatine Kinase / Q000097:blood; D004195:Disease Models, Animal; D004285:Dogs; D016189:Dystrophin / Q000172:deficiency*; D004562:Electrocardiography; D006321:Heart / Q000503:physiopathology; D020388:Muscular Dystrophy, Duchenne / Q000473:pathology / Q000503:physiopathology; D009206:Myocardium / Q000473:pathology; D014463:Ultrasonography
chemical_list: D016189:Dystrophin; D003402:Creatine Kinase