Open AccessCardiomyopathy in Dystrophin‐Deficient Hypertrophic Feline Muscular Dystrophy
Author(s) -
Gaschen Lorrie,
Lang Johann,
Lin Shuo,
AdéDamilano Manuele,
Busato André,
Lombard Christophe W.,
Gaschen Frédéric P.
Publication year - 1999
Publication title -
journal of veterinary internal medicine
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.356
H-Index - 103
eISSN - 1939-1676
pISSN - 0891-6640
DOI - 10.1111/j.1939-1676.1999.tb02193.x
Subject(s) - medicine , cats , interventricular septum , hypertrophic cardiomyopathy , dystrophin , muscular dystrophy , papillary muscle , endocardium , cardiomyopathy , cardiology , muscle hypertrophy , duchenne muscular dystrophy , diastole , anatomy , heart failure , ventricle , blood pressure
A colony of cats affected with hypertrophic feline muscular dystrophy was used to study the occurrence of cardiomyopathy associated with dystrophin deficiency. Affected male and female cats, obligate carrier females, and unaffected healthy littermates were followed from 12 weeks of age into adulthood. Thoracic radiography, 2‐D echocardiography, and 2‐D–derived M‐mode echocardiography were performed at 3‐month intervals until 12 months of age and regularly thereafter. From 9 months of age, all affected cats had larger hearts than normal and carrier animals. Left ventricular wall thickness in systole and in diastole and interventricular septal thickness in systole were greater in affected cats 12 months and older when compared with normal or heterozygous animals ( P < .05). The myocardium of affected cats was diffusely hypoechoic and thickened. Multiple hyperechoic foci were in the myocardium and papillary musculature. Shortening fraction was normal in all cats. Changes seen in carrier females included enlargement and hyperechogenicity of the papillary musculature after the age of 2 years. Gross and light microscopic examination revealed left ventricular wall thickening with multiple foci of mineralization in 2 of 5 hearts from dystrophin‐deficient cats. Although approximately 10% of the normal dystrophin amount was present in the skeletal muscle, dystrophin could not be detected in the myocardium. Early onset concentric myocardial hypertrophy was present in all adult cats. Lesions were mainly localized in the myocardium of the left ventricular free wall and interventricular septum, papillary musculature, and the endocardium. Clinical signs of heart failure developed only infrequently in cats with hypertrophic feline muscular dystrophy.