Familial hypertrophic cardiomyopathy in maine coon cats

An animal model of human disease

Mark D. Kittleson, Kathryn M. Meurs, Marcia J. Munro, Judith A. Kittleson, Si Kwang Liu, Paul D. Pion, Jeffrey Towbin

Research output: Contribution to journalArticle

164 Citations (Scopus)

Abstract

Background - A naturally occurring animal model of familial hypertrophic cardiomyopathy (FHCM) is lacking. We identified a family of Maine coon cats with HCM and developed a colony to determine mode of inheritance, phenotypic expression, and natural history of the disease. Methods and Results - A proband was identified, and related cats were bred to produce a colony. Affected and unaffected cats were bred to determine the mode of inheritance. Echocardiography was used to identify affected offspring and determine phenotypic expression. Echocardiograms were repeated serially to determine the natural history of the disease. Of 22 offspring from breeding affected to unaffected cats, 12 (55%) were affected. When affected cats were bred to affected cats, 4 (45%) of the 9 were affected, 2 (22%) unaffected, and 3 (33%) stillborn. Findings were consistent with an autosomal dominant mode of inheritance with 100% penetrance, with the stillborns representing lethal homozygotes that died in utero. Affected cats usually did not have phenotypic evidence of HCM before 6 months of age, developed HCM during adolescence, and developed severe HCM during young adulthood. Papillary muscle hypertrophy that produced midcavitary obstruction and systolic anterior motion of the mitral valve was the most consistent manifestation of HCM. Cats died suddenly (n=5) or of heart failure (n=3). Histopathology of the myocardium revealed myocardial fiber disarray, intramural coronary arteriosclerosis, and interstitial fibrosis. Conclusions - HCM in this family of Maine coon cats closely resembles the human form of FHCM and should prove a valuable tool for studying the gross, cellular, and molecular pathophysiology of the disease.

Original languageEnglish (US)
Pages (from-to)3172-3180
Number of pages9
JournalCirculation
Volume99
Issue number24
DOIs
StatePublished - Jun 22 1999
Externally publishedYes

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Familial Hypertrophic Cardiomyopathy
Animal Disease Models
Cats
Papillary Muscles
Penetrance
Homozygote
Mitral Valve
Hypertrophy
Breeding
Echocardiography
Coronary Artery Disease
Myocardium
Fibrosis
Animal Models
Heart Failure

All Science Journal Classification (ASJC) codes

  • Cardiology and Cardiovascular Medicine
  • Physiology (medical)

Cite this

Kittleson, M. D., Meurs, K. M., Munro, M. J., Kittleson, J. A., Liu, S. K., Pion, P. D., & Towbin, J. (1999). Familial hypertrophic cardiomyopathy in maine coon cats: An animal model of human disease. Circulation, 99(24), 3172-3180. https://doi.org/10.1161/01.CIR.99.24.3172

Familial hypertrophic cardiomyopathy in maine coon cats : An animal model of human disease. / Kittleson, Mark D.; Meurs, Kathryn M.; Munro, Marcia J.; Kittleson, Judith A.; Liu, Si Kwang; Pion, Paul D.; Towbin, Jeffrey.

In: Circulation, Vol. 99, No. 24, 22.06.1999, p. 3172-3180.

Research output: Contribution to journalArticle

Kittleson, MD, Meurs, KM, Munro, MJ, Kittleson, JA, Liu, SK, Pion, PD & Towbin, J 1999, 'Familial hypertrophic cardiomyopathy in maine coon cats: An animal model of human disease', Circulation, vol. 99, no. 24, pp. 3172-3180. https://doi.org/10.1161/01.CIR.99.24.3172
Kittleson MD, Meurs KM, Munro MJ, Kittleson JA, Liu SK, Pion PD et al. Familial hypertrophic cardiomyopathy in maine coon cats: An animal model of human disease. Circulation. 1999 Jun 22;99(24):3172-3180. https://doi.org/10.1161/01.CIR.99.24.3172
Kittleson, Mark D. ; Meurs, Kathryn M. ; Munro, Marcia J. ; Kittleson, Judith A. ; Liu, Si Kwang ; Pion, Paul D. ; Towbin, Jeffrey. / Familial hypertrophic cardiomyopathy in maine coon cats : An animal model of human disease. In: Circulation. 1999 ; Vol. 99, No. 24. pp. 3172-3180.
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