Pathology of end-stage remodeling in a family of cats with hypertrophic cardiomyopathy.
End-stage hypertrophic cardiomyopathy (ES-HCM), affecting 5-10% of human hypertrophic cardiomyopathy (HCM) patients, is characterized by relative thinning of the ventricular walls and septum with dilation of the ventricular lumen, decreased fractional shortening, and progression to heart failure. C. J. Baty and others recently documented similar progressive changes to ES-HCM in a family of four cats through serial echocardiograms. At the time of heart failure, these cats exhibited changes similar to those exhibited by human ES-HCM patients. Our objectives were to describe the pathologic alterations associated with ES-HCM and investigate the pathogenesis in three of the four cats. Grossly, there was left atrial dilation with relative thinning of the interventricular septum (IVS) and left ventricular free wall (LVFW). The left atrium contained large thrombi in two of the three cats, and all three cats died following thromboembolization of the aortic bifurcation. Histologically, all three cats had subendocardial and myocardial fibrosis, predominantly of the IVS and LVFW, and one cat had acute, multifocal, myocardial infarcts with mononuclear inflammatory cell infiltrates. The pathogenesis of ES-HCM is uncertain, but theories implicate occlusion of the coronary blood flow by thickening of the coronary vessels, coronary vascular thromboembolism or coronary vessel spasm, apoptosis of myocytes, and myocardial hypertrophy beyond the ability of the vasculature to supply blood. Apoptosis assays did not reveal any apoptotic myocytes. Considering the hypercoagulative state of these cats, coronary vascular thromboembolism could be a major contributing factor. We cannot exclude apoptosis or coronary vessel spasm on the basis of the data presented.
Cesta
M F
MF
Laboratory of Experimental Pathology, National Institute of Environmental Sciences, Research Triangle Park, NC 27709, USA.
Baty
C J
CJ
Keene
B W
BW
Smoak
I W
IW
Malarkey
D E
DE
D000818:Animals; D002312:Cardiomyopathy, Hypertrophic / Q000473:pathology / Q000662:veterinary*; D002371:Cat Diseases / Q000473:pathology*; D002415:Cats; D017809:Fatal Outcome; D005260:Female; D006652:Histological Techniques / Q000662:veterinary; D020287:In Situ Nick-End Labeling / Q000662:veterinary; D008297:Male; D009206:Myocardium / Q000473:pathology*; D020257:Ventricular Remodeling / Q000502:physiology*