Heart 2000;84:476-482 ( November )

Cardiovascular medicine

Hypertrophic cardiomyopathy: the interrelation of disarray, fibrosis, and small vessel disease A M Varnava^a, P M Elliott^b, S Sharma^b, W J McKenna^b, M J Davies^a

^a Department of Cardiovascular Pathology, St George's Hospital Medical School, Cranmer Terrace, London SW17 0RE, UK, ^b Department of Cardiological Sciences, St George's Hospital Medical School

Correspondence to: Dr Varnava email: avarnava{at}sghms.ac.uk

Accepted 12 July 2000

OBJECTIVETo make a quantitative assessment of the relation between disarray, fibrosis, and small vessel disease in hypertrophic cardiomyopathy.
DESIGNDetailed macroscopic and histological examination at 19 segments of the left and right ventricle and the left atrial free wall.
PATIENTS72 patients with hypertrophic cardiomyopathy who had suffered sudden death or progression to end stage cardiac failure (resulting in death or heart transplantation).
MAIN OUTCOME MEASURESThe presence of scarring, atrial dilatation, and a mitral valve impact lesion were noted, and heart weight, wall thickness, per cent disarray, per cent fibrosis, and per cent small vessel disease quantitated for each heart.
RESULTSWithin an individual heart the magnitude of hypertrophy correlated with the severity of fibrosis (p = 0.006) and disarray (p = 0.0002). Overall, however, total heart weight related weakly but significantly to fibrosis (r = 0.4, p = 0.0001) and small vessel disease (r = 0.3, p = 0.03), but not to disarray. Disarray was greater in hearts with mild left ventricular hypertrophy (maximum wall thickness < 20 mm) and preserved systolic function (60.9 (26)% v 43 (20.4)% respectively, p = 0.02) and hearts without a mitral valve impact lesion (26.3% v 18.9%, p = 0.04), but was uninfluenced by sex. Fibrosis was influenced by sex (7% in male patients and 4% in female, p = 0.04), but not by the presence of an impact lesion. No relation was found between disarray, fibrosis, and small vessel disease.
CONCLUSIONSMyocyte disarray is probably a direct response to functional or structural abnormalities of the mutated sarcomeric protein, while fibrosis and small vessel disease are secondary phenomena unrelated to disarray, but modified by factors such as left ventricular mass, sex, and perhaps local autocrine factors.


Keywords: hypertrophic cardiomyopathy; histopathology; small vessel disease

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© 2000 by Heart
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