Malalignment of the sarcomeric filaments in hypertrophic cardiomyopathy with cardiac myosin heavy chain gene mutation
A Muraishia, H Kaib, K Adachib, H Nishia, T Imaizumia
a The Third Department
of Internal Medicine, Kurume University School of Medicine, 67 Asahi-machi, Kurume, Fukuoka 830-0011, Japan, b Cardiovascular Research Institute, Kurume
University, 67 Asahi-machi, Kurume, Fukuoka 830-0011, Japan
Correspondence to: Dr Kai. email: naikai{at}med.kurume-u.ac.jp
Accepted for publication 2 June 1999
OBJECTIVE
To
investigate changes in the alignment of the sarcomeric filaments in
hypertrophic cardiomyopathy and the effects of cardiac 
myosin heavy
chain (-MHC) mutation on the sarcomeric ultrastructure.
DESIGNA retrospective analysis.
PATIENTSEndomyocardial
biopsy samples were examined by transmission electron microscopy in
seven patients with hypertrophic cardiomyopathy and -MHC
mutation, six with hypertrophic cardiomyopathy but without the
mutation, and five controls (with chest pain syndromes).
MAIN OUTCOME
MEASUREAlignment of the sarcomeric filaments and
the distance between neighbouring thick myosin filaments.
RESULTSIn controls,
cross sections of the sarcomere at the A band showed a highly organised
orthohexagonal array with 6 thin actin filaments surrounding one thick
myosin filament, whereas in hypertrophic cardiomyopathy the alignment
of the sarcomeric filaments was sparse and disrupted. In hypertrophic
cardiomyopathy with a mutation, the distance between neighbouring thick
myosin filaments was greater than in controls (mean (SD) 45.3 (4.7)
v 38.5 (3.5) nm, p < 0.05), and the
variance of the distance was greater than in controls (8.0 (0.7)
v 4.8 (1.0) nm, p < 0.001) or in patients
with hypertrophic cardiomyopathy without a mutation (6.7 (0.6) nm,
p < 0.05). In the latter, the variance of the distance was also
greater than in the controls (p < 0.01). A significant correlation
was found between the grade of the myocyte hypertrophy and the variance of the distance (r = 0.654; p < 0.01).
CONCLUSIONSThe
alignment of the sarcomeric filaments is disrupted in hypertrophic
cardiomyopathy, particularly when there is -MHC mutation.
Keywords: hypertrophic cardiomyopathy;
myosin heavy chain;
myosin filament;
sarcomere
© 1999 by Heart
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