Circulation, Vol 85, 1651-1660, Copyright © 1992 by American Heart Association
HG Klues, BJ Maron, AL Dollar and WC Roberts
BACKGROUND. Hypertrophic cardiomyopathy (HCM) is characterized by an
asymmetrically hypertrophied left ventricle and is regarded as a disease of
cardiac muscle. METHODS AND RESULTS. To assess the possibility that the
mitral valve itself may be involved in the disease process, we studied
mitral valves from 94 patients with HCM and 45 normal control subjects. The
area of the mitral leaflets was increased in patients with HCM compared
with control subjects (12.9 +/- 3.7 versus 8.7 +/- 2.0 cm2; p less than
0.001). For the overall group of patients, this increase was largely caused
by an increase in anterior leaflet length (2.2 +/- 0.5 cm for HCM versus
1.8 +/- 0.3 cm for control subjects; p less than 0.001), because
circumference did not differ between the two groups. Mitral leaflet area
was increased (greater than or equal to 12.0 cm2) in 55 (58%) of the 94
valves. In 12 of these 55 valves, both the anterior and posterior leaflets
were enlarged; the other 43 valves had asymmetrical or segmental
enlargement of either the anterior leaflet (36 patients) or a portion of
posterior leaflet (seven patients). In addition, nine patients had a
congenital malformation of the mitral apparatus in which one or both
papillary muscles inserted directly into anterior mitral leaflet (mitral
valve area was normal in seven of the nine). CONCLUSIONS. Sixty-two (66%)
of 94 mitral valves had a constellation of structural malformations,
including increased leaflet area and elongation of the leaflets or
anomalous papillary muscle insertion directly into anterior mitral leaflet.
These findings expand the morphological definition of HCM by demonstrating
that the disease process is not confined to cardiac muscle but rather many
patients also have structural abnormalities of the mitral valve that are
unlikely to be acquired or secondary to mechanical factors.
ARTICLES
Diversity of structural mitral valve alterations in hypertrophic cardiomyopathy
Pathology Branches, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, Md 20892.
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