Circulation, Vol 87, 1570-1579, Copyright © 1993 by American Heart Association
HG Klues, WC Roberts and BJ Maron
BACKGROUND. The morphological determinants of mitral valve systolic
anterior motion (SAM) and obstruction to left ventricular outflow in
patients within the broad clinical spectrum of hypertrophic cardiomyopathy
(HCM) are not completely understood, particularly the contribution of
mitral leaflet length and size. METHODS AND RESULTS. To clarify this issue,
mitral valve specimens from 43 patients with HCM and basal outflow
obstruction were used to relate morphometric measurements of leaflet area
to certain morphological and functional assessments of left ventricular
outflow tract geometry and valvular motion obtained from echocardiograms in
the same patients. Twenty-four patients (56%) had mitral valves of normal
size (leaflet area < 12.0 cm2) and 19 patients (44%) had enlarged and
elongated valves (area > or = 12.0 cm2). Compared with normal-sized
mitral valves, the enlarged valves were situated more posteriorly in a
larger left ventricular outflow tract (cross-sectional area, 3.3 +/- 1.0
versus 1.9 +/- 0.7 cm2 for normal-sized valves; p < 0.001) and also had
greater systolic excursion of the anterior leaflet (16.2 +/- 4.5 versus
13.3 +/- 3.3 mm, p < 0.02), usually with a distinctive sharp-angled bend
and localized contact of the leaflet tip with ventricular septum ("typical"
SAM); this pattern of SAM was possible because the central and distal
portions of the leaflet were relatively free of fibrous thickening. In
contrast, normal-sized mitral valves were situated more anteriorally in a
smaller left ventricular outflow tract and frequently showed a different
mechanism of SAM and subaortic obstruction with relatively limited leaflet
motion, absence of a sharp bend, and septal contact involving more
substantial portions of the anterior leaflet and contiguous chordae
("atypical" SAM); mitral-septal apposition was effected in large measure by
posterior ventricular septal motion. This pattern of SAM was invariably
associated with a more diffuse pattern of fibrous thickening. CONCLUSIONS.
Patients with obstructive HCM show patterns of mitral valve SAM that are
diverse and determined largely by the interrelation of left ventricular
outflow tract geometry, the size and mobility of the mitral leaflets, and
the presence and distribution of fibrous thickening.
ARTICLES
Morphological determinants of echocardiographic patterns of mitral valve systolic anterior motion in obstructive hypertrophic cardiomyopathy
Pathology Branche, National Heart, Lung, and Blood Institute, Bethesda, Md. 20892.
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