MORPHOLOGICAL DETERMINANTS OF ECHOCARDIOGRAPHIC PATTERNS OF MITRAL-VALVE SYSTOLIC ANTERIOR MOTION IN OBSTRUCTIVE HYPERTROPHIC CARDIOMYOPATHY

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 greater-than-or-equal-to 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.