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Circulation, Vol 84, 1188-1197, Copyright © 1991 by American Heart Association
HG Klues, WC Roberts and BJ Maron
BACKGROUND. Obstruction to left ventricular outflow in hypertrophic
cardiomyopathy (HCM) is usually due to systolic anterior motion of the
mitral valve. Occurrence of structural mitral valve abnormalities in HCM
and their significance in producing outflow obstruction (even in the
absence of typical systolic anterior motion) has not been fully
appreciated. METHODS AND RESULTS. Analysis of 78 mitral valves excised from
patients with obstructive HCM showed that 10 (13%) had anomalous insertion
of one or both left ventricular papillary muscles directly into the
anterior mitral leaflet. This malformation was identified by
echocardiography, which demonstrated direct continuity between the
hypertrophied papillary muscle and mitral leaflet, resulting in a long
rigid area of midcavity narrowing that appeared to be solely or largely
responsible for outflow obstruction. Basal subaortic pressure gradients
were large (70-150 mm Hg). Mitral valve replacement reduced the outflow
gradient substantially to 0-15 mm Hg in four patients with postoperative
cardiac catheterization. However, two other patients who underwent septal
myotomy/myectomy had persistent symptoms and incomplete relief of
obstruction (gradients 60 and 70 mm Hg) because of continued midcavity
apposition of papillary muscle and ventricular septum. CONCLUSIONS.
Anomalous papillary muscle insertion into anterior mitral leaflet
represents a mechanism of obstruction to left ventricular outflow in
patients with HCM and differs considerably from typical dynamic obstruction
caused by mitral valve systolic anterior motion that occurs in many other
patients with HCM. Recognition of this malformation emphasizes the diverse
morphological expression of HCM and also has important clinical
implications for patients requiring operation because the gradient is
likely to persist even after adequate myotomy/myectomy; consequently,
mitral valve replacement would appear to be the operation of choice in most
such patients.
ARTICLES
Anomalous insertion of papillary muscle directly into anterior mitral leaflet in hypertrophic cardiomyopathy. Significance in producing left ventricular outflow obstruction
Pathology Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, Md. 20892.
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