Circulation, Vol 56, 1034-1038, Copyright © 1977 by American Heart Association
NM Katz, MJ Buckley and RR Liberthson
The presentation, management, and follow-up of 31 patients with discrete
membranous subaortic stenosis (DMSS) is presented. DMSS comprised 16% of
185 patients with congenital left ventricular (LV) obstruction. Only one
patient was older than 40 years. The rarity of DMSS in older patients in
both our population and in the literature is noted, and possible
explanations are discussed. One-quarter of these patients had dyspnea,
chest pain, or syncope combined with electrocardiographic left ventricular
hypertrophy (LVH) and strain, and these all had peak LV outflow gradients
(PSG) greater than 85 mm Hg. One-quarter had neither symptoms nor
electrocardiographic abnormalities and all had PSG less than 90 mm Hg.
Bacterial endocarditis was found in 13%, and in 13% an immediate family
member also had congenital LV obstruction. Following surgical resection (25
patients), 18 were asymptomatic, two had residual fibromuscular
obstruction, and four developed new fibromuscular obstruction after from
one to six years (leading in one to late sudden death). Thus, even after
resection, these patients require continued re-evaluation for residual or
new LV obstruction.
ARTICLES
Discrete membranous subaortic stenosis. Report of 31 patients, review of the literature, and delineation of management
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