Document Detail

Hypertrophic obstructive cardiomyopathy and lentiginosis: a little known neural ectodermal syndrome.
MedLine Citation:
PMID:  7193405     Owner:  NLM     Status:  MEDLINE    
Eleven patients, 10 male, with classic hypertrophic obstructive cardiomyopathy and lentiginosis are described. Physical examination showed differences from the few previously reported cases in that (1) this condition was not confined to children; (2) mental retardation, sensorineural deafness and gonadal and somatic infantilism were either rare or absent; and (3) detailed family studies provided no evidence that this condition was inherited. Nine patients underwent cardiac catheterization and left ventricular angiography; all had left ventricular outflow obstruction and three had concomitant right ventricular outflow obstruction with a pressure gradient in excess of 100 mm Hg. Ten of the 11 patients were severely symptomatic, and 7, each with a left ventricular pressure gradient of more than 70 mm Hg, underwent successful septal myotomy/myectomy that resulted in marked symptomatic improvement that was maintained after long-term follow-up.
M G St John Sutton; A J Tajik; E R Giuliani; H Gordon; W P Su
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  The American journal of cardiology     Volume:  47     ISSN:  0002-9149     ISO Abbreviation:  Am. J. Cardiol.     Publication Date:  1981 Feb 
Date Detail:
Created Date:  1981-04-21     Completed Date:  1981-04-21     Revised Date:  2007-11-15    
Medline Journal Info:
Nlm Unique ID:  0207277     Medline TA:  Am J Cardiol     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  214-7     Citation Subset:  AIM; IM    
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MeSH Terms
Cardiomyopathy, Hypertrophic / diagnosis*
Child, Preschool
Ectoderm / pathology*
Lentigo / diagnosis*
Middle Aged
Nervous System Diseases / diagnosis*
Radiography, Thoracic

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