Document Detail


The binary endocardial appearance is a poor discriminator of Anderson-Fabry disease from familial hypertrophic cardiomyopathy.
MedLine Citation:
PMID:  18498962     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
OBJECTIVES: We compared the frequency of a binary endocardial appearance in patients with hypertrophic cardiomyopathy (HCM) and Anderson-Fabry disease (AFD). BACKGROUND: A recent study suggested that a binary endocardial appearance is a highly sensitive and specific discriminator of AFD from other causes of hypertrophic cardiomyopathy (HCM). METHODS: Fourteen patients with AFD (55.4 +/- 9.9 years, 9 men) and 14 patients with HCM (57.2 +/- 10.9 years, 9 men) were randomly selected from a dedicated patient database. Two-dimensional echo images were blindly reviewed by 2 experienced echocardiographers. RESULTS: Maximum left ventricular (LV) wall thickness, LV end-systolic dimension, fractional shortening, and left atrial size were similar in the 2 patient groups. The LV end-diastolic dimension was smaller in patients with HCM (p = 0.04). A binary sign was present in 8 of 28 patients (29%). The sensitivity and specificity of the binary sign as a discriminator of AFD from HCM were 35% and 79%, respectively. A binary sign was present in only 1 patient with LV wall thickness <15 mm. CONCLUSIONS: The binary endocardial appearance lacks sufficient sensitivity and specificity to be used as an echocardiographic screening tool.
Authors:
Stavros Kounas; Camelia Demetrescu; Antonios A Pantazis; Andre Keren; Philip J Lee; Derralynn Hughes; Atul Mehta; Perry Mark Elliott
Publication Detail:
Type:  Evaluation Studies; Journal Article    
Journal Detail:
Title:  Journal of the American College of Cardiology     Volume:  51     ISSN:  1558-3597     ISO Abbreviation:  J. Am. Coll. Cardiol.     Publication Date:  2008 May 
Date Detail:
Created Date:  2008-05-23     Completed Date:  2008-06-25     Revised Date:  2009-03-27    
Medline Journal Info:
Nlm Unique ID:  8301365     Medline TA:  J Am Coll Cardiol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  2058-61     Citation Subset:  AIM; IM    
Affiliation:
Heart Hospital, University College London, London, United Kingdom.
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MeSH Terms
Descriptor/Qualifier:
Adult
Aged
Cardiomyopathy, Hypertrophic, Familial / ultrasonography*
Diagnosis, Differential
Double-Blind Method
Echocardiography
Endocardium / ultrasonography*
Fabry Disease / ultrasonography*
Female
Heart Ventricles / ultrasonography
Humans
Hypertrophy, Left Ventricular / ultrasonography
Male
Middle Aged
Observer Variation
Sensitivity and Specificity
Comments/Corrections
Comment In:
J Am Coll Cardiol. 2009 Mar 3;53(9):813-4; author reply 814-5   [PMID:  19245977 ]

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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