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. 2008 May 27;51(21):2058-61.
doi: 10.1016/j.jacc.2008.02.046.

The binary endocardial appearance is a poor discriminator of Anderson-Fabry disease from familial hypertrophic cardiomyopathy

Stavros Kounas  1 , Camelia Demetrescu, Antonios A Pantazis, Andre Keren, Philip J Lee, Derralynn Hughes, Atul Mehta, Perry Mark Elliott
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Free article

The binary endocardial appearance is a poor discriminator of Anderson-Fabry disease from familial hypertrophic cardiomyopathy

Stavros Kounas et al. J Am Coll Cardiol. .
Free article

Abstract

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.

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Comment in

  • Binary sign in Anderson-Fabry cardiomyopathy.
    Pieroni M, Crea F. Pieroni M, et al. J Am Coll Cardiol. 2009 Mar 3;53(9):813-4; author reply 814-5. doi: 10.1016/j.jacc.2008.07.074. J Am Coll Cardiol. 2009. PMID: 19245977 No abstract available.

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