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CLINICAL RESEARCH: CARDIAC IMAGING

The Binary Endocardial Appearance Is a Poor Discriminator of Anderson-Fabry Disease From Familial Hypertrophic Cardiomyopathy

Stavros Kounas, MD^_*, Camelia Demetrescu, BSc, MD^_*, Antonios A. Pantazis, MD^_*, Andre Keren, MD, Philip J. Lee, DM, FRCPCH, FRCP, Derralynn Hughes, MA, DPhil, MRCP, MRCPath, Atul Mehta, MA, MD, FRCP, FRCPath and Perry Mark Elliott, MBBS, MD, FRCP, FACC, FESC^_*,_*

^_* Heart Hospital, University College London, London, United Kingdom
Hadassah University Hospital, Jerusalem, Israel
Charles Dent Metabolic Unit, National Hospital for Neurology and Neurosurgery, London, United Kingdom
Lysosomal Storage Disorders Unit, Royal Free Hospital, London, United Kingdom.

Manuscript received November 12, 2007; revised manuscript received January 24, 2008, accepted February 5, 2008.

^_* Reprint requests and correspondence: Dr. Perry Elliott, The Heart Hospital, 16-18 Westmoreland Street, London, W1G 8PH, United Kingdom. (Email: pelliott{at}doctors.org.uk).

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.

Abbreviations and Acronyms   AFD = Anderson-Fabry disease   -Gal = -galactosidase A   FS = fractional shortening   HCM = hypertrophic cardiomyopathy   LAD = left atrial diameter   LV = left ventricular   LVH = left ventricular hypertrophy   LVOT = left ventricular outflow tract

This article has been cited by other articles:

				M. Pieroni and F. Crea Binary sign in anderson-fabry cardiomyopathy. J. Am. Coll. Cardiol., March 3, 2009; 53(9): 813 - 814. [Full Text] [PDF]

				S. Kounas and P. Elliott Reply. J. Am. Coll. Cardiol., March 3, 2009; 53(9): 814 - 815. [Full Text] [PDF]