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(Circulation. 2008;118:e71-e72.)
© 2008 American Heart Association, Inc.

Images in Cardiovascular Medicine

Lipomatous Hypertrophy of the Interventricular Septum

Echocardiography, Cardiac Magnetic Resonance, and Multidetector Computerized Tomography Imaging

Eric Stephant, MD; Martine Barthelet, MD; Pierre-Yves Leroux, MD; Didier Revel, MD

From the Sainte-Anne Armed Forces Teaching Hospital, Toulon (E.S.), and Louis Pradel Hospital, Bron (M.B., P.L., D.R.), France.

Correspondence to Dr Eric Stephant, HIA Sainte-Anne, Boulevard de Sainte-Anne, 83000 Toulon, France. E-mail eric.stephant@caramail.com

An extract of the first 250 words of the full text is provided, because this article has no abstract.

A healthy 17-year-old boy with neither a personal nor familial cardiac history saw his family physician for a routine examination before participating in an athletic competition. His systolic blood pressure was normal. On physical examination, a heart murmur was detected and the boy was sent to a cardiologist for echocardiography, which showed hyperechogenic and increased interventricular septal thickness resembling an asymmetric hypertrophic cardiomyopathy (online-only Data Supplement Movie I). However, no mitral leak, no obstruction of the left ventricular outflow tract, and no systolic anterior motion were observed, and left ventricular function was normal, even during exercise testing. The ECG showed no increased PR interval and no T-wave anomaly or ventricular arrhythmia (Figure 1). Because of the unusual hyperechogenicity of the interventricular septum, indicating a possible infiltrative cardiomyopathy such as Fabry disease (glycosphingolipid storage disease), the patient underwent further investigations with cardiac magnetic resonance imaging (online-only Data Supplement Movie II and Figure 2), multidetector computerized tomography (MDCT) (Figure 3), and a blood sample analysis of -galactosidase A activity.

View larger version (111K):   Figure 1. ECG at rest: normal sinus rhythm.

View larger version (65K):   Figure 2. A, Magnetic resonance T2-weighted image with fat suppression (black blood inversion recovery sequence), 4-chamber view. B, Magnetic resonance T1-weighted image with contrast (gadolinium), 4-chamber view; hyperintense signal from the affected area. C, Magnetic resonance postcontrast T1-weighted image with fat suppression (chemical shift selective presaturation technique); spectral attenuation of the fat within the area.

View larger version (131K):   Figure 3. MDCT, short-axis view: negative value in region of interest (–57 HU) confirming fat . . . [Full Text of this Article]