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Hypertrophic Cardiomyopathy with Unusual Extensive Scarring Pattern: Danon Disease Cover

Hypertrophic Cardiomyopathy with Unusual Extensive Scarring Pattern: Danon Disease

Open Access
|Oct 2016

Figures & Tables

Figure 1.

(A) Electrocardiogram showed sinus tachycardia with Wolff-Parkinson-White pre-excitation pattern. (B) Transthoracic echocardiogram apical 4-chamber view showed severe concentric left ventricular hypertrophy; the thickness of interventricular septum and lateral wall were 27 mm and 21 mm. LA: left atrium; LV: left ventricle; RA: right atrium; RV: right ventricle.

Figure 2.

Late gadolinium enhancement (LGE) cardiac magnetic resonance. The top row (4-chamber, 3-chamber, and 2-chamber LGE images) and the bottom row (from basal to apical short axis LGE images, left to right) showed diffuse LGE in the right ventricular insertion points and mid-myocardium, most prominent in the left ventricular lateral segments (arrows).

Figure 3.

Cardiac magnetic resonance delayed enhancement TI 600 ms showed a definite left ventricular apical thrombus (arrow).

Figure 4.

(A) Endomyocardial biopsy showed enlarged and vacuolated cardiomyocytes, hematoxylineosin statin, original magnification 400×. (B) Ultrastructure of myocardial biopsy showed myocardial fibers with accumulation of autophagic vacuoles, with degenerating mitochondria, glycogen, small vesicles, and granular debris. Magnification 8000×.

Language: English
Page range: 227 - 229
Published on: Oct 1, 2016
Published by: Houston Methodist DeBakey Heart & Vascular Center
In partnership with: Paradigm Publishing Services

© 2016 Dan D. Le, Paulino Alvarez, Roberto Barrios, Michael Arriaga, Mariyam Cherry, Dipan Shah, C. Huie Lin, published by Houston Methodist DeBakey Heart & Vascular Center
This work is licensed under the Creative Commons Attribution-NonCommercial 4.0 License.