Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) in clinical practice

Figures

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  Figure 1. Desmosomes (arrows) connect cytoskeletons of adjacent cells (myofibrils, double arrows) and contain centrally dense lamellae composed of polypeptides, plakoglobin, and other proteins. Clusters of mitochondria (asterisks) can be seen

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  Figure 2. The desmosomes (ellipse) show dense cell borders and glycoproteins in the intercellular space. Plakoglobin is a plaque protein of intercellular junctions

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  Figure 3. An electrocardiogram (ECG) showing monomorphic ventricular tachycardia (VT) with a left bundle branch block pattern with superior axis from a patient with arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D). Figure reproduced from ref. 23 with permission

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  Figure 4. An electrocardiogram (ECG) showing the presence of an epsilon wave (black arrow) that is pathognomonic of arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D)

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  Figure 5. Modified four‐chamber view during transthoracic echocardiography demonstrating a dilated right ventricle

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  Figure 6. Four‐chamber view of a cardiovascular magnetic resonance study in diastole (left panel) and systole (right panel) showing a very dilated RV

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  Figure 7. Short‐axis section of an explanted heart at the time of heart transplantation for ARVC/D demonstrates the right ventricular wall to be composed of fat tissue, and there is prominent right ventricular trabeculation. The myocardium of the numerous prominent trabecles appears macroscopically unaffected, which has been confirmed histologically

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  Figure 8. Full‐thickness section of the anterior right ventricular (RV) wall and interventricular septum (IVS) including epicardial fat (EF) using conventional hematoxylin and eosin (H&E) (left) and collagen stains (Sirius red) (right) at ×0.42 magnification. There is patchy fibro‐fatty replacement of the muscular wall affecting the anterior right ventricular wall and the right interventricular septum. The myocardial architecture of the left IVS is unaffected. Asterisk: coronary artery. The single‐headed arrow points toward endocardium. The double‐headed arrow marks the areas affected by fibro‐fatty replacement

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  Figure 9. Plakoglobin staining from different areas of right‐sided interventricular septum, at ×10 magnification. There is a variegated staining reaction with focal positive staining, and abrupt transition to an area of fibro‐fatty replacement showing negative staining reaction for Plakoglobin in rare cardiomyocytes (encircled) (left). There is patchy mild decreased staining reaction of heart muscle cells adjacent to hyalinized mid‐wall fibrosis. The endocardium is marked by an asterisk. The double‐headed arrow marks the area of subendocardial fibrous replacement