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Arrhythmogenic Right Ventricular Cardiomyopathy by Cristina Basso, Gaetano Thiene, Domenico Corrado, Annalisa Angelini, Andrea Nava, and Marialuisa Valente Circulation Volume 94(5):983-991 September 1, 1996 Copyright © American Heart Association, Inc. All rights reserved. An asymptomatic 17-year-old boy died suddenly during a soccer game. Cristina Basso et al. Circulation. 1996;94:983-991 Copyright © American Heart Association, Inc. All rights reserved. Same case as discussed in legend to Fig 1. a, In vitro NMR cross-sectional view showing a uniformly whitish right ventricular free wall with anterior and inferior aneurysms; note also a spotty involvement of the posterolateral wall of the left ventricle. b, Corresponding cross section of the heart specimen with infundibular and inferior subtricuspidal aneurysms. c, Panoramic histological view of the inferior aneurysm showing wall thinning with fibrofatty replacement (Azan stain; original magnification, ×2.5). Cristina Basso et al. Circulation. 1996;94:983-991 Copyright © American Heart Association, Inc. All rights reserved. Same case as discussed in legends to Figs 1 and 2. a, In vitro NMR scan in the long-axis view showing extensive involvement of the anterior free wall of the right ventricle and patchy involvement of the posterior wall of the left ventricle; the interventricular septum is spared. b, Corresponding panoramic histological view of the left ventricle showing a spot of fibrofatty replacement of the myocardium (Azan stain; original magnification, ×2.5). Cristina Basso et al. Circulation. 1996;94:983-991 Copyright © American Heart Association, Inc. All rights reserved. A 26-year-old male cyclist died suddenly at rest. Cristina Basso et al. Circulation. 1996;94:983-991 Copyright © American Heart Association, Inc. All rights reserved. Same case as discussed in legend to Fig 4. a, In vitro NMR scan in the long-axis view showing bright streaks in the pulmonary infundibulum; left ventricle and interventricular septum are normal. b, The pulmonary infundibulum appears translucent. c, Panoramic histological view of the pulmonary infundibulum showing regional fibrofatty replacement (Azan stain; original magnification, ×2.5). Cristina Basso et al. Circulation. 1996;94:983-991 Copyright © American Heart Association, Inc. All rights reserved. Histology of the fibrofatty variety of ARVC. a, Myocardial atrophy with fibrous and fatty tissue replacement (Azan stain; original magnification, ×240). b, Patchy inflammatory infiltrates associated with myocytolysis and scarring (hematoxylin and eosin; original magnification, ×240). c, CD45-positive mononuclear infiltrates adjacent to dying and damaged myocytes (avidin biotin peroxidase complex method; original magnification, ×240). d, Adipocytes taking the place of myocyte loss (hematoxylin and eosin; original magnification, ×240). Cristina Basso et al. Circulation. 1996;94:983-991 Copyright © American Heart Association, Inc. All rights reserved. A 39-year-old man with a family history of sudden death and left bundle-branch block morphology ventricular arrhythmias died suddenly at rest. a, Gross view of the heart specimen with massive fatty replacement of the right ventricular free wall. b, Histology of the right ventricular myocardium showing almost transmural fatty replacement, frequently reaching the endocardium, in the absence of fibrous tissue (Azan stain; original magnification, ×2.5). Cristina Basso et al. Circulation. 1996;94:983-991 Copyright © American Heart Association, Inc. All rights reserved. Control heart from a 20-year-old man who died in a motor vehicle accident. Cristina Basso et al. Circulation. 1996;94:983-991 Copyright © American Heart Association, Inc. All rights reserved. Transmission electron microscopic view of the right ventricular myocardium from heart specimens removed at the time of cardiac transplantation, with a histological picture of fibrofatty replacement. a, Cell debris, consisting mainly of swollen mitochondria and lysosomes, are visible adjacent to normal myocytes, suggesting myocyte death (from a 28-year-old woman with biventricular involvement, congestive heart failure, and a history of palpitations and ventricular arrhythmias). b, A lymphocyte is present within fibrotic interstitium, close to a damaged myocyte (from a 41-year-old man with biventricular involvement, congestive heart failure, and a history of palpitations and ventricular arrhythmias). Cristina Basso et al. Circulation. 1996;94:983-991 Copyright © American Heart Association, Inc. All rights reserved.