Valentina Agostini, Alberto Foà, Barbara Corti, Ornella Leone
Department of Pathology, S.Orsola-Malpighi University Hospital, Bologna, Italy
DOI: http://dx.doi.org/10.18053/jctres.aecvp2016.025
This case is a 49 year-old man who suffered cardiopulmonary arrest refractory to resuscitation maneuvers during physical exercise. Cardiovascular and familiar anamnesis was negative. The patient was a multi-tissue/multi-organ donor, so the heart was explanted to remove valves for the tissue bank, then examined at the Pathology Institute. The heart weighed 460 g, and was mildly dilated and altered in shape due to rounded apex. Multiple short-axis slices revealed left ventricle (LV) dilatation; the LV myocardial subepicardial border was irregular and scalloped due to the presence of multiple fibro-fatty areas infiltrating/replacing the mid-mural myocardium, circumferentially distributed, prevalently in the mid-basal antero-lateral and apical infero-lateral walls. The right ventricle (RV) was mildly dilated in the inflow tract, with a near regular subepicardial border; no parietal aneurysms or significant coronary artery alterations were found. Histology confirmed the extensive, diffuse fibro-fatty replacement of the LV myocardium, originating from the subepicardial layer and spreading toward the mid-mural area. The surrounding myocardium showed severe myocellular alterations (hypertrophy, dismetry, vacuolization, irregularly shaped nuclei). Fibro-fatty replacement also involved the mid-basal anterior septum, while the RV had only mild fatty infiltration with no significant fibrosis or myocyte alterations. No inflammation was found. Both macroscopy and microscopy findings strongly suggested a left dominant arrhythmogenic cardiomyopathy (LDAC). Genetic analyses are in progress. Very few cases with full pathology are recorded for LDAC, the rarest and most recently described subtype of AC, probably clinically under-recognized. With this case we want to emphasize the importance of pathology evaluation of discarded hearts.
Email: agostini.valentina@gmail.com
