AUTHORS: D. Dermengiu 1 , S.Dermengiu 1 , M.Costache 2 , S.Hostiuc 1 , L.Zarma 3 , M.Ceausu 1 1 National Institute of Legal Medicine, Bucharest, Romania, 2 Carol Davila University of Medicine and Pharmacy, Bucharest, Romania, 3 C.C.Iliescu Institute of Cardiology, Bucharest, Romania Arrhythmogenic right ventricular cardiomyopathy is a genetically transmitted cardiac disorder, characterized by increased myocardial instability and an increased risk for se- vere ventricular arrhythmias and sudden death. Histologically there are two main types: fatty, associated with sudden death at rest, and ibrofatty, associated with sudden death during exercise. he purpose of t his poster is to present a sudden death caused by an association of a ibrofatty type of arrhythmogen ic right ventricular cardiomyopathy in a young athlete, associated with a hemodynamically signi icant myocardial bridging on the let an terior descending artery, the latter causing an atypical distribution pattern of the ibrofatty iniltrat ion, that posed diagnostic di icult ies. A 25 year-old man collapsed on the football ield and was pronounced dead by the ambulance about 30 minutes later. He was a professional football player until a year ago, when he interrupted this activity as he was diagnosed with hepatitis. Six months ago he had another blackout on the football ield. As he was oten feeling tired and had thoracic pains, he went to a cardiology hospital, seven and than one month before his death. Both examinations revealed bradycardia, a QRS angle of 90 degrees, EVs, difuse hypovoltage, and repolarization abnormalities in the anterior territory (V1-V4), and lat T waves in all derivations. he ECG suggested a high probability of cardiomiopathy, either primary or ischemic, with a possible pericardial involvement, and the physicians recommended cardiac echography, myocardial scintigraphy, cardiac MRI, and coronarography. he patient refused these additional examinations. During the forensic autopsy was identiied a let ven tricle with an intense pale area, subendocardial, at the level of the interventricular septum. he thickness of the interventricular septum was 1.6-1.8 cm. he myocardial tissue contained numerous yellowish stripes, with areas of conluence and dissection. he right ventricle had a thickness of 0.1-0.3 cm, with an irregular pattern of the wall, with signiicant replacement of the myocardial tissue with adipose formations. he let an terior descendent coronary artery had a signiicant myocardial bridging, starting at about 2cm from the origin and extending down about 7cm, with a thickness varying from 0.2 to 0.6 cm. he circumlex artery inishes on the pulmonary face of the let ven tricle. he right coronary artery, having a normal diameter, without atherosclerosis, ended on the diaphragmatic surface, in the superior third of the posterior interventricular groove. Cardiac histological examination showed interstitial vascular edema, with hypoxic myocardial lesions, signiicant subepicardial lipomatosis, with extension in the adjacent myocardium. Trichrome Masson stain showed mild-moderate interstitial and perivascular lipomatosis, a pattern suggestive for ARVC. Even if the sudden deaths in athletes is a considered a rare phenomenon, with an estimated prevalence of between less than 1/100.000 cases[1] and about 3.5/100.000 cases[2], recent reports suggests that the cardiovascular risks associated with professional sports are signiicantly underestimated[3]. he presence of structural heart diseases, like hypertrophic cardiomyopathy or ARVC, even if only probable, should forbid the practice of performance sports[4]. As ARVC is one of the most frequent causes of sudden death in professional athletes[4], every athlete should be routinely checked for it, especially as most screening tests are readily available. In our case the ECG suggested the probability of a severe cardiovascular disorder (most likely a cardiomyopathy), that would normally forbid that person to go on the football court. However, the patient has not followed the medical recommendation, nor in restricting strenuous physical activities, nor in further researching the cause of the electrical abnormalities. Histologically, ARVC has two main phenotypes: - fatty (lipomatous) - transmural adiposus iniltration, with pseudo-hypertrophy of the right ventricle, afecting mostly the anterolateral and infundibular regions of the right ventricle. he postero-inferior part of the right ventricle and the let ven tricle are usually normal. Histologically there is identiiable a fatty iniltration, with rare foci of ibrosis, and normal residual myocytes. Sudden death is not linked with an increased physical activity (sudden death at rest). - ibrofatty - the wall is thinner, oten translucent, aneurisms are present in nearly half the cases, usually located in the apex cordis, infundibulum or the postero-inferior wall (the triangle of the right ventricular dysplasia). Septal involvement is rare but the let ven tricle is afected in almost half the cases. Histologically are identiiable ibro-fatty iniltration, myocyte degeneration, apoptosis, and T-cell lymphocyte inlammatory iniltrate[5-11]. Out patient had the ibrofatty type of ARVC, that is known to be associated with an increased risk of sudden death during strenuous activities. Moreover, the patient had a hemodynamically signiicant myocardial bridging on the let an terior descendant artery. he involvement of the interventricular septum, with increased ibrosis and lipomatosis, may be, at least partially caused by the myocardial bridge[12], explaining why the patient had increased ibro- lipomatosis in this area, known to be rarely involved in ARVC. Figure 1. Fatty iniltration and dilatation of the right ventricle Figure 2. Hemodynamically signiicant myocardial bridging on LAD Figure 3. Fatty iniltration and severe interstitial ibrosis in the RV (Masson, 4x). Inlet - severe fatty iniltration in the RV (HE, 4x) DISCUSSIONS CASE REPORT 1. Chandra N, Bastiaenen R, Papadakis M, Sharma S. Sudden Cardiac Death in Young Athletes. Journal of the American College of Cardiology 2013,61:1027-1040. 2. Harmon KG, Asif IM, Klossner D, Drezner JA. Incidence of sudden cardiac death in national collegiate athletic association athletes. Circulation 2011,123:1594-1600. 3. Pelliccia A, Fagard R, Bjornstad HH, Anastassakis A, Arbustini E, Assanelli D, et al. Recommendations for competitive sports participation in athletes with cardiovascular disease - A consensus document from the Study Group of Sports Cardiology of the Working Group of Cardiac Rehabilitation and Exercise Physiology and the Working Group of Myocardial and Pericardial Diseases of the European Society of Cardiology. European Heart Journal 2005,26:1422-1445. 4. hiene G, Nava A, Corrado D, Rossi L, Pennelli N. Right Ventricular Cardiomyopathy and Sudden-Death in Young-People. New England Journal of Medicine 1988,318:129-133. 5. Racette S, Sauvageau A. 1. Arrhythmogenic right ventricular cardiomyopathy/dysplasia mimicking rape homicide: A case report. Medicine Science and the Law 2005,45:356-360. 6. de la Grandmaison GL, Le Bihan C, Durigon M. Assessment of right ventricular lipomatosis by histomorphometry in control adult autopsy cases. International Journal of Legal Medicine 2001,115:105-108. 7. Tabib A, Loire R, Miras A, hivolet-Bejui F, Timour Q, Bui-Xuan B, et al. Unsuspected cardiac lesions associated with sudden unexpected perioperative death. European Journal of Anaesthesiology 2000,17:230-235. 8. Tabib A, Loire R, Chalabreysse L, Meyronnet D, Miras A, Malicier D, et al. Circumstances of death and gross and microscopic observations in a series of 200 cases of sudden death associated with arrhythmogenic right ventricular cardiomyopathy and/ or dysplasia. Circulation 2003,108:3000-3005. 9. Tabib A, Miras A, Taniere P, Loire R. Undetected cardiac lesions cause unexpected sudden cardiac death during occasional sport activity - A report on 80 cases. European Heart Journal 1999,20:900-903. 10. Fornes P, Lecomte D. Pathology of sudden death during recreational sports activity - An autopsy study of 31 cases. American Journal of Forensic Medicine and Pathology 2003,24:9-16. 11. D'amati G, Leone O, Di Gioia CRT, Magelli C, Arpesella G, Grillo P, et al. Arrhythmogenic right ventricular cardiomyopathy: Clinicopathologic correlation based on a revised deinition of pathologic patterns. Human Pathology 2001,32:1078-1086. 12. Hostiuc S, Curca GC, Dermengiu D, Dermengiu S, Hostiuc M, Rusu MC. Morphological changes associated with hemodynamically signiicant myocardial bridges in sudden cardiac death. he horacic and cardiovascular surgeon 2011,59:406-410 References