Chauvel A, Blanc J F, Jouary T, Bioulac-Sage P
Service d'Anatomie Pathologique, Hôpital Pellegrin, Bordeaux.
Ann Pathol. 1998 Nov;18(5):425-8.
We report a case of arrhythmogenic right ventricular cardiomyopathy revealed by sudden death during exercise in a 13-year-old patient. Postmortem diagnosis was made on multiple tissue samples taken from right ventricular free wall, showing light adipous infiltration of the myocardium at gross examination. Arrhythmogenic right ventricular cardiomyopathy is histologically characterized by fibro-fatty replacement of right ventricular myocardium. Left ventricular involvement may be observed. Diagnosis at an early stage is often difficult. Etiology remains unknown. Since familial occurrence has been documented, postmortem identification is useful for the other members of the family.
我们报告一例13岁患者在运动中猝死，尸检发现致心律失常性右室心肌病。尸检诊断基于取自右室游离壁的多个组织样本，大体检查显示心肌有轻度脂肪浸润。致心律失常性右室心肌病的组织学特征是右室心肌纤维脂肪替代。可观察到左室受累。早期诊断往往困难。病因仍不明。由于已有家族发病的记录，尸检鉴定对家族其他成员有用。
