Arrhythmogenic right ventricular cardiomyopathy
Arrhythmogenic right ventricular cardiomyopathy
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DOI：
10.1136/bcr.05.2011.4242
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发表时间：
2011-08
期刊：
影响因子：
0.9
通讯作者：
Roopali Soni;Y. Oade
中科院分区：
文献类型：
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作者：
Roopali Soni;Y. Oade
心律失常性右室心肌病（ARVC）是一种罕见的遗传性心肌疾病，可导致年轻人和运动员室性心动过速和猝死。它导致纤维脂肪替代右心室和左心室心外膜下区域。它是肥厚性心脏病后年轻人心脏性猝死的最常见原因。诊断可能很困难，目前尚无治愈ARVC的方法。预防猝死是最重要的管理策略。儿科医生需要意识到出现心悸、疲劳、晕厥或心脏骤停的青少年和青壮年发生ARVC的可能性。作者提出了两个表面上健康的十几岁男孩突然意外死亡的案例。死后心肌检查强烈提示两例均为ARVC。
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a rare inherited disease of the heart muscle that causes ventricular tachyarrhythmias and sudden death in young people and athletes. It results in fibrofatty replacement of the right ventricle, and the subepicardial region of the left ventricle. It is the most common cause of sudden cardiac death in young people after hypertrophic heart disease. Diagnosis can be difficult and at present there is no cure for ARVC. Prevention of sudden death is the most important management strategy. Paediatricians need to be aware of the possibility of ARVC in adolescents and young adults presenting with palpitations, fatigue, syncope or cardiac arrest. The authors present two cases of apparently healthy teenage boys who died suddenly and unexpectedly. Postmortem examination of the myocardium was strongly suggestive of ARVC in both cases.