Abstract：
BACKGROUND: Sudden deaths (SD) in young people including competitive athletes, albeit uncommon, are usually attributable to genetic, congenital or acquired cardiovascular conditions. However, it is under-appreciated that mitral valve prolapse (MVP), a relatively common valvular heart disease, is associated with SD in this youthful population.
METHODS: Forty-three MVP-related SDs are identified from 2 large cardiovascular registries with pathologic, clinical, and demographic findings reported.
RESULTS: Events occurred in both genders, but females were unexpectedly common (49%); median age was 22 ± 8 years, and 29 (67%) were engaged in competitive sports, including 17 with preparticipation examination. Of the 43 MVP cases, 21 died suddenly during or just after vigorous exercise including 6 during organized sports. Sixteen (37%) had been evaluated by a cardiologist resulting in confirmed MVP diagnosis in 11.. Pathologic findings characteristic of MVP included: bileaflet myxomatous involvement in all cases; and areas of interstitial or replacement myocardial fibrosis in 79%, most evident in posterolateral left ventricular wall.
CONCLUSIONS: Arrhythmogenic myxomatous degeneration (MVP) is an under-recognized cause of SD in young people including competitive athletes, disproportionally affecting females and requiring requires a high index of clinical suspicion. Frequency of left ventricular fibrosis in these young people with MVP suggests a mechanism for ventricular tachyarrhythmias and SD, relevant to future risk stratification.
METHODS: Forty-three MVP-related SDs are identified from 2 large cardiovascular registries with pathologic, clinical, and demographic findings reported.
RESULTS: Events occurred in both genders, but females were unexpectedly common (49%); median age was 22 ± 8 years, and 29 (67%) were engaged in competitive sports, including 17 with preparticipation examination. Of the 43 MVP cases, 21 died suddenly during or just after vigorous exercise including 6 during organized sports. Sixteen (37%) had been evaluated by a cardiologist resulting in confirmed MVP diagnosis in 11.. Pathologic findings characteristic of MVP included: bileaflet myxomatous involvement in all cases; and areas of interstitial or replacement myocardial fibrosis in 79%, most evident in posterolateral left ventricular wall.
CONCLUSIONS: Arrhythmogenic myxomatous degeneration (MVP) is an under-recognized cause of SD in young people including competitive athletes, disproportionally affecting females and requiring requires a high index of clinical suspicion. Frequency of left ventricular fibrosis in these young people with MVP suggests a mechanism for ventricular tachyarrhythmias and SD, relevant to future risk stratification.
摘要：
背景：包括竞技运动员在内的年轻人猝死（SD），虽然不常见,通常归因于遗传，先天性或获得性心血管疾病。然而,人们对二尖瓣脱垂（MVP）的认识不足，一种比较常见的心脏瓣膜病,在这个年轻的人群中与SD有关。
方法：从2个大型心血管疾病登记处鉴定出43个与MVP相关的SDs，临床,和人口调查结果报告。
结果：两种性别都发生了事件，但女性出乎意料地常见（49％）；中位年龄为22±8岁，29人(67%)从事竞技体育，包括17人参加预考试。在43个MVP案例中，21人在剧烈运动期间或之后突然死亡，其中6人在有组织的运动中死亡。心脏病专家对16例（37％）进行了评估，结果证实了11例的MVP诊断。.MVP的病理特征包括：所有病例的双叶粘液瘤受累；79%的间质性或置换性心肌纤维化区域，在左心室后外侧壁中最明显。
结论：致心律失常性粘液瘤变性（MVP）是年轻人（包括竞技运动员）中SD的公认原因，不成比例地影响女性，需要很高的临床怀疑指数。这些患有MVP的年轻人中左心室纤维化的频率提示室性心律失常和SD的机制。与未来风险分层相关。
方法：从2个大型心血管疾病登记处鉴定出43个与MVP相关的SDs，临床,和人口调查结果报告。
结果：两种性别都发生了事件，但女性出乎意料地常见（49％）；中位年龄为22±8岁，29人(67%)从事竞技体育，包括17人参加预考试。在43个MVP案例中，21人在剧烈运动期间或之后突然死亡，其中6人在有组织的运动中死亡。心脏病专家对16例（37％）进行了评估，结果证实了11例的MVP诊断。.MVP的病理特征包括：所有病例的双叶粘液瘤受累；79%的间质性或置换性心肌纤维化区域，在左心室后外侧壁中最明显。
结论：致心律失常性粘液瘤变性（MVP）是年轻人（包括竞技运动员）中SD的公认原因，不成比例地影响女性，需要很高的临床怀疑指数。这些患有MVP的年轻人中左心室纤维化的频率提示室性心律失常和SD的机制。与未来风险分层相关。
