云南不明原因猝死患者中ARVC病理诊断病例发病特征分析  

Analysis of the incidence characteristics of pathologically diagnosed ARVC patients with unexplained sudden death in Yunnan

作　　者：王跃兵[1] 杨林[1] 李兆祥[1] 马琳[1] 董毅[1] 习严梅 唐雪[1] 孙梦遥 黄文丽[1] Wang Yuebing;Yang Lin;Li Zhaoxiang;Ma Lin;Dong Yi;Xi Yanmei;Tang Xue;Sun Mengyao;Huang Wenli(Yunnan Institute for Endemic Disease Control and Prevention,Dali 671000,China)

机构地区：[1]云南省地方病防治所,大理671000

出　　处：《中华地方病学杂志》2018年第12期1011-1016,共6页Chinese Journal of Endemiology

摘　　要：目的分析云南不明原因猝死(云南猝死)尸检致心律失常性右室心肌病(ARVC)病理诊断病例的发病特征,为云南猝死与ARVC致病关系研究提供基础依据。方法整理9例云南猝死ARVC病理诊断病例的病理检查结果,结合流行病学调查资料,综合分析云南猝死ARVC病理诊断病例的发病特征。结果9例病例包括女性8例、男性1例,年龄为16~47岁;猝死时间集中在6—8月,主要分布于7个重病村的8个家庭。其中3例有云南猝死家族遗传史,2例有精神刺激史,1例有毒沟褶菌食用史。急性症状和体征表现为心慌、胸闷、呼吸急促和意识丧失等。病理观察为典型的ARVC改变,均以右心室病变为主,出现不同程度的心脏扩大和心室壁广泛脂肪组织浸润,其中6例脂肪浸润几乎达心壁全层。除ARVC病理变化外,有8例病例同时伴有心肌炎、心脏发育不良、肾病、肺水肿、肺炎和胰腺炎中的1种或几种病理性改变。在9例病例中,5例病因明确为ARVC,2例为ARVC合并肺水肿,1例为ARVC合并急性出血坏死性胰腺炎,1例为ARVC合并毒沟褶菌中毒。病例家属的临床检查异常指标主要表现为心律失常型心电图改变和心肌酶异常增高。结论9例病例具有典型云南猝死的流行病学发病特征,心脏组织学变化符合ARVC病理诊断标准。ARVC可能是部分云南猝死的病因,有待提高尸检例数结合病理检查和相关致病基因检测加以佐证。Objective To analyze the characteristic of Yunnan unexpected sudden death (YUSD)cases by pathological diagnosis of arrhythmogenic right ventricular cardiomyopathy (ARVC),in order to offer clue for ARVC etiologic research of YUSD.Methods The pathological diagnosis results of 9 cases of sudden death of ARVC in Yunnan,as well as epidemiological investigation data,were used to comprehensively analyze the pathological features of the pathological diagnosis of ARVC in Yunnan.Results The 9cases including 8 females and 1 male, aged 16-47 years.The sudden death time was from June to August,mainly distributed in 8 families from the disease seriously ridden 7 villages.Three of them had a genetic history of family YUSD,2 cases had a history of mental stimulation,1case had eaten Trogia venenota;and acute symptoms and signs were palpitation,chest tightness,shortness of breath,and loss of consciousness.Pathological observations were the typical ARVC change, mainly right ventricular lesions,with different degrees of cardiac enlargement and extensive adipose tissue infiltration in the ventricular wall.Among them,6 cases of fat infiltration almost reached the full thickness of the heart wall.In addition to the pathological changes of ARVC,8 cases were accompanied by one or several pathological changes in myocarditis,cardiac dysplasia,nephropathy,pulmonary edema,pneumonia and pancreatitis.Of the 9 cases,5 cases were diagnosed with ARVC,2 cases with ARVC and pulmonary edema,1 case with ARVC and acute hemorrhagic necrotizing pancreatitis,and 1 case with ARVC and Trogia venenata poisoning.The clinical examination abnormalities of the family members of the cases mainly showed arrhythmogenic electrocardiography changes and abnormal myocardial enzymes.Conclusions The nine cases have showed typical epidemiology characteristics of YUSD,and cardiachistological changes are consistent with the ARVC pathological diagnostic criteria.A part of YUSD cases may be caused by ARVC,and the inference will be proved by cadaveric pathologic examination a

关 键 词：猝死 致心律失常性右室心肌病 病理诊断 

分 类 号：R541.78[医药卫生—心血管疾病]