29例云南不明原因聚集性猝死的尸检病理学研究

Pathological study of unexpected sudden death clustered in family or village in Yunnan province:report of 29 cases of autopsy

目的分析云南不明原因聚集性猝死的病理特点,为其病因探索和实施干预提供依据。方法收集云南省进行这类猝死监测以来临床与病理资料较完整的尸检材料29例,占同期死亡病例的10.2%。年龄8至69岁(平均32岁),男16例、女13例。核查心脏标本,观察了主要脏器的组织病理改变,对其中5例还做了心脏传导系统检查。心肌炎的病理诊断参照 Dallas 标准和世界心脏联合会的共识,标准克山病的病理诊断依据国家相关卫生行业标准。结果心脏主要病理改变有:淋巴细胞性心肌炎11例、中性粒细胞性心肌炎3例、致心律失常性右室心肌病4例、左室肥厚合并流出道异常肌束2例、缺血性心脏病2例、过敏性支气管炎和慢性支气管炎合并肺气肿2例、主要脏器未找到明显致死性病理形态改变5例。29例中均未见克山病和扩张型心肌病的病理形态改变。心肌炎均为局灶性,5例心脏传导组织检查见2例有希氏束或左束支起始段急性出血,1例炎症波及房室结。4对家庭聚集性病例的病理改变不同,4例首发猝死中3例为心肌炎、1例慢性感染,续发猝死中3例与急性心肌缺血有关,1例为致心律失常性右室心肌病。多数病例伴肺水肿,少数伴呼吸道和肺部感染。部分出现淤血性或缺血性肝细胞坏死。结论云南不明原因聚集性猝死的基本病理改变不同,多数为心肌炎症、心脏发育异常和其他致死性心肺疾患,未见克山病的病理学证据,致心律失常性右室心肌病等基础性心脏病应引起重视,猝死的聚集性可能是多种因素的综合表现。

Objective To investigate the pathological features and causes of sudden death clustered in family or village in Yunnan province so as to provide the morphological basis for exploring its etiology and medical intervention. Methods Autopsy was performed on 29 cases of clustered in family or village in Yunnan province during the period 1991 -2006, 16 males and 13 females, aged 32 （8 -69）. accounting for 10. 2% of whole sudden unexpected deaths occurring in the same period. The heart, lung, liver, spleen, brain, kidney, intestinal tract, and other organs were examined macroscopically and histologically, including a study of cardiac conduction system in 5 cases. Pathological diagnosis of myocarditis was based on the Dallas Criteria and World Heart Federation＇s consensus while the histological evaluation of Keshan disease referred the China national guideline for pathological diagnosis of Keshan disease. Results Based on the main pathological changes and the causes of death, these cases were classified into seven groups （ group A - G）. Group A comprised 11 cases （38%） with lymphocytic myocarditis accompanied with focal myocardial necrosis or degeneration. Group B comprised 3 cases （ 10% ） with neutrophil myocarditis accompanied with focal myocytolysis or coagulation necrosis. Group C comprised 4 cases （ 14% ） with arrhythmogenic right ventricle cardiomyopathy in which fatty infiltration of myocardium was the only pathological finding. Group D comprised 2 cases （7%） with ischemic heart disease in which fresh or old foci of myocardial infarction were found but coronary stenosis was shown only in one case. Group E comprised 2 cases （7%） with left ventricle hypertrophy and obstructive muscle bundle in the outflow of left ventricle. Group F comprised 2 cases （7%） with allergic bronchitis or chronic bronchitis and pulmonary emphysema. Group G comprised the remaining 5 cases （17%） without any pathological finding that could explain sudden death. No cases suffered with Keshan disease and dilated cardiomyopathy. Focal but not diffuse inflammatory infiltration was the prominent histological feature of myocarditis in Yunnan cases. Among the five cases with histological examination of cardiac conduction system, 2 cases were detected to suffer from acute hemorrhage in His bundle and its left branching site, and the atrioventricular node of 1 case was involved. Different pathological changes coexisted in 4 pairs of family members as a cluster of sudden deaths. 3 of 4 first deaths had focal myocarditis and the other one had chronic infection. But 3 secondary deaths had myocardial ischemia and the other one had arrhythmogenic right ventricle cardiomyopathy. Pulmonary edema, acute respiratory infection and congestive or ischemic liver necrosis were found in some cases simultaneously. Conclusion The pathological changes of the cases of clustered sudden death in Yunnan province are various, such as myocarditis, myocardial dysplasia and the other lethal heart-lung disorders. No case of Keshan disease has been found. Arrhythmogenic right ventricle cardiomyopathy and other foundational heart diseases might act as a background. It is very hard to contribute only one etiological factor to the clustering of sudden death in Yunnan. It was most likely that multiple factors cluster and trigger an outbreak of death in a definite time and space.