Analysis of the incidence characteristics of pathologically diagnosed ARVC patients with unexplained sudden death in Yunnan / 中华地方病学杂志

ABSTRACT

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 9 cases 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,1 case had eaten Trogia venenata;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 and related pathogenic gene detection.